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  • Articles: DFG German National Licenses  (1,620)
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  • Articles: DFG German National Licenses  (1,620)
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  • 2000-2004  (820)
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  • 1
    ISSN: 1436-3771
    Keywords: Key words Age ; Children ; Dental fluorosis ; Severity ; Tooth eruption
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The purpose of this study was to investigate the influence of age on the severity of dental fluorosis in children exposed to drinking water with either low or high fluoride concentrations. The children selected for this study were aged 10–14 years, with 28 permanent teeth and at least 1 tooth pair with fluorosis. The children were permanent residents of districts in western Uganda with either 0.5 mg (n=33) or 2.5 mg fluoride/l in drinking water (n=186). All vestibular tooth surfaces were examined for fluorosis using the modified Thylstrup and Fejerskov (TF) index. In the high fluoride community, the proportion of teeth per child with TF scores ≥4, and ≥5 was significantly higher among children aged 13–14 years compared to those aged 10–12 years. Children’s chronological age correlated positively and significantly with the median TF scores for all teeth, including early erupting (first molars and incisors) and late erupting teeth (canines, premolars and second molars). In linear regression analyses, the median TF score for all teeth, as well as for early erupting and late erupting teeth, increased significantly with age. On the other hand, in the low fluoride community there was no significant association between age and the severity of fluorosis. This study showed a significant increase in the severity of fluorosis with increasing age in a high fluoride community, whereas no change in severity with age was observed in a low fluoride community.
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  • 2
    ISSN: 1436-2813
    Keywords: Key Words: genetic changes ; prognostic factor ; breast cancer ; amplification ; loss of heterozygosity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: ERBB2 , INT2, and MYC genes, in 131 patients with breast carcinoma, 49 of whom had lymph node involvement, but none of whom had distant metastases. Among the several chromosome arms tested, LOH at 17q was correlated with lymph node metastasis. Amplification of the ERBB2, MYC, and INT2 genes was found more frequently in tumors from patients with lymph node metastases than in tumors from those without lymph node metastases. Univariate analysis demonstrated that LOH at 17q and INT2 amplification were factors influencing disease-free survival (DFS). A multivariate analysis was performed on 89 tumors that were able to be evaluated for both LOH at 17q and INT2 amplification, and the results showed that patients who had tumors with these genetic changes were more likely to have a poor prognosis. The findings of this study suggest that investigating genetic changes, in addition to conventional clinicopathologic factors, may contribute to defining groups of breast cancer patients with differences in prognosis.
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Adsorption 6 (2000), S. 137-147 
    ISSN: 1572-8757
    Keywords: adsorption ; kinetics ; linear driving force model ; process design
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Physics , Process Engineering, Biotechnology, Nutrition Technology
    Notes: Abstract The Linear Driving Force (LDF) model for gas adsorption kinetics is frequently and successfully used for analysis of adsorption column dynamic data and for adsorptive process designs because it is simple, analytic, and physically consistent. Yet, there is a substantial difference in the characteristics of isothermal batch uptake curves on adsorbent particles by the LDF and the more rigorous Fickian Diffusion (FD) model. It is demonstrated by using simple model systems that the characteristics of the adsorption kinetics at the single pore or the adsorbent particle level are lost in (a) evaluating overall uptake on a heterogeneous porous solid, (b) calculating breakthrough curves from a packed adsorbent column, and (c) establishing the efficiency of separation by an adsorptive process due to repeated averaging of the base kinetic property. That is why the LDF model works in practice.
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  • 4
    ISSN: 1572-879X
    Keywords: ammonia synthesis ; kinetics ; ruthenium catalysts ; promotional effect
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Notes: Abstract The kinetics of NH3 synthesis over carbon-based ruthenium catalysts promoted with barium or alkali was studied. Both the ammonia partial pressure dependencies of the reaction rates (T = 400°C, p = 63 bar, H2 : N2 = 3 : 1) and the pressure variations of the activity (T = 370°C, p= 4–63 bar, H2 : NN2 = 3 : 1) were found to be different for Ba and for the alkali (K, Cs). Ba–Ru/C proved to be more sensitive to the NH3 content and to the total pressure. The rate of synthesis over the alkali-promoted catalysts is, in turn, much stronger influenced by the ruthenium dispersion. TOFs of NH3 synthesis for the promoted samples at 370°C and 4 bar (Ba 0.085 1/s, Cs 0.05 1/s, K 0.035 1/s) are significantly higher than that for the Ru(0001) basal plane (0.0085 1/s results from the literature data at 370°C, 2 bar). The most active Ru/C samples (Ba or Cs) exceed significantly the fused iron catalyst, especially at high conversions.
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Topics in catalysis 11-12 (2000), S. 327-333 
    ISSN: 1572-9028
    Keywords: hydrodenitrogenation ; toluidine ; methylcyclohexylamine ; kinetics ; nickel-promoted molybdenum sulphide
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Notes: Abstract The hydrodenitrogenation (HDN) of o-toluidine and its reaction intermediates was studied over a NiMo/γ-Al2O3 catalyst. The kinetics of the HDN of methylcyclohexylamine and of the hydrogenation of cyclohexene were also studied. Hydrogenation of o-toluidine alone produces methylcyclohexene and methylcyclohexane. When a sufficient quantity of cyclohexene is added during the HDN of toluidine, methylcyclohexylamine, the first intermediate in the hydrogenation of toluidine, becomes detectable. Because of its strong adsorption constant and high rate constant for reacting further to methylcyclohexene and methylcyclohexane, methylcyclohexylamine is not observed in the HDN of toluidine. Adding cyclohexene decreases the adsorption of methylcyclohexylamine, thus enabling its detection. The rate and adsorption constants of methylcyclohexylamine and cyclohexene in the HDN of methylcyclohexylamine were calculated by fitting the kinetic data to a Langmuir–Hinshelwood equation. A two-site model was used to describe the surface reactions, with one site for the methylcyclohexylamine reactions and the other for the cyclohexene reaction.
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    The journal of headache and pain 1 (2000), S. 169-172 
    ISSN: 1129-2377
    Keywords: Key words Migraine ; Sleep ; Sleep apnea ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In our previous study we found a high prevalence of disordered sleep breathing in migraine children vs. controls. Since no quantitative studies about sleep respiratory disorders have been carried out in migraine children, we performed a polysomnographic (PSG) study in 10 migraine patients (7 boys, 3 girls; mean age 8.11 years, range, 5.8–14.5) attending the Headache Center of our department, to evaluate the presence of sleep apnea. Mothers completed a headache diary and a sleep diary for at least 1 month and filled out a sleep questionnaire. PSG data showed a normal sleep architecture in 3 cases, an insomnia pattern in 2, a reduction of slow wave sleep in 3 and a reduction of REM sleep in 2. Respiratory analysis revealed that 2 of 10 patients had obstructive sleep apnea. These 2 patients presented habitual snoring and associated sleep disturbances such as restless sleep and hypnic jerks. Sleep apnea may be a subtle and often undiagnosed symptom in several migraine patients. The report of habitual snoring associated with other sleep disturbances such as restless sleep and other parasomnias may be a sign of sleep apnea in migraine children.
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  • 7
    ISSN: 1129-2377
    Keywords: Key words Cluster headache ; Familial occurrence ; Genetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A Danish genetic study showed increased risk of cluster headache (CH) among relatives of CH patients. We studied the families of 191 CH patients (118 males, 73 females; mean age 45.9 years) attending the Milan Headache Center. Information on 3589 relatives was collected by direct interview of the probands (n = 118) or by mailed questionnaire (n = 73). The diagnostic criteria of the IHS were used. A positive family history was found in 19% (37 of 191) of the families. A total of 32 first-degree (32 of 1036, 3.1%) and 15 second-degree (15 of 2553, 0.6%) relatives were affected. The relative risk of CH was 26.89 (95% CI, 17.57–36.21) in the first-degree relatives and 4.35 (95% CI, 2.13–5.21) in second-degree relatives. This study shows increased familial risk of cluster headache in an Italian population and confirms that cluster headache is, in some families, and inherited disorder.
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    The journal of headache and pain 1 (2000), S. S135 
    ISSN: 1129-2377
    Keywords: Key words Migraine ; Headache ; Genetics ; Serotonin ; Dopamine ; Mitochondria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Migraine carries a significant hereditary determination. Familial hemiplegic migraine (FHM) has been recently linked to mutations in the CACNA1A gene on chromosome 19. CACNA1A codes for a subunit of a neural calcium channel. Other linkage loci on chromosome 1q21-23 and 1q31 have been reported. Several linkage and association studies have been performed to determine the role of the CACNA1A gene, and of other candidate genes implicated in the metabolism of serotonin and dopamine, in the more common types of migraine. Co-morbidity of migraine with vascular events has been analysed versus genetic prothrombotic factors and mitochondrial DNA, and genes involved in the inflammatory cascade have been explored. Though no definite conclusions have emerged from these studies as yet, molecular genetics of migraine can be expected to unravel the complex aetiologies of these fascinating diseases.
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    The journal of headache and pain 1 (2000), S. S147 
    ISSN: 1129-2377
    Keywords: Key words Dopamine ; Migraine ; Genetics ; DRD2
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Molecular genetics offers a novel approach to the understanding and management of migraine since the disorder is known to have a strong genetic component. In recent studies, polymorphisms in the genes for dopamine receptors have been evaluated. Both positive and negative association studies have been reported. In particular, these data suggest that activation of the DRD2 receptor plays a modifying role in the pathophysiology of migraine. As a result, existing data provide a molecular rationale for the documented efficacy of dopamine D2 receptor antagonists in the treatment of migraine. Therefore, at the present time, molecular genetic data provide support for the hypothesis that susceptibility to migraine may be modified, in part, by variations in dopamine DRD2 receptor function.
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    The journal of headache and pain 1 (2000), S. S157 
    ISSN: 1129-2377
    Keywords: Key words Migraine ; Chronic tension type headache ; Genetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Pathophysiological studies have dominated migraine research for several years. However, these studies are difficult to interpret because it is difficult to decide whether the observed phenomena are primary or secondary to the migraine attack. For that reason it is important that future migraine research focus on studies that concern migrain etiology. Migraine is a paroxysmal disorder. It is most likely and ion-channel disorder like familial hemiplegic migraine. The present paper focuses on genetic factors in migraine and chronic tension-type headache.
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  • 11
    ISSN: 1129-2377
    Keywords: Key words Coeliac disease ; Headache ; Children ; HLA antigens
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The linkage between HLA antigens and disease susceptibility has been investigated in several diseases. Two different mechanisms are known to act in the relation between the HLA system and headache: linkage and association of alleles. Among neurological disorders associated with coeliac disease (CD) we focused on headache in 1997. From a group of 70 coeliac children, we studied 10 children with headache (3 boys and 7 girls). For each subject we evaluated clinical history and HLA antigens. The incidence of headache was not different with respect to the prevalence of headache in the general population. The HLA setting is not different between the 2 groups examined. However, we highlight 2 cases for the particular HLA setting.
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  • 12
    ISSN: 1569-8041
    Keywords: BRCA1 ; breast cancer ; p53 ; survival
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background:The association between BRCA1 germ-linemutations and breast cancer prognosis is controversial. A historical cohortstudy was designed to determine the prognosis for women with axillary lymphnode negative hereditary breast cancer. Patients and methods:We tested pathology blocks from 118Ashkenazi Jewish women with axillary lymph node negative breast cancer for thepresence of the two common BRCA1 founder mutations, 185delAG and5382insC. Patients were followed up for a median of 76 months. SomaticTP53mutations were screened for by immunohistochemistry, and directsequencing was performed in the BRCA1-positive tumours. Results:Sixteen breast cancer blocks (13.6%) carried aBRCA1 mutation. Young age of onset, high nuclear grade, negativeestrogen receptor status and over-expression of p53 were highly associatedwith BRCA1-positive status (P-values all 〈0.01).BRCA1 mutation carriers had a higher mortality than non-carriers(five-year overall survival, 50% and 89.6%, respectively,P = 0.0001). Young age of onset, estrogen receptor negative status,nuclear grade 3, and over-expression of p53 also predicted a poor outcome. Coxmultivariate analyses showed that only germ-line BRCA1 mutationstatus was an independent prognostic factor for overall survival (P= 0.01). Among nuclear grade 3 tumours, the BRCA1 mutation carrierstatus was a significant prognostic factor of death (risk ratio 5.8,95% confidence interval: 1.5–22, P = 0.009). Sequencingof BRCA1-related breast cancers revealed one TP53missensemutation not previously reported in breast cancer. Conclusions:Using a historical cohort approach, we haveidentified BRCA1 mutation status as an independent prognostic factorfor node negative breast cancer among the Ashkenazi Jewish women. Thosemanaging women carrying a BRCA1 mutation may need take these findingsinto consideration. Additionally, our preliminary results, taken together withthe work of others suggest a different carcinogenic pathway inBRCA1-related breast cancer, compared to non-hereditary cases.
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  • 13
    ISSN: 1569-8041
    Keywords: BCL-2 ; breast cancer ; HER-2 ; p53 ; predictive factor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background:The selection of therapies for breast cancer is todaybased on prognostic features (chemotherapy, radiotherapy), hormone receptorstatus (hormonal therapy) and HER-2 status (trastuzumab therapy). HER-2,p53and BCL-2are tumour-related proteins that have thepotential to further improve individualisation of patient management, bypredicting response to chemotherapy, hormonal therapy and radiotherapy. Materials and methods:This paper reviews the rationale for theuse of these proteins as predictive factors, as well as the publishedliterature addressing the use of each one to predict response to hormonaltherapy, chemotherapy and radiotherapy. Results:HER-2, p53and BCL-2remaininadequately assessed as predictive factors in breast cancer. HER-2 evaluationis required for the selection of patients for trastuzumab (Herceptin®)therapy, as trials of this therapy have been limited to HER-2 overexpressors.HER-2 overexpression may be predictive of resistance to hormonal therapy.Anthracyclines are effective therapy for breast cancer regardless of HER-2status, but patients whose tumours overexpress HER-2 appear to receive thegreatest relative benefit from this therapy. Studies of HER-2 as a predictorof response to CMF and to radiotherapy are inconclusive at this time. No datayet exist to support the use of p53or BCL-2as predictivefactors in the therapy of breast cancer. Conclusions:At this point in time, there is inadequate evidenceto support the use of HER-2, p53or BCL-2to guide theselection of hormonal therapy, chemotherapy or radiotherapy for breast cancer.
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  • 14
    ISSN: 1569-8041
    Keywords: breast cancer ; camptothecins ; colorectal cancer ; GI147211 ; non-small-cell lung cancer ; topoisomerase I
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background:GI147211 is a water-soluble synthetic analogue ofcamptothecin showing promising in vivoand in vitroantitumor activity and an acceptable toxicity profile. Patients and methods:Between April 1995 and November 1996, 67eligible patients with pretreated breast cancer (25 patients) andchemo-naïve colorectal (19 patients) and non-small-cell lung cancer (23patients) were entered into three multicentric, non-randomized phase IItrials. Treatment schedule consisted of intravenous GI147211 administered ata dose of 1.2 mg/m2/day for five consecutive days every threeweeks. Results:Hematological toxicity was common with grade 3–4neutropenia in 54% of patients and neutropenic fever together or notassociated with infection in 14.5% of patients. Grade 3–4thrombocytopenia and grade 2–4 anemia were observed in 20% andin 68% of patients, respectively. Non-hematological toxicity wasgenerally mild to moderate and consisted mainly of gastrointestinal toxicity,asthenia and alopecia. A dose-escalation to 1.5 mg/m2/d wasfeasible in 17 (25%) patients. The antitumor activity of GI147211 wasmoderate in breast cancer patients (3 partial responses (PRs), response rate(RR) 13%) and minimal in non-small cell lung cancer patients (2 PRs,RR 9%). No objective responses were obtained in colorectal patients. Conclusions:GI147211, at the dose and schedule employed in thisstudy, showed an acceptable safety profile but a modest antitumor activity inthe examined tumor types.
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  • 15
    ISSN: 1437-2320
    Keywords: Key words Von Hippel-Lindau disease ; Hemangioblastoma ; Endolymphatic sac tumor ; Angiogenesis ; Genetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Von Hippel-Lindau disease (VHL) is a hereditary cancer syndrome caused by germline mutations of the VHL tumor suppressor gene. Major progress has been made in the last decade in both clinical and fundamental aspects of VHL. The VHL gene product, pVHL, has major and multiple functions: pVHL regulates not only first angiogenesis but also extracellular matrix formation and the cell cycle. A molecular diagnosis of VHL is now available, leading to a transformation in clinical management of patients and their families. Diagnosis of VHL has to be suspected in patients with a VHL-related tumor without familial history and especially in case of hemangioblastoma or endolymphatic sac tumors. Such patients should be systematically investigated for clinical and molecular evidence of VHL disease. Treatment of symptomatic hemangioblastomas remains mainly neurosurgical, often in emergency, but stereotactic radiosurgery is emerging as an alternative therapeutic procedure. In the future, antiangiogenic drugs could represent a potential medical treatment of CNS hemangioblastomas in view of their highly vascular structure. Lastly, visceral manifestations of VHL disease are also of critical importance and require early detection for effective treatment.
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  • 16
    Electronic Resource
    Electronic Resource
    Springer
    Adsorption 6 (2000), S. 349-357 
    ISSN: 1572-8757
    Keywords: sulfadiazene ; adsorption ; kinetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Physics , Process Engineering, Biotechnology, Nutrition Technology
    Notes: Abstract To investigate the nature of interactive forces between sulfadiazene molecules and alumina surface the experiments were performed for the adsorption of sulfadiazene (SD) from its aqueous sulution onto the alumina surfaces at 25 ± 0.2°C and the influence of factors such as increasing concentration of SD (4.0–20.0 × 10−3 mol cm−3), the time required for adsorption equilibrium, pH (2.0–12.0) and temperature (5–45°C) of the adsorption medium, the presence of ions like Cl−, SO2− 4 and PO3− 4 (0.01–0.30 M) and organic solvents (5% v/v) were observed on the course of adsorption of SD. Various adsorption and kinetic parameters such as adsorption coefficient, the rate constants for adsorption and desorption were also evaluated. The results of the above cited studies facilitated to formulate the mechanisms of interaction between SD and alumina surfaces. From application view point the present work may be a potential tool for an effective chromatographic separation of sulfa drugs from industrial effluents.
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  • 17
    Electronic Resource
    Electronic Resource
    Springer
    Biomedical microdevices 2 (2000), S. 305-316 
    ISSN: 1572-8781
    Keywords: membranes ; breast cancer ; oncology ; cell column regulation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine , Technology
    Notes: Abstract Using microfabrication technology, we have developed a new experimental apparatus and technique which allow isolation of individual cells and which facilitate the study of kinetic volume changes and membrane permeability. The key component of the apparatus is a microdiffusion chamber which was constructed using silicon microfabrication technology and standard photolithography. The central unit of the chamber is a 1 μ m thick silicon nitride membrane with a center hole on the order of 2–3 μ m in diameter. The device is novel in its analysis of a single cell, instead of the traditional array of cells, and its avoidance of the damage artifacts and computational difficulties which are inherent in other, commonly used methods of cellular analysis. The device is used in conjunction with a predictive computer model which simulates the response of the entire membrane or a portion of the membrane to various permeant and impermeant concentrations. This study introduces the apparatus and the model, and illustrates the effectiveness of the new procedure by determining several membrane permeability coefficients for HBL-100 (healthy human breast line). The empirical data and theoretical data were combined to yield a water permability (L p) of 1.1 ± 0.5μ m/(min-atm) (mean ± 1 standard deviation) (N= 5) during the uncoupled transport of water at 22 ±C. In the presence of 6 M glycerol, the water permeability (L p), permeability coefficient (P S), and the reflection coefficient (σS) were determined to be 2.0 ± 0.63 μ m/(min-atm), 2.7E-5 ± 6.1E-6 cm-sec-1, and 0.76 ± 0.5 (N = 6). No previous values of these coefficients could be found for HBL-100 cells.
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  • 18
    Electronic Resource
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    Springer
    Catalysis letters 64 (2000), S. 65-75 
    ISSN: 1572-879X
    Keywords: NO reduction ; CH3OH ; La2O3 ; methyl nitrite ; kinetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Notes: Abstract Nitric oxide (NO) reduction by methanol was studied over La2O3 in the presence and absence of oxygen. In the absence of O2, CH3OH reduced NO to both N2O and N2, with selectivity to dinitrogen formation decreasing from around 85% at 623 K to 50–70% at 723 K. With 1% O2 in the feed, rates were 4–8 times higher, but the selectivity to N2 dropped from 50% at 623 K to 10% at 723 K. The specific activities with La2O3 for this reaction were higher than those for other reductants; for example, at 773 K with hydrogen a specific activity of 35 μmol NO/s m2 was obtained whereas that for methanol was 600 μmol NO/s m2. The Arrhenius plots were linear under differential reaction conditions, and the apparent activation energy was consistently near 14 kcal/mol with CH3OH. Linear partial pressure dependencies based on a power rate law were obtained and showed a near‐zero order in CH3OH and a near‐first order in H2. In the absence of O2, a Langmuir–Hinshelwood type model assuming a surface reaction between adsorbed CH3OH and adsorbed NO as the slow step satisfactorily fitted the data, and the model invoking two types of sites provided the best fit and gave thermodynamically consistent rate constants. In the presence of O2 a homogeneous gas‐phase reaction between O2, NO, and CH3OH occurred to yield methyl nitrite. This reaction converted more than 30% of the methanol at 300 K and continued to occur up to temperatures where methanol was fully oxidized. Quantitative kinetic studies of the heterogeneous reaction with O2 present were significantly complicated by this homogeneous reaction.
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  • 19
    Electronic Resource
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    Springer
    Catalysis letters 69 (2000), S. 103-107 
    ISSN: 1572-879X
    Keywords: dicyclopentadiene ; Wacker oxidation ; Pd(AcO)2 ; benzoquinone ; kinetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Notes: Abstract The oxidation of dicyclopentadiene catalyzed by palladium(II) acetate and benzoquinone in the presence of perchloric acid was studied. Tricyclodecenone in high selectivity (85–98%) at a conversion of dicyclopentadiene up to 76% was obtained. The kinetic model assumed the significant inhibition complexation between dicyclopentadiene and tricyclodecenone with the catalytic species.
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  • 20
    ISSN: 1569-8041
    Keywords: biological/pathological characteristics ; breast cancer ; prognosis ; progression ; symptomatic/asymptomatic patients
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background:It is well known that mammographic screening reducesbreast cancer mortality. One possible explanation for this effect is thatscreening makes it possible to detect smaller breast cancers with fewerinvolved nodes, but another hypothesis is that some screening-detected tumorsare in a pathologically and biologically different phase of evolution fromthose that are detected clinically. The aim of the present study was tocompare the biological, pathological and clinical characteristics ofsymptomatic vs. asymptomatic breast cancers. Patients and methods:The study considers a series of 1916consecutive patients who underwent surgery for stage I and II infiltratingbreast cancer at Verona hospitals after having undergone ultrasound andmammography (at least one of which was positive). They were divided into twogroups on the basis of why they decided to undergo the imaging examinations:group A refers to the 1247 patients with a palpable lump, and group B to the616 who were asymptomatic. Results:The patients in group A were older, and had larger tumorsand a higher percentage of positive nodes than those in group B; they also hadsignificantly higher grade tumors, higher Ki-67 levels, and a higherpercentage of ER and PgR negative and c-erbB-2 positive tumors (allof the P-values were significant). A logistic regression analysisadjusted for tumor diameter and age showed a reduction in the significance ofeach of the considered variables, but all of them remained significantlyassociated with the modality of diagnosis except ER, PgR andc-erbB-2. Conclusions:Our results suggest that asymptomatic tumors arebiologically different from their clinically presenting counterparts, thusconfirming the hypothesis that progression towards greater malignancy mayoccur during the natural history of breast cancer.
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  • 21
    ISSN: 1569-8041
    Keywords: 4-OH-IF ; breast cancer ; drug combination ; human cell lines ; primary cultures ; VNB
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background:Vinorelbine and ifosfamide are active drugs againstbreast cancer, but the best treatment schedule has yet to be defined bypreclinical or clinical studies. The antitumor activity of4-hydroxy-ifosfamide (4-OH-IF), the active form of ifosfamide, and vinorelbine(VNB) and their interaction were investigated in two established breast cancercell lines (MCF-7 and BRC-230) and in 10 primary breast cancer cultures. Materials and methods:Cytotoxic activity was evaluated by ahighly efficient clonogenic assay (HECA). The median-effect principle wasapplied to evaluate synergistic and antagonistic interactions and thecorresponding combination index values were calculated. Cell cycleperturbations were analysed by flow cytometry. Results:In MCF-7 and BRC-230 cell lines the sequence VNB for 4hours followed by 4-OH-IF for 24 hours produced an antagonistic effect.Conversely, the inverse sequential scheme, 4-OH-IF → VNB providedsynergistic effects on both cell lines. The synergism was associated with astrong block in the G2-M phase. Synergistic activity of 4-OH-IF → VNBsequence was confirmed in 7 of 10 primary breast cancercultures. Conclusions:In conclusion, the sequence 4-OH-IF → VNBappeared to be the most effective scheme both in established cell lines andin primary breast cancer cultures.
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  • 22
    ISSN: 1569-8041
    Keywords: adjuvant treatment ; breast cancer ; tamoxifen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Aim:Immediate adjuvant tamoxifen reduces disease recurrence andimproves survival in patients with early breast cancer. However, is it toolate to administer tamoxifen to patients who have already undergone treatment,but were unable to benefit from this adjuvant therapy? The French NationalCancer Centers (FNCLCC) have investigated the efficacy of delayed tamoxifenadministration in a randomized controlled trial. Patients and methods:From September 1986 to October 1989, womenwith primary breast cancer, who had undergone surgery, radiotherapy, and/orreceived adjuvant chemotherapy but not hormone therapy more than two yearsearlier, were randomized to receive either 30 mg/day tamoxifen or notreatment. The 10-year disease-free and overall survival rates of the twogroups of patients and of various subgroups were determined according to theKaplan–Meyer method and compared by the log-rank test. Results:This intention-to-treat analysis comprised 250 women inthe tamoxifen group and 244 in the control group. Patient characteristics(age, T stage, number of positive nodes, receptor status, and interval sincetumor treatment) were comparable in both groups. Delayed adjuvant tamoxifensignificantly improved overall survival only in node-positive patients and inpatients with estrogen receptor-positive (ER+) or progesteronereceptor-positive (PR+) tumors. Disease-free survival, however, wassignificantly improved in the global population and in several patientsubgroups (node-positive, ER+, PR+). Patients in whom the interval betweenprimary treatment and delayed adjuvant tamoxifen was greater than five yearsalso had significantly improved disease-free survival. Conclusions:Overall and disease-free survival results indicatethat delayed adjuvant tamoxifen administration (30 mg/day) is justified inwomen with early breast cancer, even if this treatment is initiated two ormore years after primary treatment.
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  • 23
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    Springer
    Annals of oncology 11 (2000), S. 1349-1351 
    ISSN: 1569-8041
    Keywords: breast cancer ; radiotherapy ; tumor lysis syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Tumor lysis syndrome (TLS) is a rare serious acute complication of cancertherapy, reported mainly following chemotherapy in patients with large tumorload and chemosensitive disease. These are mainly patients with non-Hodgkin'slymphoma, leukemia and rarely in solid tumors. It is less frequently describedafter radiotherapy for lymphoid and hematological malignancies. TLS followingradiotherapy for solid tumors is a very rare complication. In thisreport/review we describe a seventy-three-year-old male patient withprogressive metastatic carcinoma of the breast to the lungs, liver and bone.He was referred for radiotherapy because of generalized bony pains. Thepatient was planned for sequential hemi-body irradiation starting with themore symptomatic upper half body. After premedication, he was given 8.5 Gy tothe mid point at the maximum chest separation with anterior lung attenuatorlimiting uncorrected lung dose to 6.15 Gy. A further 3.5 Gy electron boost tothe fungating breast tumor was given to the 100%. Forty-eight hours after irradiation he developed hyperkalemia,hyperphosphatemia, hyperuricemia, hypocalcemia and renal failure. Theseclinical and biochemical changes are typical of tumor lysis syndrome (TLS).Despite hydration, and treating the hyperuricemia, the patient developed comaand died eight days after irradiation. The prophylaxis and management of TLS and in high-risk patients aredescribed to avoid this frequently fatal complication.
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  • 24
    ISSN: 1569-8041
    Keywords: breast cancer ; erbB2 ; HER-2/neu ; tamoxifen ; therapy resistance
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Aim:We aimed to study the importance of erbB2 status in earlystage postmenopausal breast cancer for patients who participated in a trialof five vs. two years of adjuvant tamoxifen. Patients and methods:We analysed the erbB2 status of the tumoursfrom 577 patients participating in the trial, either by a DNA amplificationassay (n = 181) or by measurement of the protein level with flowcytometry (n = 396). Results:ErbB2 was overexpressed or gene amplified in 102 of thepatients (18%). Overall, erbB2-positive patients had a significantlylower recurrence-free probability than others, 62% at five years ascompared to 83%, and showed a significantly decreased breast cancersurvival rate (P = 0.0007). ErbB2 status was significantlyassociated with recurrence and death in Cox multivariate analysis, adjustingfor nodal status, tumour size and estrogen receptor status. The relative riskof recurrence (RR) for five vs. two years of tamoxifen was analysed inrelation to erbB2 status for patients still disease-free two years aftersurgery. Whereas erbB2-negative patients showed significant benefit fromprolonged treatment (RR = 0.62, 95% confidence interval (95%CI): 0.42–0.93), no benefit was evident for erbB2-positive patients (RR= 1.1, 95% CI: 0.41–3.2). When the same analysis was restrictedto ER-positive patients a similar difference in relative hazard was obtainedbut the difference was not strictly significant (P = 0.065). Conclusions:For early stage breast cancer patients treated withadjuvant tamoxifen, overexpression of erbB2 is an independent marker of poorprognosis. The results suggest that overexpression decreases the benefit fromprolonged tamoxifen treatment.
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  • 25
    ISSN: 1569-8041
    Keywords: breast cancer ; cyclophosphamide ; elderly ; idarubicin ; oral chemotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 26
    ISSN: 1569-8041
    Keywords: adjuvant therapy ; breast cancer ; docetaxel ; feasibility
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background and purpose:Docetaxel is an active agent in thetreatment of metastatic breast cancer. We evaluated the feasibility ofdocetaxel-based sequential and combination regimens as adjuvant therapies forpatients with node-positive breast cancer. Patients and methods:Three consecutive groups of patients withnode-positive breast cancer or locally-advanced disease, aged ≤70 years,received one of the following regimens: a) sequential A → T → CMF:doxorubicin 75 mg/m2 q 3 weeks × 3, followed by docetaxel 100mg/m2 q 3 weeks × 3, followed by i.v. CMF days 1 + 8 q 4weeks × 3; b) sequential accelerated A → T → CMF: A and T wereadministered at the same doses q 2 weeks; c) combination therapy: doxorubicin50 mg/m2 + docetaxel 75 mg/m2 q 3 weeks × 4,followed by CMF × 4. When indicated, radiotherapy was administeredduring or after CMF, and tamoxifen started after the end of CMF. Results:Seventy-nine patients have been treated. Median age was48 years. A 30% rate of early treatment discontinuation was observedin patients receiving the sequential accelerated therapy (23% duringA → T), due principally to severe skin toxicity. Median relativedose-intensity was 100% in the three treatment arms. The incidence ofG3–G4 major toxicities by treated patients, was as follows: skintoxicity a: 5%; b: 27%; c: 0%; stomatitis a: 20%;b: 20%; c: 3%. The incidence of neutropenic fever was a:30%; b: 13%; c: 48%. After a median follow-up of 18months, no late toxicity has been reported. Conclusions:The accelerated sequential A → T → CMFtreatment is not feasible due to an excess of skin toxicity. The sequentialnon accelerated and the combination regimens are feasible and under evaluationin a phase III trial of adjuvant therapy.
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  • 27
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    Der Nervenarzt 71 (2000), S. 164-172 
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Psychisch kranke Eltern ; Depressive Eltern ; Entwicklungsbedingungen ; Psychosoziale Bedingungen ; Kinder ; Forschungsprobleme ; Forschungsaufgaben ; Forschungskriterien ; Key words Parents with psychiatric disorders ; Depressive parents ; Developmental conditions ; Psychosocial condition ; Children ; Research problems ; Research tasks ; Research criteria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Children of psychiatrically ill parents represent a risk group that has received growing attention during the last years. The risk for this group to develop a psychiatric illness is markedly increased due to genetic and psychosocial factors. The development of effective preventive concepts requires a thorough knowledge of the psychosocial factors. In this paper, deficits and problems of research in psychosocial transmission mechanisms are discussed taking the example of children of depressive parents. Conclusions from this exemplary considerations may serve as guidelines for future research. The authors suggest that the focus be rather placed on coping strategies and developmental psychopathology. Further, research criteria are formulated that refer to theoretical models as well as to study design.
    Notes: Zusammenfassung Kinder psychisch kranker Eltern stellen in psychiatrischer Hinsicht eine Risikogruppe dar, die in den letzten Jahren zunehmend in den Blickpunkt gerückt ist. Die Wahrscheinlichkeit für psychische Erkrankungen ist bei dieser Gruppe aufgrund von genetischen und psychosozialen Faktoren deutlich erhöht. Die Entwicklung von effektiven Präventionskonzepten setzt eine genaue Kenntnis der psychosozialen Faktoren voraus. In der vorliegenden Arbeit werden Defizite und Probleme bei der Erforschung der psychosozialen Transmissionsmechanismen am Beispiel der Kinder von depressiven Eltern diskutiert, und es werden Schlussfolgerungen herausgearbeitet, an denen sich die künftige Forschung orientieren sollte. Es wird vorgeschlagen, bewältigungsorientierte und entwicklungspsychopathologische Konzepte stärker zu berücksichtigen; darüber hinaus werden Forschungskriterien formuliert, die sich sowohl auf die theoretischen Modelle ebenso wie auf die Untersuchungsdesigns beziehen.
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  • 28
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    HNO 48 (2000), S. 828-831 
    ISSN: 1433-0458
    Keywords: Schlüsselwörter Cochlear Implant ; Resthörige Kinder ; Indikation ; Bildung ; Keywords Cochlear implant ; Children ; Indication ; Education ; Residual hearing
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Background and objective. Recent indications for cochlear implant in children are bilateral total cochlear deafness and an age of 2 or more. Reports on successful implantations in adults with residual hearing pose the question of whether this indication might be expanded to children with residual hearing. Patients/Methods. In a retrospective analysis of 106 hearing-impaired children with binaural amplification, we were able to ask parents in 90 cases about their children's education. The pure-tone average of the frequencies of 1–4 kHz was correlated to education. Results. The results showed that all children except one with a pure-tone average of ≤90 dB could successfully attend regular school or kindergarten. However, those with a pure-tone average of ≥91 dB had to be educated in special units for children with impaired hearing. Conclusions. Our results indicate that amplification in children with profound hearing loss (pure-tone average ≥91 dB) is not sufficiently effective to enable them to attend regular schools or kindergarten. We conclude that the only chance to integrate these children into the world of hearing might be cochlear implantation.
    Notes: Zusammenfassung Hintergrund und Fragestellung. Die derzeit gültigen Richtlinien zur Indikation eines “Cochlear Implants” (CI) bei Kindern sind eine beidseitige cochleäre Taubheit sowie ein Lebensalter von über 2 Jahren. Berichte über die guten Hörerfolge nach Implantation von resthörigen, nicht tauben Erwachsenen werfen die Frage nach dieser Indikationserweiterung auch bei Kindern auf. Ziel unserer Arbeit war es, die Effizienz der Hörgeräteversorgung von schwerhörigen Kindern an Hand des Bildungsweges zu beurteilen um daraus Schlüsse zur Indikationserweiterung für ein CI zu ziehen. Patienten/Methodik. In einer retrospektiven Analyse von 106 beidseitig schwerhörigen, mit Hörgeräten versorgten Kindern konnte der Bildungsweg durch Befragung der Eltern in 90 Fällen erhoben werden. Ergebnisse. Durch Ermittlung der mittleren Hörschwelle in den Frequenzen 1–4 kHz und Vergleich mit dem Bildungsweg konnte gezeigt werden, dass nahezu alle Kinder (bis auf eines) mit einer Hörschwelle ≤90 dB Regelschulen oder -kindergärten mit Erfolg besuchen konnten. Alle Kinder mit einer Hörschwelle ≥91 dB mussten spezielle Einrichtungen für Hörbehinderte besuchen. Schlussfolgerungen. Da nach unseren Ergebnissen schwerhörige Kinder mit einer mittleren Hörschwelle ≥91 dB trotz Hörgeräteversorgung nicht in der Lage sind, Regelschulen oder -Kindergärten erfolgreich zu besuchen, schließen wir daraus, dass das Hauptziel einer Versorgung, nämlich der Erwerb einer ausreichenden Sprachkompetenz und -Produktion, nur durch ein CI erreicht werden kann.
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  • 29
    ISSN: 1433-0458
    Keywords: Schlüsselwörter Cochlear Implant ; Ambulante Rehabilitation ; Kinder ; Erwachsene ; Ergebnisse ; Keywords Cochlear implant ; Outpatient rehabilitation ; Cost effectiveness ; Results ; Children ; Adults
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Background and objective. This study compares the results of the outpatient-based program of the Cochlear Implant Center Ruhr with inpatient-based rehabilitation, which is almost exclusively performed in Germany. Patients/methods. The Department of Otorhinolaryngology at the University of Essen in Germany provided 52 patients with either 22- or 24-channel Nucleus cochlear implants from March 1996 to July 1999. Almost all patients (n=49) were rehabilitated on an outpatient basis, which is the standard in many cochlear implant centers outside Germany. Results. The longest follow-up period at the University of Essen Department of Otorhinolaryngology was 36 months. Minor complications occurred in 10% of the patients. After 24 months, the first three implanted patients were able to discriminate 100% of numbers and over 60% of syllables in the Freiburg speech discrimination test. The patients who developed an understanding of open speech were able to discriminate 31 words per minute with cochlear implant and without lipreading after 24 months. Children were seen to double their Schmid-Giovannini scores at 6 months postimplantation. Conclusions. The Essen outpatient-based cochlear implant program demonstrates results in speech development and speech understanding equal to those of centers providing inpatient rehabilitation. A special advantage is continuous rehabilitation with professionals known to the child for several years. In children especially, exhaustive commuting reduces school attendance and is a burden on the accompanying guardians. As an inpatient, however, the child is torn from his familiar environment. Parents with several children have particular difficulties in accompanying their child and indeed this may not always be possible.
    Notes: Zusammenfassung Hintergrund und Fragestellung. In dieser Arbeit werden die Ergebnisse der ambulanten Rehabilitation nach Cochlear-Implant-Versorgung mit denen der stationären Rehabilitation verglichen, die bisher in Deutschland fast ausschließlich durchgeführt wird. Von März 1996 bis Juli 1999 wurden an der Universitäts-Hals-Nasen-Ohren-Klinik Essen 52 taube oder an Taubheit grenzende Patienten mit einem 22-kanaligen bzw. 24-kanaligen Nucleuscochlear-Implant versorgt. Fast alle Patienten (n=49) konnten wohnortnah ambulant rehabilitiert werden, wie dies dem internationalen Standard entspricht. Ergebnisse. Der längste bisherige Nachbeobachtungszeitraum an der Universitäts-Hals-Nasen-Ohren-Klinik Essen sind 36 Monate. Nach 2 Jahren wurden von den 3 am längsten nachbeobachteten Patienten 100% der Zahlen und über 60% der Einsilber im Freiburger Sprachtest verstanden. Im “speech tracking” erreichten Patienten mit CI und ohne Lippenabsehen nach 24 Monaten 31 Wörter/min. Die Kinder zeigten 6 Monate nach Implantation eine Verdopplung des Scores im Test nach Schmid-Giovannini. Schlussfolgerungen. Das Essener Modell zeigt, dass eine ambulante Rehabilitation nach CI zu vergleichbaren Ergebnissen in der Sprachentwicklung und im Sprachverstehen führt, wie sie von anderen Zentren vorgelegt wurden, in denen fast ausschließlich stationär rehabilitiert wird. Besonders bei Kindern bedeuten lange Anfahrtswege mit einwöchigem stätionärem Aufenthalt Schulausfälle und eine Belastung für die begleitenden Eltern und Familienangehörigen zu Hause, sowie erhebliche Fahrtkosten. Stationäre Aufenthalte zur Rehabilitation reißen zudem das Kind aus seiner gewohnten Umgebung und sind für Eltern mit mehreren Kindern oft unmöglich.
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  • 30
    ISSN: 1433-0458
    Keywords: Schlüsselwörter Hörstörung ; Prävalenz ; Konnatale Hörstörungen ; Erworbene Hörstörungen ; Progredienz ; Infektionen ; Kinder ; Keywords Hearing loss ; Prevalence ; Connatal hearing loss ; Acquired hearing loss ; Progressive hearing loss ; Infections ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract The results of international investigations on connatally acquired hearing loss are compared with the data of the German Registry on Childhood Hearing Loss (4058 cases). The connatal hearing disorders have shown a notable change in the last years regarding to aetiology and prevalence. In contrast to countries of the third world in developed nations the prevalence of permanent childhood hearing loss has been reduced down to 1 in 1.000 births. The results let assume a prevalence of approximately 1:1.200 births in Germany. For instance the number of rubella embryopathia decreased effectively. In contrast CMV infections and alcohol fetopathia are playing an increasing role. In the patients of the German Registry on Childhood Hearing Loss the percentage of certainly progressive hearing loss is 10.3 within the 4058 children with permanent hearing impairment. Diagnostic procedures first of all for the early diagnosis of CMV but also of toxoplasmosis are considerable because these infections may result in treatable hearing loss. Also consequent hearing tests are demanded in children with alcohol fetopathia.
    Notes: Zusammenfassung Im vorliegenden Beitrag werden die Daten internationaler Studien zu angeborenen erworbenen Hörstörungen mit den Ergebnissen aus 4058 Fällen im Deutschen Zentralregister für kindliche Hörstörungen (DZH) verglichen und ausgewertet. Die angeborenen Erkrankungen des Hörvermögens haben innerhalb der letzten Jahre bezüglich Ätiologie und Prävalenz einen deutlichen Wandel erlebt. Im Gegensatz zu Ländern der 3. Welt ist die Prävalenz permanenter kindlicher Hörstörungen in den westlichen Industrienationen auf ca. 1:1.000 gesunken. In Deutschland liegt die Prävalenz nach ersten Ergebnissen des DZH bei ca. 1,2:1.000. So ist beispielsweise der Anteil der Rötelnembryopathien stark zurückgegangen. Dagegen spielen heute die Zytomegalievirus-(CMV)-Infektion und die Alkoholfetopathie eine größere Rolle. Im Patientenkollektiv des DZH mit 4058 permanent hörgestörten Kindern beträgt der Anteil gesichert progredienter Verläufe 10,3%. Diagnostische Verfahren, vor allem zur Früherkennung von CMV und Toxoplasmose, gewinnen zunehmend an Bedeutung. Ebenso ist eine konsequente Hördiagnostik auch bei Kindern mit Alkoholfetopathie zu fordern.
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  • 31
    ISSN: 1433-0458
    Keywords: Schlüsselwörter Cochlear Implant ; Kinder ; Eltern ; Psychische Belastung ; Erwartungshaltung ; Keywords Cochlear Implant ; Children ; Parents ; Psychosocial stress ; Expectations
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Established knowledge. It is known that parents of hard-of-hearing children suffer from an increase in psychosocial stress. Scientific question. How does the psychosocial situation of parents with children who have cochlear implants change during rehabilitation? Aim of study. It was the aim of this study to demonstrate how parents evaluate retrospectively their own psychological well-being during the process of rehabilitation. Methods and results. We interviewed 87 parents by questionnaire which were mailed to them. Fifty-seven mothers and 46 fathers responded (59% return rate). Parents reported a significant increase in stress, as perceived by themselves, after the time of diagnosis. Of the parents, 25% continued to suffer from psychic stress during rehabilitation as could be demonstrated by the SCL-90-R questionnaire criteria. The expectations by parents were realistic prior to implantation but thereafter increased significantly with time. Conclusions. The psychological state of parents during the critical phase, after a diagnosis of deafness has been made for their child, has to be considered. Even after an initial phase of shock, parents seemed to be stressed to an extent that required therapeutic intervention.
    Notes: Zusammenfassung Bisheriges Wissen zum Thema. Eine generell erhöhte psychosoziale Belastung der Eltern hörbehinderter Kinder ist aus der Literatur bekannt. Wissenschaftliche Fragestellung. Wie verändert sich die psychosoziale Situation der Eltern von mit einem Cochlear Implant (CI) versorgten Kindern im zeitlichen Verlauf der Rehabilitation? Ziel der Arbeit. Ziel war es darzustellen, wie die Eltern ihr eigenes psychisches Befinden während der Rehabilitation ihres Kindes retrospektiv einschätzen. Methoden und Arbeitsergebnisse. 87 Elternpaare wurden getrennt in einer postalischen Fragebogenerhebung befragt; 57 Mütter und 46 Väter antworteten (59% Rücklauf). Die Eltern gaben ab dem Zeitpunkt der Diagnosestellung eine subjektiv deutlich erhöhte Belastung an. 25% der Eltern waren auch im weiteren Verlauf der Rehabilitation anhand der Beurteilungskriterien des SCL-90-R in einem klinisch relevanten Ausmaß psychisch belastet. Die Erwartung der Eltern vor der Implantation war realistisch und stieg im Verlauf der Rehabilitation statistisch signifikant an. Schlussfolgerungen. Die starke psychische Belastung in der Selbsteinschätzung der Eltern von CI-Kindern in der Phase der Diagnosestellung bestätigt die Theorie und klinische Praxis, dass die Eltern in dieser kritischen Phase besonderer Betreuung bedürfen. Auch über die Phase des initialen Schocks hinaus scheinen die Eltern in einem Ausmaß psychisch belastet zu sein, welches eine gezielte zusätzliche psychologische Betreuung der Eltern erforderlich machen könnte.
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  • 32
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Epidemiologie ; Cholezystolithiasis ; Kinder ; Sonographie ; Risikofaktoren ; Key words Epidemiology ; Cholecystolithiasis ; Children ; Ultrasound ; Risk factors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Objective. To assess the prevalence of and risk factors (e. g., obesity and positive family history) for the development of cholecystolithiasis in a non-selected collective of children and adolescents. Design and participants. A series of 482 children and adolescents aged 6–18 years underwent upper abdominal diagnostic ultrasound examinations as part of a whole-community investigation (response rate: 78%) for Echinococcus multilocularis in a town in southern Germany. Results. Gallbladder stones were identified in three of 482 children, corresponding to a prevalence of 0.6%. None of the three children was overweight at the time of the examination. Additional risk factors (pronounced weight reduction and Wilson's disease) were present in one study subject. Conclusions. Findings of the present study do not confirm the significance of obesity as a risk factor for cholecystolithiasis in children and adolescents.
    Notes: Zusammenfassung Fragestellung. Ziel der Studie war es, Häufigkeit und Risikofaktoren der Cholezystolithiasis, insbesondere Adipositas und familiäre Belastung, an einem unselektierten Kollektiv von Kindern und Jugendlichen zu untersuchen. Methode und Studienkollektiv. Ein Studienkollektiv von 482 Kindern einer Gemeinde in Süddeutschland zwischen 6 und 18 Jahren wurde in einer Vollerhebung (Responserate: 78%) im Rahmen einer Screeninguntersuchung zu Echinococcus multilocularis sonographisch untersucht. Ergebnisse. Bei 3 von 482 Kindern, entsprechend einer Prävalenz von 0,6%, wurden Gallenblasensteine festgestellt. Keines der 3 Kinder war zum Zeitpunkt der Untersuchung übergewichtig. Als weitere Risikofaktoren fanden sich bei einem Studienteilnehmer eine starke Gewichtsreduktion sowie ein M. Wilson. Schlussfolgerungen. Adipositas konnte bei Kindern und Jugendlichen in der vorliegenden Studie nicht als Risikofaktor für die Cholezystolithiasis bestätigt werden.
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  • 33
    ISSN: 1433-044X
    Keywords: Schlüsselwörter Thoraxtrauma ; Atelektasen ; Pädiatrie ; Bauchlage ; Keywords Thoracic trauma ; Atelectasis ; Children ; Prone position
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract We report on the ventilation in prone position in a 5-year-old traumatized child with severe thoracic and abdominal injuries (lung contusion, rib fractures, rupture of liver and spleen). Under continuous analgosedation, the young patient was ventilated in prone position for 6 h, since acute lung injury and atelectasis persisted despite various therapeutic measures (artifical ventilation in the pressure controlled mode, fiberoptic bronchoscopy, reexpansion maneuver). After initiation of the prone position, we observed a rapid increase i narterial oxygenation, which persisted in the following period. The hemodynamic situation remained stable. The complete disappearance of atelectasis was demonstrated radiologically after supine repositioning. After cessation of analgosedation, the extubation was performed 2 days later. Furthermore, we found no side effects of the prone position on the injured abdomen, and the liver function improved rapidly. Although there is a lack of experience with ventilation in prone position in pediatric intensive care, our report might be a recommendation for the indication of this technique in children.
    Notes: Zusammenfassung In dieser Kasuistik wird über die erfolgreiche Anwendung der Beatmung in Bauchlage bei einem 5-jährigen Mädchen berichtet, welches von einem Pkw überrollt worden war und sich Thorax- und Abdominalverletzungen (Rippenserienfraktur, Lungenkontusion, Leber- und Milzeinrisse) zugezogen hatte. Wegen des akuten Lungenversagens mit persistierenden Atelektasen, die durch wiederholte fiberoptische Bronchiallavagen und durch Reexpansionsmanöver nicht zu beheben waren, wurde der Entschluss zur 6-stündigen Lagerung auf den Bauch gefasst, obwohl über den Effekt dieser Lagerungsmaßnahme bei traumatisierten Kindern wenig bekannt ist und zu möglichen negativen Auswirkungen auf das schwerverletzte Abdomen eine Informationen vorliegen. Die Beatmung in Bauchlage führte zur raschen Verbesserung des pulmonalen Gesaustausches, die hämodynamische Situation wurde nicht beeinflusst. Die radiologische Kontrolle nach Rücklagerung zeigte eine vollständigen Rückgang der Atelektasen; die kleine Patientin konnte bald darauf extubiert werden. Weder laborchemisch noch klinisch wurde ein schädigender Einfluss auf das verletzte Abdomen gefunden. Die Beatmung in Bauchlage hat sich als Routineverfahren bei der Behandlung des Lungenversagens des Erwachsenen etabliert; nach der hier beschriebenen Erfahrung ist diese Maßnahme auch bei traumatisierten Kindern in Betracht zu ziehen, insbesondere wenn andere Maßnahmen nicht ausreichend sind.
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  • 34
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    Monatsschrift Kinderheilkunde 148 (2000), S. 113-117 
    ISSN: 1433-0474
    Keywords: Schlüsselwörter ; Kinder ; Säuglinge ; Protonenpumpenhemmer ; Omeprazol ; Pharmakologie ; Gastroösophageale Refluxkrankheit ; Ösophagitis ; Helicobacter-pylori-Infektion ; Ulkus ; Key words ; Children ; Infants ; Omeprazole ; Gastroesophageal reflux disease ; Esophagitis ; Pharmacology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Proton pump inhibitors (omeprazole, lansoprazole, and pantoprazole) have revolutionized the therapy of peptic disease of the upper gastrointestinal tract and reduced the indications for surgical intervention. These substances inhibit the H+/K+-ATPase in the parietal cells with an acid suppressing potency that is much higher than that of H2-receptor-antagonists. The first proton pump inhibitor, omeprazole, has been introduced ten years ago. Since then, sufficient experience with this drug in children has accumulated. Omeprazole is released for children aged one year or older. A new formulation makes the drug applicable in tube fed patients. The main indications for the use of omeprazole are endoscopically verified peptic esophagitis and gastric and duodenal ulcerations of different etiologies. In the treatment of Helicobacter pylori-infection a sufficient acid suppression is essential for the efficacy of the antibiotics. The recommended pediatric dose ranges between 0,7–1,4 mg/kg bodyweight and day. However, some children with ulcerative esophagitis may need daily doses up to 3.5 mg/kg. Healing of the esophageal mucosa should be monitored by endoscopy. After remission, doses can often be reduced for maintenance therapy. Side effects are rare and do not seem to increase with higher doses. Possibly interactions with other medications, i.e. antiepileptic drugs, may occur. A long-term therapy lasting more than 6 months in a child should be monitored by an experienced pediatric gastroenterologist.
    Notes: Zusammenfassung Protonenpumpeninhibitoren (Omeprazol, Lansoprazol, Pantoprazol) haben die Therapie peptischer Erkrankungen im oberen Gastrointestinaltrakt revolutioniert und die Indikation für chirurgische Therapiemaßnahmen deutlich eingeschränkt. Diese Substanzen hemmen die H+-/K+-ATPase in den Parietalzellen und haben eine sehr viel stärkere säuresuppressive Wirkung als H2-Rezeptor-Antagonisten. Mit dem ersten Vertreter dieser Substanzklasse, Omeprazol, liegen nach 10jähriger Anwendung inzwischen ausreichend Erfahrungen bei Kindern vor. Die Substanz ist für Kinder ab dem 1. Lebensjahr zugelassen und in einer neuen Darreichungsform auch bei Sondenernährung anwendbar. Die wichtigsten Indikationen für den Einsatz von Omeprazol sind eine endoskopisch gesicherte peptische Ösophagitis und Ulzerationen verschiedener Genese im Magen und Duodenum. Für eine effektive Therapie der Helicobacter-pylori-Infektion ist die ausreichende Säuresuppression essentiell für die Wirksamkeit der eingesetzten Antibiotika. Die therapeutische Dosis liegt zwischen 0,7 und 1,4 mg/kg KG und Tag, in Einzelfällen werden zur Abheilung einer Refluxösophagitis bis zu 3,5 mg/kg und Tag benötigt. Die Abheilung erosiver und ulzeröser Läsionen muß endoskopisch kontrolliert werden. Für die Erhaltungstherapie nach Abheilung sind niedrigere Dosen angemessen. Bei kurzfristiger Anwendung sind Nebenwirkungen selten und scheinen nicht dosisabhängig zu sein. Potentielle Wechselwirkungen mit anderen Medikamenten, besonders Antikonvulsiva, müssen beachtet werden. Eine Langzeittherapie bei Kindern über Monate oder Jahre muß gut überwacht werden und gehört in die Hand eines damit erfahrenen Kindergastroenterologen.
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  • 35
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    Trauma und Berufskrankheit 2 (2000), S. S136 
    ISSN: 1436-6274
    Keywords: Schlüsselwörter ; Vordere Kreuzbandverletzung ; Kinder ; Rekonstruktion ; Indikation ; Key words ; ACL lesion ; Children ; Reconstruction ; Indications
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract For ACL lesions in children near to the end of the growth phase the treatment can be treated identical to that in adults. For younger children (8–12 years) the treatment must be conservative, combined with controls at short intervals. Behaviour during sport should be modified. If instability is experienced in activities of daily life or during sport a reconstruction of the ACL should be done using a central tibial transepiphyseal hamstring reconstruction with a femoral over-the-top position to avoid possible growth disturbances.
    Notes: Zusammenfassung Kreuzbandverletzungen bei Kindern, die nahe ihres Wachstumsabschlusses sind (14. bis 17. Lebensjahr) können wie Kreuzbandverletzungen von Erwachsenen behandelt werden. Kreuzbandverletzungen bei Kindern, die 13 Jahre und jünger sind, sollten zunächst abwartend behandelt werden (Kniebandage, Koordinationstraining, Belastungsreduzierung). Stellt sich im täglichen Leben oder bei sportlicher Belastung eine Instabilität im Sinne eines Giving way heraus, so sollte das Kreuzband rekonstruiert werden. Dabei ist es nach allen vorliegenden Daten unbedenklich, die Tibia zentral mit einem 8-mm-Bohrloch zu durchbohren und ein ligamentäres Transplantat durchzuziehen. Femoral ist es aus Sicherheitsgründen eher sinnvoll, statt einer transossären Bohrung die Over-the-top-Position zu wählen. Auch andere, rein epiphysäre Verankerungsmethoden (Semitendinosusplastik mit transossärer Drahtfixation) sind möglich.
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  • 36
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    Trauma und Berufskrankheit 2 (2000), S. S138 
    ISSN: 1436-6274
    Keywords: Schlüsselwörter ; Vorderes Kreuzband ; Kind ; Wachstumsfuge ; Fehlwachstum ; Key words ; Anterior cruciate ligament ; Children ; Growth plate ; Growth disturbance
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Lesions of the anterior and posterior cruciate ligaments are relatively rare in childhood and adolescence; in this age group they need even more consistent and aggressive treatment than in adults as far as conservation of the menisci and definitive stabilization are concerned. Virtually no cases in which serious growth disturbance has arisen following transepiphyseal drilling are known from the literature. Thus, cruciate ligament suturing should also not be done in children, because the long-term efficacy has not been confirmed. The operative procedure is presented in detail and is related to bone age. Early transepiphyseal drilling is permissible.
    Notes: Zusammenfassung Ligamentäre Kreuzbandverletzung beim Kind und im Adoleszentenalter sind relativ selten, sie bedürfen im Vergleich zum Erwachsenen ¶einer noch konsequenteren und aggressiven Behandlung bezüglich Meniskuserhalt und definitiver Stabilisierung. In der Literatur sind praktisch keine Fälle bekannt, bei denen es tatsächlich zu einem gravierenden Fehlwachstum nach transepiphysärer Bohrung gekommen ist. Insofern sollte auch die Kreuzbandnaht beim Kind wegen der nicht bewiesenen Langzeiteffizienz unterlassen werden. Das Vorgehen im Einzelnen in Bezug zum Skelettalter wird dargestellt. Frühzeitige transepiphysäre Bohrungen sind erlaubt.
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    Clinical and experimental nephrology 4 (2000), S. 318-322 
    ISSN: 1437-7799
    Keywords: Key words IgA nephropathy ; Nephrotic syndrome ; Children ; Age at onset
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background. The prognostic significance of nephrotic syndrome (NS) in children with IgA nephropathy (IgAN) is unclear. Methods. NS was found in eight children with IgAN (mean onset age, 9.3 years). The clinicopathological findings of these eight children were investigated. Results. Five patients presented with macroscopic hematuria, while the remaining three were discovered in a school urinary screening program or by chance urinalysis. Six patients developed NS at the onset, and two developed NS later in the course of IgAN. All patients were treated with corticosteroids. At the end of follow-up, heavy proteinuria persisted in four children, one of whom had renal dysfunction at the onset of NS and developed end-stage renal failure, and two of whom developed NS after the onset of IgAN. Proteinuria decreased to less than 1 g/day 3 months after NS in four patients, two of whom showed disappearance of proteinuria afterward. Renal biopsy specimens revealed mesangial proliferation and crescent formation in all patients. The degree of persisting proteinuria was correlated with the presence of glomerular sclerosis, fibrous crescents, tubulo-interstitial changes on light microscopy, and depositions of C3 on immunofluorescence microscopy. Conclusions. Children who developed NS after the onset of IgAN developed renal dysfunction; the prognosis of those who showed chronic histopathological changes on renal biopsy specimens was poor, even in these young children.
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  • 38
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    Pediatric surgery international 16 (2000), S. 589-591 
    ISSN: 1437-9813
    Keywords: Key words Domestic animals ; Children ; Injury ; Evaluation ; Prevention
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Domestic animals are a potential cause of serious injury to handlers and children. In developed countries dogs are the most common cause of such injuries, but in developing countries childhood injuries from domestic animals have not been given much attention. A retrospective analysis of major injuries from domestic animal attacks in children aged 12 years or less in a developing country showed 17 injuries in 16 children. The locations were the abdomen (6), head and neck (4), extremities (3), external genitalia (3), and back (1). A big-horned cow was the cause of injury in 11 patients, a donkey in 4 and a ram in 1. Children under 10 years were injured by provoked animals, mostly as bystanders, while older children were handlers. Four abdominal injuries were penetrating with evisceration and contusion of bowel and required a laparotomy. One blunt splenic injury was managed non operatively and one by partial splenectomy. Two head injuries were managed non operatively. All other injuries were examined and explored with the child under general anaesthesia. These injuries were debrided and closed primarily or closure was delayed when contamination was heavy or adequate debridement was limited by vital neighbouring structures. Broad-spectrum antibiotics and tetanus prophylaxis were given for all open wounds. The average duration of hospital stay was 7.3 days. Three patients had wound infections resulting in long hospitalizations. Two patients died (12.5%), 1 from a severe head injury and 1 from an overwhelming infection. The management of major injuries from domestic animals in children requires meticulous evaluation and examination during general anaesthesia to establish the extent of injury and avoid missed injuries. Prevention of these injuries requires public education about the proper and compassionate handling of domestic animals.
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  • 39
    ISSN: 1437-9813
    Keywords: Key words Insulinoma ; Arterial stimulation ; Calcium ; Localization ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The role of pre- and intraoperative procedures for the localization of insulinomas has been extensively debated. We report a case of successful treatment using preoperative selective intra-arterial calcium injection and intraoperative glucose monitoring. A 12-year-old boy with hypoglycemic attacks had a large insulinoma in the head of the pancreas on computed tomography. Preoperative selective angiography combined with arterial stimulation-venous sampling (ASVS) by intra-arterial injection of calcium revealed no other insulinomas in the body and tail of the pancreas. Elevation of serum glucose on intraoperative monitoring confirmed complete enucleation of the insulinoma. Preoperative ASVS can accurately localize an insulinoma, and may help to increase the success rate of surgery and avoid blind pancreatectomy.
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  • 40
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    Pediatric surgery international 16 (2000), S. 595-598 
    ISSN: 1437-9813
    Keywords: Key words Pleuropulmonary blastoma ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Pleuropulmonary blastoma (PPB) is a rare malignant neoplasm affecting children. A retrospective review was carried out of patients diagnosed as having PPB at one institute over a period of 16 years. The presentation, diagnosis, treatment, and family history were studied. PPB usually presents with symptoms and signs of respiratory tract infection, and the diagnosis may be delayed. There are no distinguishing clinical features or imaging studies. The diagnosis is made on histologic examination of tumour material and is sometimes difficult to differentiate from benign cystic lung lesions. The treatment is primarily complete excision of the tumour, followed in some cases by intense chemotherapy. PPB is a strong predictor of the presence of tumours in close relatives. Four patients treated at our institute are discussed along with a review of the literature.
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  • 41
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    Pediatric surgery international 16 (2000), S. 132-133 
    ISSN: 1437-9813
    Keywords: Key words Sigmoid volvulus ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Sigmoid volvulus is a common cause of large-bowel obstruction in elderly individuals, but is quite rare in childhood. We report six cases in patients under 20 years of age. One had Hirschprung's disease. Gangrenous sigmoid colon was found in three cases and resection was performed. Sigmoidopexy (one case) and extraperitonealization (two cases) were performed for viable sigmoid colon. There were no recurrences after 5.7 years of follow-up.
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  • 42
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    Keywords: Key words Echinococcosis ; Liver/interventional procedure ; Cyst/percutaneous drainage ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To evaluate the efficacy of ultrasound (US) guided percutaneous sclerotherapy in the pediatric population, 14 hydatid liver cysts (HLC) in eight male patients whose ages ranged between 6 and 16 years (mean 9.9 years) were treated. The maximum diameter was 110 mm. Albendazole was administered orally to all patients for 1 week before percutaneous treatment and for 3–6 months after the procedure to prevent dissemination of the disease. Cyst puncture was performed with 20 G Chiba needles using US guidance. More than one-half of the estimated cyst volume was aspirated, then 20% hypertonic saline (7 cysts) or sterile 96% alcohol (7 cysts) equivalent to one-third of the estimated cyst volume was injected into the cavity and left for 5–15 min. Finally, all the fluid in the cavity was reaspirated. Catheterization was not performed. Follow-up US examinations were performed every month during the first 6 months and every 3 months thereafter. The follow-up period ranged between 6 and 51 months (mean 15 months). No major complications were seen during or after the procedures. Two cysts in two patients completely disappeared. Volumes of the 11 cysts in five patients who were followed for 6–21 months were markedly reduced (22%–64% of the initial volume) and thick septations and solid debris-like structures were seen within the cyst cavities. There was no significant change in 1 cyst. US-guided percutaneous sclerotherapy is thus a safe and effective treatment of HLC in children.
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    Pediatric surgery international 16 (2000), S. 388-391 
    ISSN: 1437-9813
    Keywords: Key words Anorectal injury ; Children ; Diagnosis ; Morbidity ; Mortality
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Anorectal injuries (ARI) are uncommon in children in civil practice. In developed countries the injuries are mainly due to sexual abuse and firearms. This report reviews the experience in tropical Africa. A retrospective study of children aged 12 years or less managed for ARI over 10 years was undertaken. There were seven children, four girls and three boys. Four injuries were due to blunt trauma and three to penetrating trauma. Six patients presented within 6 h of injury and one after 24 h. Five had rectal bleeding, which was associated with vaginal bleeding in one girl. One girl each had vaginal bleeding and vaginal discharge without rectal bleeding. Diagnosis was by rectal examination and proctoscopy. In three patients a laparotomy was necessary to exclude an intraperitoneal rectal injury (IRI); this was positive in one case. One patient with abdominal findings had a laparotomy as the primary procedure. Overall, five patients had rectal injuries (extraperitoneal 3, intraperitoneal 2), which were associated with an anal injury in three while one patient had only an anal injury. An IRI was missed at initial assessment in one girl. Associated injuries were to the vaginal wall (3), urethra (1) and head (1). IRIs were treated by repair and proximal colostomy. Extraperitoneal injuries were treated by colostomy and drainage; in two patients the injuries were accessible and were repaired. Anal and external-sphincter injuries were repaired in two cases. Vaginal lacerations were repaired and other associated injuries treated accordingly. Three patients had wound infections. Faecal continence was maintained in all patients who had anal and external-sphincter injuries. One girl died of peritonitis from a missed IRI. It is concluded that ARI remains uncommon in children. Morbidity and mortality can, however, be high. Meticulous rectal palpation and visualisation is necessary to avoid missing injuries.
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  • 44
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    Pediatric surgery international 16 (2000), S. 411-413 
    ISSN: 1437-9813
    Keywords: Key words Central venous catheters ; Children ; Ultrasound assessment of venous patency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To investigate the value of Doppler ultrasound scan (USS) assessment of internal jugular vein (IJV) patency after previous open central-venous cannulation (CVC), a prospective study of 66 consecutive children (median age 4.5 years; range 4 months–17 years) who had previously undergone open insertion of at least one indwelling IJV line and required further CVC for completion of therapy was undertaken. All underwent Doppler USS examination prior to surgery. Where patency of the previously cannulated vein was suggested ultrasonographically, the accuracy of this finding was confined at open surgical exploration. Initial CVCs were in situ for a median of 9 months (1 month–4 years) prior to removal. The median interval to repeated CVC was 11 months (3 weeks–45 months). In 79 Doppler USS, 70 (88.6%) veins appeared patent, 3 (4.2%) stenosed, and 6 (7.6%) obliterated. Of the 70 “USS patent” veins, 66 were explored. Patency was confirmed surgically in 59 (89.4%) and a new CVC successfully inserted. Seven (10.6%) apparently patent veins on USS were found to be obliterated at open exploration. Review of USS images in these cases suggested that enlarged collateral veins were usually responsible. Overall, successful recannulation was possible in 74.6% of all previously accessed veins. In children requiring repeated CVC, Doppler USS of neck veins is a valuable but not entirely reliable guide to the presence of underlying vessel patency and should be interpreted with caution. At least three-fourths of previously cannulated IJVs remain patent after catheter removal and can be reused for CVC.
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    Pediatric surgery international 16 (2000), S. 429-430 
    ISSN: 1437-9813
    Keywords: Key words Amebic liver abscess ; Children ; Pediatric ; Stomach ; Rupture
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The occurrence of an amebic liver abscess (ALA) rupturing into the stomach is reported. ALAs in children can have atypical presentations, resulting in delayed diagnosis and increased morbidity and mortality. Timely treatment is usually followed by complete recovery.
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  • 46
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    Pediatric surgery international 16 (2000), S. 433-436 
    ISSN: 1437-9813
    Keywords: Key words Hernia ; Spigelian hernia ; Children ; Cryptorchidism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Congenital spigelian hernia (SH) is very rare in the pediatric age group. This is a report of two cases of SH in 1-week and 3-month-old male infants. A review of the literature revealed only 35 cases of SH in children younger than 17 years of age, bringing the total including our 2 cases to 37. There were 25 males and 12 females, a ratio of 2.1:1. Their ages ranged from 6 days to 17 years (mean 4.52 years). The hernia was situated on the right side in 13, the left side in 19, and was bilateral in 4. In one case the side of the hernia was not mentioned. In 29 cases the hernia was spontaneous while in 5 it was caused by trauma. In 3 children the hernia developed postoperatively, in 2 following repair of a congenital diaphragmatic hernia and in 1 following excision of a mediastinal neuroblastoma. Two children presented with a strangulated SH. Eleven of the 35 previously reported children had associated conditions; in 5 there was an ipsilateral undescended testis (UDT). Our two infants with SH also had an ipsilateral UDT. The significance of this association is discussed.
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    Pediatric surgery international 16 (2000), S. 454-456 
    ISSN: 1437-9813
    Keywords: Key words Priapism ; Children ; Arteries ; Fistula ; Embolisation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Priapism is an uncommon problem in childhood. Most of the reported cases are in boys with sickle-cell disease or leukaemia. It occurs as a result of venous outflow obstruction, resulting in engorgement of the corpora cavernosa, and is termed “low-flow” priapism. In a small group of children priapism is due to uncontrolled arterial inflow, usually as a result of direct trauma. The authors report a case of post-traumatic arterial priapism in a child, successfully treated with selective embolisation of the internal pudendal artery. Recognition of this distinct entity is important, as it carries a good prognosis when appropriately treated.
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  • 48
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    Clinical and experimental nephrology 4 (2000), S. 225-230 
    ISSN: 1437-7799
    Keywords: Key words Peritoneal dialysis ; Dialysis index ; Urea kinetics ; Adequacy ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background. Peritoneal dialysis (PD) is an established treatment for children with end-stage renal failure. Creatinine clearance and urea kinetics are used to quantitate the dialysis treatment, but the means to assess the adequacy of dialysis in children are still controversial. Methods. We studied serum chemistry, dietary protein intake (DPI), protein catabolic rate (PCR), weekly urea clearance/body water (Kt/Vurea), weekly creatinine clearance (Ccr/week), clinical signs and symptoms during PD treatment, and peritoneal transport function in 17 children (4 to 18 years of age) with end-stage renal disease treated with PD. Fourteen children were on continuous ambulatory peritoneal dialysis (CAPD) and 3 were on automated peritoneal dialysis. Results. The mean values of the parameters tested were: blood urea nitrogen, 71 mg/dl; creatinine, 9.8 mg/dl; total protein, 6.4 g/dl; albumin, 4.0 g/dl; total Ccr, 70 l/week per 1.73 m2; DPI, 1.76 g/kg per day; PCR, 1.17 g/kg per day, and total Kt/Vurea, 2.28/week. The mean patient's clinical assessment score was 11.7, out of 15 and the mean doctor's clinical assessment score was 11.7, out of 14. The correlation between Kt/Vurea and creatinine clearance was 0.84 (P 〈 0.0001). Kt/Vurea and clinical assessment scores (patient's and doctor's scores) did not show a good correlation (r = 0.32; P = 0.228, and r = 0.47; P = 0.064, respectively). Peritoneal function seemed to be preserved after an average duration of 32 months on PD. Conclusions. These patients appeared to be fairly well dialyzed, judging from the values for the various dialysis indices obtained in this study and comparing them with adult indices.
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    Pediatric surgery international 16 (2000), S. 562-564 
    ISSN: 1437-9813
    Keywords: Key words Inguinal hernia ; Children ; Direct ; Recurrence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Among 109 children treated laparoscopically for inguinal hernias, 5 had direct hernias, more than would normally be anticipated. Two of the hernias were recurrences of indirect hernias operated upon previously using the open technique. Direct hernias are easier to detect with the laparoscopic technique.
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    Pediatric surgery international 16 (2000), S. 50-52 
    ISSN: 1437-9813
    Keywords: Key words Intestinal volvulus ; Children ; Idiopathic ; Adhesion/bands ; Resection rate ; Outcome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  In developed countries, intestinal volvulus in children is most frequently due to malrotation. To review the experience in Nigeria, a retrospective analysis of 28 patients managed over 25 years at the Ahmadu Bello University Teaching Hospital, Zaria, Nigeria, was undertaken. There were 22 boys and 6 girls with an age range of 4 days to 14 years (median 4 years). There were equal numbers over and less than 5 years of age. Vomiting (89%) and abdominal distension (79%) were the most prominent features. Thirteen children (46%) had fever, associated with bowel gangrene in 5, while 8 (29%) presented with severe dehydration and shock. A plain abdominal radiograph was the only investigation performed, but the features were not specific for volvulus. In 11 children (39%) the volvulus was idiopathic, in 9 (32%) due to adhesions or bands, in 5 (18%) to malrotation, and in 1 each a Meckel's diverticulum, internal herniation, and ventriculoperitoneal shunt. Twenty-three patients had a small-bowel, 4 sigmoid, and 1 caecal volvulus. The bowel resection rate for gangrene was 46% (small bowel 9, sigmoid 3, caecum 1). All patients with malrotation had Ladd's procedure performed. Wound infections occurred in 10 patients (36%), complete wound dehiscence in 1, and recurrence in 1 (idiopathic terminal ileal volvulus). The mortality was 21%, mostly from overwhelming infection (2 neonates, 11-year-old, 3 ≥ 5 years). Intestinal volvulus in our environment differs in aetiology from other reports. The resection rates are similar, however. This condition carries high morbidity and mortality.
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    Pediatric surgery international 16 (2000), S. 35-39 
    ISSN: 1437-9813
    Keywords: Key words Pancreatic trauma ; Duodenal trauma ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Diagnosis of duodenal and pancreatic injuries is frequently delayed, and optimal treatment is often controversial. Fourteen children with duodenal and/or pancreatic injuries secondary to blunt trauma were treated between 1980 and 1997. The pancreas was injured in all but 1 child. An associated duodenal injury was present in 4. The preoperative diagnosis was suspected in only 6 patients based on clinical signs and ultrasonography. One patient was treated successfully conservatively; all the others required surgical management. At operation, three procedures were used: peripancreatic drainage, suture of the gland or duodenum with drainage, and primary distal pancreatic resection without splenectomy. A duodenal resection with reconstruction by duodeno-duodenostomy was performed in 1 case. The overall complication rate was 14%: 1 fistula and 1 pseudocyst. Pancreatic ductal transection was recognized 3 days after the initial laparotomy by endoscopic retrograde cholangiopancreatography (ERCP). The mortality was 7%; 1 patient died from septic and neurologic complications. When the diagnosis of pancreatic ductal injuries is a major problem, ERCP may be a useful diagnostic procedure. Pancreatic injuries without a transected duct may often be treated conservatively. The surgical or conservative management of duodenal hematomas is still controversial; other duodenal injuries often need surgical treatment.
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    Archives of orthopaedic and trauma surgery 120 (2000), S. 336-337 
    ISSN: 1434-3916
    Keywords: Key words Pulled elbow ; Children ; Trauma ; Immobilization ; Nursemaid’s elbow
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To evaluate the effectiveness in decreasing recurrence of cast application after manual reduction of pulled elbow. Sixty-four children with pulled elbow were randomized into two treatment groups: Group A underwent manipulative reduction followed by splinting the elbow in a flexed and supinated position for 2 days; group B underwent manipulative reduction only. Both groups were examined 2, 5, and 10 days later. None of the 33 patients in group A had a pulled elbow at follow-up. Four (13%) of 31 patients in group B had a pulled elbow 2–5 days later. Immobilizing the elbow for 2 days after manipulative reduction improves the success of treatment of a pulled elbow.
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    Reading and writing 13 (2000), S. 81-103 
    ISSN: 1573-0905
    Keywords: Children ; Reading comprehension ; Working memory
    Source: Springer Online Journal Archives 1860-2000
    Topics: Education
    Notes: Abstract Working memory capacity is described as a pool of limited resources that carry out processing and storage functions. Its role has been emphasised in adults' reading comprehension. The present study had two aims: First, to study the relationship between working memory capacity and reading comprehension in fourth-grade children. Second, to study the nature of the working memory resources involved in reading comprehension, i.e., are they specific or general?To test the first point, the predictive power of working memory capacity was compared with two reading-related basic skills, vocabulary and decoding skills. To test the second point, different working memory tasks were devised using verbal, numerical and spatial materials. All the tasks were administered to 48 fourth-grade children. The results showed that working memory capacity was a direct predictor of reading comprehension when contrasted with vocabulary and decoding skills. Moreover, it seemed that working memory would be better described as a system specialised for the processing of symbolic information in that only the verbal and in a lesser extent the numerical working memory tasks were significant predictors of reading comprehension. The spatial task did not correlate with reading comprehension. The reasons accounting for the predictive power of working memory tasks were discussed.
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    Der Anaesthesist 49 (2000), S. 275-278 
    ISSN: 1432-055X
    Keywords: Schlüsselwörter Tropisetron ; postoperatives Erbrechen ; Adenotonsillektomie ; Antiemetika ; Kinder ; Key words Tropisetron ; PONV ; Adenotonsillectomy ; Antiemetics ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Background: Postoperative nausea and vomiting (PONV) after tonsillectomy is a common problem in children. Tropisetron is a new 5HT3 receptor antagonist and is successfully used in paediatric patients receiving cancer therapy. The aim of the study was to assess efficacy and safety of a single intravenous dose of tropisetron for prevention of PONV in paediatric patients at risk for postoperative vomiting. Methods: In a randomised, double-blind, placebo-controlled trial, we studied 98 children aged 2–12 years undergoing tonsillectomy or adenotonsillectomy. Patients received placebo or tropisetron 0.1 mg (=0.1 ml)/kg body weight immediately after induction of anesthesia. A standard general anesthetic technique (Sevoflurane/N2O/O2 without neuromuscular blockers or opioids) was used. Perioperative vital signs, grade of sedation and episodes of postoperative nausea and vomiting were recorded. Results: No vomiting episodes occurred in 65.3% of the tropisetron treated patients compared to 34.7% of the placebo group (p=0.0024). Only 10.2% of the tropisetron treated patients vomited more than 3 times compared to 22.4% of the control patients (p=0.0004). The need for antiemetic rescue medication was significantly lower in the study group (10.4%) compared to 28.6% (p=0.025). No significant adverse effects of the study medication were shown. Conclusion: A single intravenous prophylactic dose of tropisetron effectively reduces the incidence of PONV during the first 24 postoperative hours after tonsillectomy and/or adenoidectomy. Because of the low incidence of adverse effects, the prophylactic use of tropisetron seems to be safe and justified in paediatric surgical patients at high risk for postoperative vomiting.
    Notes: Zusammenfassung Fragestellung: Erbrechen und Übelkeit nach Tonsillektomien bei Kindern sind ein häufiges Problem. Aufgrund der positiven Erfahrungen mit Tropisetron, einem neueren 5HT3 Rezeptor-Antagonisten bei Chemotherapien in der pädiatrischen Onkologie und mit anderen 5HT3 Rezeptor-Antagonisten in der Kinderchirurgie, prüften wir die Wirksamkeit und Sicherheit einer Einzeldosis Tropisetron zur Prävention von postoperativem Erbrechen bei chirurgischen Kindern mit erhöhtem Risiko für postoperatives Erbrechen. Methodik: Bei 98 Kindern im Alter von 2– 12 Jahren, die eine Tonsillektomie oder Adenotonsillektomie benötigten, führten wir eine randomisierte, doppelblinde, plazebokontrollierte Studie durch. Die Patienten erhielten Tropisetron oder Plazebo in einer Dosis von 0,1 mg (=0,1 ml)/kg KG i.v. unmittelbar nach der Narkoseeinleitung. Die Narkose erfolgte standardisiert mit Sevofluran/N2O/O2 ohne Einsatz von Opioiden und Muskelrelaxanzien. Vitalparameter, Sedationstiefe, das Auftreten von postoperativem Erbrechen und unerwünschte Wirkungen wurden aufgezeichnet. Ergebnisse: In der Tropisetrongruppe zeigten 65,3% der Kinder kein postoperatives Erbrechen, im Gegensatz zu nur 34,7% der Kinder in der Plazebogruppe (P=0,0024). Mehr als 3 Episoden von postoperativem Erbrechen zeigten nur 10,2% der Patienten in der Tropisetrongruppe im Vergleich zu 22,4% der Patienten der Kontrollgruppe (P=0,0004). Auch der Bedarf an antiemetischer Zusatzmedikation war in der Tropisetrongruppe mit 10,4% signifikant niedriger als in der Kontrollgruppe mit 28,6% (P=0,025). Bedeutsame Nebenwirkungen der Studienmedikation konnten nicht dokumentiert werden. Schlussfolgerungen: Eine prophylaktische intravenöse Einzelgabe von Tropisetron reduziert bei Kindern wirksam das Auftreten von postoperativem Erbrechen während der ersten 24 h nach einer Tonsillektomie oder Adenotonsillektomie. Die geringe Inzidenz von Nebenwirkungen rechtfertigt unserer Ansicht nach die prophylaktische Anwendung von Tropisetron bei Kindern nach Tonsillektomien.
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  • 55
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    The journal of headache and pain 1 (2000), S. S153 
    ISSN: 1129-2377
    Keywords: Key words Migraine ; Genetics ; Dopamine ; Hypersensitivity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Migraine is a common chronic disorder with an etiology still mostly unknown. Several neurotransmitters such as dopamine and serotonin are considered to be involved in the pathogenesis of the disease and the study of their systems is crucial in the understanding of migraine. Dopaminergic receptors are variously represented in human CNS and periphery. The hypothesis that a hypersensitivity of the dopaminergic system may have a role in migraine is based on clinical and genetic data. Genetic data are represented by association studies using dopaminergic genes as candidate genes which show that the D2 receptor gene appears to be involved in the pathogenesis of migraine.
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  • 56
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    The journal of headache and pain 1 (2000), S. S141 
    ISSN: 1129-2377
    Keywords: Key words Migraine ; Genetics ; Human leukocyte antigens ; Heredity ; Susceptibility
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Human leukocyte Antigens (HLA) are encoded by genes located on chromosome 6p21. Genes important in migraine are being recognized in two basic ways: association studies and linkage analysis. One of the strongest associations is with the HLA region. Actually, genome scan studies suggest that multiple genes are involved in both migraine without aura (MWoA) and migraine with aura (MWA). However, both MWoA and MWA are disorders in which multiple factors, including environmental and genetic factors, confer disease susceptibility. Linkage analysis is identifying new candidate genes that will help to explain the etiology of migraine. In this review previous studies regarding genetic susceptibility to migraine are analyzed, particularly those related to the HLA region. I discuss evidence that HLA shared-hyplotypes in MWoA-affected pairs in different than that expected, that HLA-DR2 antigen provides additional basis for the proposed genetic heterogeneity between MWoA and MWA, and lastly that TNFB gene studies seem to play an important role in the susceptibility to MWoA. In the past years, major advances hae been made in understanding the genetic foundation of MWoA and MWA. Our reported genome-scan studies support the concept that MWoA/MWA are coinherited with a particular HLA region. However, the examination of candidate genes (Ca2+ channel, vascular, CNS, etc.) in a large migraine population seems to be the correct direction in which we have to move. More MWoA/MWa gene studies are needed to test this developing hypothesis and to further establish the complete genetic scenario of migraine.
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  • 57
    ISSN: 1364-6753
    Keywords: Key words Parkinson's disease ; Familial Parkinson's disease ; Synuclein ; Parkin ; Genetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: ABSTRACT¶Parkinson's disease (PD) is a neurodegenerative disease with clinical features resulting from deficiency of dopamine in the nigrostriatal system. Most PD cases are sporadic and the primary cause of the disease is still unknown. Recently, familial PD and parkinsonism have received much attention because these forms of the disease might provide clues to the genetic risk factors involved in the pathogenesis of idiopathic PD. To date, two causative genes, α-synuclein and the parkin gene, have been identified. α-Synuclein is involved in the pathogenesis of an autosomal dominant form of PD and constitutes a major component of the Lewy body, which is a pathological hallmark of idiopathic PD. In addition, mutations in the parkin gene have been identified as the cause of autosomal recessive juvenile parkinsonism (AR-JP). AR-JP manifests itself as a highly selective degeneration of the substantia nigra and the locus coeruleus, but without Lewy body formation. In addition to these two genes, four chromosomal loci have been linked to other forms of familial PD. Furthermore, there are a number of other pedigrees of familial PD in which linkage to known genetic loci has been excluded. Molecular cloning of these disease genes and elucidation of the function of their gene products will greatly contribute to our understanding of the pathogenesis of idiopathic PD.
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  • 58
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    Accreditation and quality assurance 5 (2000), S. 409-413 
    ISSN: 1432-0517
    Keywords: Key words Metrology ; Comparisons ; Chemistry ; Standards
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Notes: Abstract  After stressing the importance in the modern world of accurate and reproducible measurements, the actions taken by the Bureau International des Poids et Mesures to set up, together with the regional metrology organizations, a series of key comparisons are described. They are the technical foundation of a mutual recognition of national measurement standards arrangement prepared in conjunction with the National Metrology Institutes (NMIs). This arrangement also includes the recognition of calibration and measurement certificates issued by these institutes. Then, the consequences of this arrangement for trade are described. The case of chemical analysis is illustrated by the application of the Kyoto protocol on the reduction of greenhouse gases. But the global workload to be taken up by the International Committee of Weights and Measures, its Consultative Committee for Amount of Substance and the NMIs is huge.
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  • 59
    ISSN: 1432-1211
    Keywords: Key words Skin ; Genetics ; TNFA ; ¶Inflammation ; PCR-RFLP
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract  Mechanisms underlying susceptibility to skin irritants are not clearly understood. Cytokines play a key role in inflammation, and functional polymorphisms in cytokine genes may affect responses to irritants. We investigated the relationship between polymorphism in the tumor necrosis factor (TNF) α-chain gene and responses to irritants. Volunteers (n=221) tested with sodium dodecyl sulfate (SDS) and benzalkonium chloride (BKC) were divided into responders and nonresponders and high and low irritant-threshold groups. DNA was assayed for the TNF-308 polymorphism by a polymerase chain reaction-restriction fragment length polymorphism method. There was a significant increase in the A allele (P=0.030) and AA genotype (P=0.023) in both the SDS low irritant-threshold group and in SDS responders (A allele P=0.022, AA genotype P=0.048). In the BKC low irritant-threshold group, we found a significant increase in the A allele (P=0.002) and AA genotype (P=0.016). Individuals with a low threshold to both irritants demonstrated a significant increase (P=0.002) in the A allele. This is the first description of a nonatopic genetic marker for irritant susceptibility in normal individuals. Genotyping for theTNF-308 polymorphism may thus contribute to screening of individuals deemed at risk of developing irritant contact dermatitis.
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  • 60
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    European journal of pediatrics 159 (2000), S. 575-578 
    ISSN: 1432-1076
    Keywords: Key words Haemophilia ; HIV-negative patients ; Children ; Growth ; Body mass index
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract It has been shown that HIV-positive haemophilic children develop growth retardation. As not only the HIV infection but also other disease-related factors might compromise growth in these children, growth data were analysed in a longitudinal cross-sectional manner in 84 HIV-negative haemophilic patients from two university clinics. A total of 2–24 height and weight measurements (median 6) were recorded in each patient resulting in 683 single values collected between 1977–1995. Height SDS of all haemophilic boys was −0.31 ± 2.13 (mean ± SD, NS versus 0) and body mass index SDS was 0.21 ± 3.49 (mean SD, NS versus 0) at first measurement and remained unchanged throughout the observation period. Neither height nor body mass index differed with respect to the severity of haemophilia (mild/moderate/severe) or the study centre (Vienna/Prague). Conclusion Growth in HIV-negative patients with haemophilia is not affected in spite of the immunological abnormalities attributed to the substitution therapy or the bleeding episodes in the joints with the potential effect on the growth plate.
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  • 61
    ISSN: 1432-1076
    Keywords: Key words Sodium cromoglycate ; Children ; Exercise-induced asthma ; Urinary eosinophil protein X excretion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This double-blind, randomised and cross-over study was designed to compare the preventive effect against exercise-induced bronchoconstriction (EIB), defined as the percentage decrease in FEV1≥15% after 6 min of exercise, of 2 mg and 10 mg of sodium cromoglycate (SCG), administered through a metered dose inhaler via spacer, in asthmatic children. Each of the 30 subject (age 11.6 ± 3.2 years) was tested on five occasions. For inclusion, EIB in test1 was required. In tests 2 to 5, all subjects inhaled 2 mg or 10 mg of SCG 20 min and 120 min before exercise in a randomised order. In order to assess excretion of eosinophil protein X (EPX) accompanying EIB, urine samples were collected before and after exercise. The mean percentage fall in FEV1 (±SD) in test 1 was 26.8 ± 9.8%. Inhalation of 2 mg and 10 mg of SCG 20 min before exercise provided a significant preventive effect in 83% and 77% and inhalation 120 min before exercise provided a preventive effect in 63% and 70%, respectively (n=30). Variance analysis did not reveal a statistically different absolute fall in FEV1 after exercise when both doses (120 min before exercise) were compared (P=0.356). In an unselected subgroup of 12 children, urinary EPX increased after the challenge without SCG premedication (test 1) (mean change: +48.7 μg/mmol creatinine, P=0.034), whereas no significant increase was found in case of SCG premedication (mean change in μg/mmol creatinine): 2 mg/20 min: +12.1; 2 mg/120 min: +8.5; 10 mg/20 min: −10.4 and 10 mg/120 min: −23.5; P 〉 0.1). Conclusion Administration of 10 mg of sodium cromoglycate is no more effective in preventing exercise-induced bronchoconstriction than 2 mg regardless of whether the medication is given 20 or 120 min before exercise. The preventive effect of sodium cromoglycate on exercise-induced bronchoconstriction in asthmatic children is associated with the inhibition of urinary eosinophil protein X excretion.
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  • 62
    ISSN: 1432-1076
    Keywords: Key words Insulin aspart ; Insulin analogue ; Type 1 diabetes ; Pharmacokinetics ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The pharmacokinetics of the novel, rapid-acting insulin aspart were compared with those of soluble human insulin following subcutaneous administration in nine children (aged 6–12 years) and nine adolescents (aged 13–17 years) with stable type 1 diabetes. The study had a randomised, double-blind, two-period crossover design. Each patient received a single subcutaneous dose of insulin aspart or human insulin (0.15 IU/kg body weight) 5 min before breakfast and the plasma insulin and glucose concentrations were measured at intervals during the following 5 h. The pharmacokinetic profile of insulin aspart differed significantly from that of human insulin with a higher mean maximum serum insulin (Cmax ins), 881 ± 321 (SD) pmol/l versus 422 ± 193 pmol/l for human insulin (P 〈 0.001); and with a shorter median serum insulin t max ins, 40.0 min (interquartile range: 40–50 min) versus 75.0 min (interquartile range: 60–120 min) for human insulin, (P 〈 0.001). An age-related effect on Cmax ins and area under the curve (AUC0–5h ins) was observed with higher values in adolescents than in children for both insulin aspart and human insulin. Postprandial glycaemic control was improved with insulin aspart; the baseline-adjusted ΔCmax glu being lower for insulin aspart compared with human insulin (increase of 7.6 ± 5.1 versus 9.4 ± 4.4 mmol/l respectively, P 〈 0.05). The incidence of adverse events was similar for the two insulin types. Conclusion The more rapid onset of action of insulin aspart versus human insulin, previously observed in adults, is confirmed in a paediatric population with type 1 diabetes.
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  • 63
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    European journal of pediatrics 159 (2000), S. 530-534 
    ISSN: 1432-1076
    Keywords: Key words Schistosomiasis ; Children ; Travellers ; Ultrasonography ; Immunodiagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Reports on schistosomiasis in children growing up in Europe are rare despite increased travel activity. We report on eight male and three female German children aged 50 months to 15 years with schistosomiasis. Six children were asymptomatic, whereas two presented with typical signs of Katayama fever. Persisting haematuria, headache with eosinophilia and pyelonephritis were observed in one child each. An exposure was reported for six of the children. Two were examined solely because schistosomiasis was diagnosed in a family member. All had antibodies against schistosomal antigens in at least two of three screening tests. However, schistosomal ova (Schistosoma haematobium) were detected in urine and faecal specimens from only three children. A tumour-like lesion of the bladder was found by ultrasound in only one of the children who also exhibited haematuria. Neither eosinophilia nor elevated IgE levels were constant findings. Six to 12 months after praziquantel treatment, parasitological and ultrasound checks were negative and levels of specific antibodies decreased. However, 2 years later, elevated antibody levels were detected in one girl without evidence of any new exposure. She became antibody-negative 1 year after a second course of treatment. Conclusion In contrast to residents of endemic areas, parasitological and ultrasound examinations seem to be inferior to immunodiagnostics in children from non-endemic areas at temporary risk for schistosomiasis.
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  • 64
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Dermatologie ; Primary Health Care ; Langzeitergebnisse ; Kinder ; Kenia ; Keywords Dermatology ; Primary health care ; Long-term effects ; Children ; Kenya
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Background and Objective. In spite of the importance of skin diseases in Africa south of the Sahara, dermatology is insufficiently represented within the established primary health care systems. Aim of this study was to find out whether an integrated dermatology project could reduce the prevalence of dermatoses. Patients/Methods. Since 1994 trained community health workers have carried out regular weekly visits to schools and nurseries in 10 communities in rural western Kenya. Epidemiological studies were done in 13 schools in 4 communities involving 5780 and 4961 pupils one year before (1993) and 5 years (1999) following the introduction of the dermatology project. Results. Within this period the prevalence of bacterial skin infections fell from 12.7% to 11.3% (n.s.). Mycoses rose from 10.1% to 13.9% (p〈0.05), while arthropod infections (mainly scabies) remained unchanged with a prevalence of 8.3% in 1993 and 8.0% in 1999 (n.s.). Dermatitis also showed no changes (1.7% in both years). Conclusions. The prevalence of infective dermatoses depends not only on medical treatment but also far more on socio-economic factors.
    Notes: Zusammenfassung Hintergrund und Fragestellung. Trotz der großen Bedeutung der Dermatosen in Schwarzafrika ist die Dermatologie in den dort etablierten Basisgesundheitsdiensten völlig unzureichend repräsentiert. Untersucht werden sollten die Langzeitauswirkungen eines in das Primary Health Care-System integrierten Dermatologieprojekts in Bezug auf die Prävalenz von Hauterkrankungen. Patienten/Methodik. Seit 1994 besuchen ausgebildete Community Health Workers einmal wöchentlich Schulen und Vorschulkindergärten in 10 Gemeinden im ländlichen Westkenia. In 13 Schulen von 4 Gemeinden wurden 1 Jahr vor (1993) und 5 Jahre nach (1999) Projektinitiierung Reihenuntersuchungen an 5780 bzw. 4961 Kindern durchgeführt. Ergebnisse. Innerhalb dieses Zeitraums sank die Prävalenz bakteriell bedingter Dermatosen von 12,7% auf 11,3% (n.s.). Bei den Mykosen fand sich ein Anstieg von 10,1 auf 13,9% (p〈0,05). Arthropodenbedingte Infektionen (vorwiegend Skabies) blieben mit 8,3% 1993 und 8,0% 1999 (n.s.) im Wesentlichen gleich. Ekzeme wiesen zu beiden Zeitpunkten eine Prävalenz von 1,7% auf. Schlussfolgerungen. Die Prävalenz infektiöser Dermatosen hängt nur zu einem geringen Teil von der medizinischen Versorgung, sondern überwiegend von sozioökonomischen Faktoren ab.
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  • 65
    ISSN: 1432-1076
    Keywords: Key words Coeliac disease ; Children ; Iron deficiency anaemia ; Occult blood ; AbbreviationsCD coeliac disease ; ID iron deficiency ; GFD gluten-free diet
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract It has recently been suggested that in adults with coeliac disease, faecal blood loss may play a role in the development of iron deficiency. A group of 45 children diagnosed with coeliac disease during 1996 and 1997 were therefore prospectively evaluated for the presence of gluten in their diet, iron deficiency anaemia, and faecal occult blood. Sixty children admitted for elective surgery or asthma served as controls. Faecal occult blood was found in four iron deficient children on normal diet, of whom three were newly diagnosed. Occult blood loss disappeared in three of the four children when gluten was removed from their diet. Faecal occult blood was found in 26.7% of children on gluten-containing diet, but not in children on gluten-free diet (P=0.01), or in control children (P=0.001). Conclusion Our data suggest that the incidence of occult blood loss in coeliac disease occurs mainly in newly diagnosed cases and responds to a gluten-free diet. Occult blood testing may not be warranted in the absence of iron deficiency anaemia nor in children with iron deficiency anaemia who are on a gluten-free diet.
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  • 66
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Atopisches Ekzem ; Kinder ; Elternschulung ; Video ; Mütter ; Key words Atopic eczema ; Parental education ; Video ; Children ; Mothers
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Background and Objective. Psychological problems in children and parents related to children's atopic eczema (AE) may impede the success of treatment. We studied the question, if behavior-based parental education in groups (DPE) or standardized video-education (VPE) could enhance dermatological treatment effects and reduce skin-damaging behaviors in children and stress in their mothers. Patients/Methods. 47 mothers attending the university outpatient-clinic for dermatology and their AE-children (mean age 4 years) participated in the study. 18 mothers underwent the DPE (10 sessions), 15 mothers worked with VPE at home. Dermatological standard treatment (CG; N=14) served as control for a 16-weeks-evaluation-period. Results. AE-symptoms improved overall, but the effectiveness of the treatments differed significantly, improval with parent education and was best with VPE. Psychological problems of mothers were equally reduced with DPE and VPE. Conclusions. It is suggested that VPE is a cost effective and less time consuming method for supporting dermatological therapy of AE in children.
    Notes: Zusammenfassung Hintergrund und Fragestellung. Das atopische Ekzem (AE) im Kindesalter kann psychologische Probleme bei Kind und Eltern zur Folge haben, die den Behandlungserfolg erschweren. Die Effektivität direkter verhaltensorientierter Elterngruppenschulungen (DES) bzw. standardisierter Videoschulungen (VES) gegenüber der dermatologischen Standardbehandlung (KG) zur Besserung des AE, des Kratzverhaltens der Kinder und krankheitsbedingter Belastungen der Mütter wurde überprüft. Patienten/Methodik. An der Studie nahmen 47 Mütter und deren AE-Kinder (Durchschnittsalter 4 Jahre) aus der Neurodermitisambulanz der Universitätshautklinik teil, 18 Mütter besuchten die DES (10 Gruppensitzungen), 15 Mütter arbeiteten mit der VES zu Hause. Die Behandlung der KG (n=14) erfolgte im vergleichbaren 4-Monats-Zeitraum. Ergebnisse. Das AE war über alle Behandlungsbedingungen gebessert, ihre Effekstärken unterschieden sich jedoch signifikant: Elternschulungen waren effektiver als Standardbehandlung, den stärksten Effekt hatte die VES. Belastungen der Mütter reduzierten sich nach beiden Schulungsformen. Schlussfolgerungen. Die Ergebnisse verweisen auf eine Zeit und Kosten sparende Möglichkeit, Videoschulungen für die Unterstützung der Therapie des AE im Kindesalter zu nutzen.
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    Intensive care medicine 26 (2000), S. 942-949 
    ISSN: 1432-1238
    Keywords: Key words Ventilator-associated pneumonia ; Cardiac surgery ; Children ; Pediatric intensive care ; Complications ; Extubation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Objective: This study was undertaken to determine the delay of extubation attributable to ventilator-associated pneumonia (VAP) in comparison to other complications and complexity of surgery after repair of congenital heart lesions in neonates and children.¶Methods: Cohort study in a pediatric intensive care unit of a tertiary referral center. All patients who had cardiac operations during a 22-month period and who survived surgery were eligible (n = 272, median age 1.3 years). Primary outcome was time to successful extubation. Primary variable of interest was VAP. Surgical procedures were classified according to complexity. Cox proportional hazards models were calculated to adjust for confounding. Potential confounders comprised other known risk factors for delayed extubation.¶Results: Median time to extubation was 3 days. VAP occurred in 26 patients (9.6 %). The rate of VAP was not associated with complexity of surgery (P = 0.22), or cardiopulmonary bypass (P = 0.23). The adjusted analysis revealed as further factors associated with delayed extubation: other respiratory complications (n = 28, chylothorax, airway stenosis, diaphragm paresis), prolonged inotropic support (n = 48, 17.6 %), and the need for secondary surgery (n = 51, 18.8 %; e. g., re-operation, secondary closure of thorax). Older age promoted early extubation. The median delay of extubation attributable to VAP was 3.7 days (hazards ratio HR = 0.29, 95 % CI 0.18–0.49), exceeding the effect size of secondary surgery (HR = 0.48) and other respiratory complications (HR = 0.50).¶Conclusion: VAP accounts for a major delay of extubation in pediatric cardiac surgery.
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  • 68
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    Intensive care medicine 26 (2000), S. S057 
    ISSN: 1432-1238
    Keywords: Key words Critical illness ; Intensive care ; Severity of illness ; Scoring systems ; Genetics ; Susceptibility ; Education
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Risk of critical illness is determined both by genetic and environmental influences, particularly those relating to infectious and cardiovascular diseases. Physiologically-based scoring systems cannot measure prior risk because they do not quantify physiological reserve independently of the acute illness. Genetic profiling could be useful for risk assessment. Early detection of critical illness involves identifying physiological ’triggers' for referral; this requires the education of nursing and medical staff in their significance. Analysis of the relationship between risk factors and interventions may need complex modelling techniques. Therapeutic strategies depend on the nature of the underlying problem: the most useful are likely to be those which enhance tissue oxygen delivery and resistance to infection.
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    Pediatric nephrology 15 (2000), S. 248-251 
    ISSN: 1432-198X
    Keywords: Key words Nephrotic syndrome ; Acute renal failure ; Children ; Peritonitis ; Ischemic tubular necrosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Idiopathic acute renal failure (IARF) is an uncommon but severe complication in children with relapsing nephrotic syndrome and may require long-term dialytic support until recovery of renal function takes place. Due to limited understanding of the pathophysiology of IARF, specific guidelines for its prevention and therapy have not been developed. Among triggering factors, peritonitis was present in half of all pediatric patients with this complication described in the English literature over the past 15 years. We report an additional nephrotic child who developed IARF following spontaneous bacterial peritonitis. The renal biopsy showed tubular epithelial changes consistent with acute tubular necrosis. A discussion of related literature and possible pathogenesis of this association is presented.
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  • 70
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    Pediatric nephrology 15 (2000), S. 302-316 
    ISSN: 1432-198X
    Keywords: Key words Calcium channel blockers ; Children ; Pharmacokinetics ; Hypertension
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The calcium channel blockers (CCBs) are a diverse group of antihypertensive medications with variable pharmacokinetics and clinical effects. Although CCBs have been widely applied to the treatment of hypertensive children, data regarding the pharmacokinetics, efficacy and safety of these agents in children are extremely limited. In this review we briefly summarize the mechanism of action of CCBs and then summarize pertinent pharmacokinetic information on each of the CCBs commonly used in children, including amlodipine, diltiazem, felodipine, isradipine, intravenous nicardipine, nifedipine and verapamil. Clinically important drug interactions and adverse effects are discussed, as well as the potential role of CCBs in renal protection. Available pediatric efficacy and safety data are summarized, and recommendations made regarding the rational use of CCBs in the management of pediatric hypertension.
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  • 71
    ISSN: 1432-198X
    Keywords: Key words Glomerulocystic kidney disease ; Oligohydramnion ; Renal failure-neonate ; Genetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Two newborns with glomerulocystic kidney disease manifesting as late onset oligohydramnion and neonatal anuria, yet without severe respiratory distress, are presented. They had a similar perinatal course and associated clinical manifestations. No associated congenital or inherited malformation syndrome could be defined. Both infants’ parents were first degree cousins and belonged to the same small Bedouin tribe, and neither they nor the infants’ siblings had polycystic kidneys or renal insufficiency, pointing to either a possible genetic etiology or a common external toxic exposure.
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  • 72
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    Pediatric nephrology 15 (2000), S. 57-59 
    ISSN: 1432-198X
    Keywords: Keywords X-linked hypophosphatemia ; Dipyridamole ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  X-linked hypophosphatemia (XLH) is characterized clinically by rickets, hypophosphatemia and hyperphosphaturia. Conventional treatment of XLH with oral phosphate and vitamin D is associated with hypercalcuria and nephrocalcinosis. Recently, intravenous and oral dipyridamole has been reported to decrease fractional excretion of phosphate in adults with idiopathic hyperphosphaturia. Our objective was to determine whether oral dipyridamole therapy reduces urinary phosphate excretion and increases serum phosphate concentration in children with XLH. A prospective study was performed in six children with XLH. The average age of the patients at the start of the study was 12.5±1.0 years. The effects of 12 weeks of oral dipyridamole therapy, at 4.4±0.4 mg/kg body weight per day, on serum phosphorous, parathyroid hormone (PTH), 1,25 (OH)2 vitamin D, osteocalcin, tubular maximum for phosphate reabsorption (TmP/GFR), urinary calcium excretion, and cyclic adenosine 3’,5’-monophosphate (cAMP) excretion, were compared to baseline levels. Our results show that there was no change in serum phosphorous concentration or TmP/GFR after 12 weeks of dipyridamole therapy. Dipyridamole therapy also had no effect on serum PTH, serum 1,25 (OH)2 vitamin D, alkaline phosphatase, osteocalcin levels, urinary calcium or cAMP excretion. We therefore concluded that in children with XLH, a 12-week course of dipyridamole had no effect on serum phosphorous or its urinary excretion. Dipyridamole therapy is unlikely to improve the bone disease in children with XLH.
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  • 73
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    Pediatric nephrology 14 (2000), S. 1016-1021 
    ISSN: 1432-198X
    Keywords: Key words Nephrocalcinosis ; Sonography ; Renal function ; Body growth ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We carried out a retrospective survey on 152 children and adolescents with nephrocalcinosis (NC) in 22 German centers of pediatric nephrology. Etiology, clinical manifestations, growth and development, sonographic appearance of NC and renal function were analyzed. The median age at the time of diagnosis was 3.3 (range 0.1–21) years and the median duration of follow-up was 4.1 years. In 34% of children NC was associated with idiopathic hypercalciuria (IHC) and in 32% with various hereditary tubular disorders. In 9% NC was observed subsequent to prophylactic bolus administration of vitamin D in infancy. A positive family history was found in 36%. Clinical manifestations were mainly failure to thrive during the 1st year of life (46%), psychomotor/mental retardation (28%) and urinary tract infection (34%). In 14% nephrolithiasis was associated. During the follow-up the proportion of patients with the most severe degree of NC (stages 2b or 3) increased from 40% to 55% and that of hypercalciuria decreased from 79% to 52%. Body height was 〈2 standard deviation scores (SDS) of normal in 41% at the time of diagnosis and in 32% at the last observation; the increase in relative height was significant only for IHC. Glomerular filtration rate (GFR) and urinary concentration capacity changed only slightly with time. At the last investigation GFR was 〈50 ml/min/1.73 m2 in 6% and concentration capacity 〈800 mosmol/kg in 48% of patients. The degree of NC was negatively correlated with GFR and concentration capacity.
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  • 74
    ISSN: 1432-2129
    Keywords: Schlüsselwörter ; Kopfschmerz ; Kinder ; Akuttherapie ; Flupirtin ; Paracetamol ; Keywords ; Children ; Acute treatment ; Tension-type headache ; Flupirtine ; Paracetamol
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Background: About 10% of all schoolchil- dren are suffering from migraine and 50% from tension-type headache. Headache of acute onset usually will be treated with analgesic substances like paracetamol, acetylsalicylic acid or ibuprofen, the first one being the reference drug for tension-type headache in childhood. In case of lacking improvement or side-effects there is demand for an alternative safe substance for the acute analgesic therapy. Methods: In a double-blind randomised investigation flupirtine and paracetamol were given in two consecutive attacks of episodic tension-type headache. 30 children, 6–12 years old, were included. Dosage was determined according to age and weight. The children documented the acute headache intensity and duration in a special diary. Results: Headache intensity was reduced during 2 h after intake in 89% of the 19 children treated. The reduction was 6,5 to 3,1 for flupirtine and 6,9 to 3,3/10 for paracetamol. There was no statistically significant difference between the two substances. Relevant side-effects could not be observed. Conclusion: Flupirtine has shown a convincing clinical effect treating acute episodic tension-type headache for children. The substance was well tolerated by the patients. In addition, flupirtine provides a high degree of safety.
    Notes: Zusammenfassung Hintergrund: Etwa 10% aller Schulkinder leiden nach neueren deutschen epidemiologischen Untersuchungen zumindest gelegentlich an Migräne und etwa 50% an Kopfschmerzen vom Spannungstyp. Häufig nehmen sie bei Spannungskopfschmerzen analgetische Monosubstanzen wie Paracetamol, Azetylsalizylsäure oder Ibuprofen ein. Bei nicht ausreichender Wirkung bzw. Unverträglichkeit besteht Bedarf nach weiteren Substanzen für die Akutanwendung. Methode: In einer doppelblindrandomisierten und gekreuzten Anordnung wurden Paracetamol bzw. das analgetisch und muskelrelaxierend wirksame Flupirtin 30 6- bis 12jährigen Kindern für 2 episodische Spannungskopfschmerzattacken angeboten. 10 Kinder benötigten nach dem Erstkontakt keine Medikation mehr, 1 Kind lehnte die Einnahme grundsätzlich ab. Ergebnisse: Die Kopfschmerzstärke verringerte sich laut Kopfschmerztagebuch innerhalb von 2 h nach der Einnahme auf einer numerischen Schmerzskala (0–10) von 6,5 auf 3,1 unter Flupirtin und von 6,9 auf 3,3 unter Paracetamol bei 89% der verbliebenen 19 Kinder. Statistisch signifikante Unterschiede zwischen beiden Substanzen bestanden nicht. Als Nebenwirkung trat 1-mal Erbrechen unter Paracetamol auf. Schlussfolgerung: Flupirtin hat sich in der Akutphase von episodischen Spannungskopfschmerzen beim Kind bewährt. Es verfügt über eine gute Verträglichkeit. Im Vergleich zu Paracetamol scheint v.a. bei akzidenteller Überdosierung eine größere Sicherheit zu bestehen.
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  • 75
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    Der Schmerz 14 (2000), S. 302-308 
    ISSN: 1432-2129
    Keywords: Schlüsselwörter Schmerzmessung ; Diagnostik ; Instrumente ; Neugeborene ; Kinder ; Keywords Pain ; Measurement ; Assessment ; Instruments ; Neonates ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Background. The assessment and measurement of pain is essential in the implementation and control of pain relieving strategies. The measurement of pain in infants and children should be based on the consideration of age, cognitive level, psychological status, intercurrent diseases and the social context in order to register the child's individual situation and to avoid misinterpretation. Diagnosis. In the preverbal infant, behavioral and physiological cues have to be interpreted by the caregivers. For the assessment of pain in children of four and older who have at least a basic understanding of the pain concept self assessment methods (as rating scales, specific pain interviews, diaries and questionnaires) can be used. In any case the instruments used should be age appropriate. The instruments used for the different age groups are presented with comments on quality and clinical applicability.
    Notes: Zusammenfassung Hintergrund. Voraussetzung zur Durchführung und Kontrolle einer adäquaten und effektiven Schmerztherapie sind die Erfassung und Quantifizierung des Schmerzes in seinen verschiedenen Dimensionen. Bei der Diagnostik des Schmerzes im Kindesalter müssen Alter, kognitiver Entwicklungsstand, psychologischer Status, interkurrierende Erkrankungen und der soziale Kontext berücksichtigt werden, um das Kind in seiner individuellen Situation erfassen zu können und Fehleinschätzungen zu vermeiden. Schmerzdiagnostik im Kindesalter. Im präverbalen Alter werden die physiologischen und verhaltensbezogenen Schmerzäußerungen von Neugeborenen, Säuglingen und Kleinkindern durch eine Fremdbeurteilung erfasst. Bei Kindern ab etwa 4 Jahren, die über ein einfaches Verständnis von Schmerz verfügen, sind Verfahren der Selbsteinschätzung wie einfache Ratingskalen, spezifische Schmerzinterviews, Tagebücher und Fragebögen die primär einzusetzenden Instrumente. In jedem Fall sollten die Instrumente altersgerecht gestaltet sein. Für die verschiedenen Altersgruppen werden Instrumente der Schmerzerfassung mit qualitativen Hinweisen auf deren Güte und klinische Anwendbarkeit vorgestellt.
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  • 76
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    Der Schmerz 14 (2000), S. 319-323 
    ISSN: 1432-2129
    Keywords: Schlüsselwörter Schmerz ; Kinder ; Impfung ; Verbrennung ; Injektion ; Keywords Pain ; Children ; Vaccination ; Burn ; Injection
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Phenomenon pain. While pain is one of the main reasons for an unscheduled visit to the paediatrician, pain due to painful procedures is of major importance in scheduled visits. Actual pain therapy is illustrated in the treatment of burns. Incomplete analgesia may have an unfavourable impact on morbidity and mortality. The pain score does not correlate with the extent of the burned area, and is regularly underestimated. General anaesthesia or analgo-sedation are warranted during the care of the burned patient. Unsufficient analgesia. Consequence of insufficient analgesia during primary care is an increased need of analgesics, and an increased pain treatment failure rate during subsequent procedures. Pain is interfering with anxiety, sleep disturbancies and post-tramatic psychologic alterations. All those symptoms must be treated adequately. Acute illness and injections. This article covers pain from otitis media, pharyngitis, Guillain-Barré syndrome, purpura fulminans, Toxic Epidermal Nekrolysis, as well as the usage of local anaesthesia during injections, not to forget the application of non-pharmacologic methods for pain therapy and prophylaxis.
    Notes: Zusammenfassung Phänomen Schmerz. Das Phänomen “Schmerz” begleitet fast alltäglich den Kontakt zwischen Arzt und krankem Kind. Auf der einen Seite sind Schmerzen der häufigste Grund für eine ungeplante Kinderarztkonsultation, auf der anderen Seite sind bei geplanten Kinderarztbesuchen häufig schmerzhafte Prozeduren durchzuführen. Anhand der starken Schmerzen bei den im Kleinkindalter häufigen Verbrennungen werden schmerztherapeutische Prinzipien konkretisiert: Schmerztherapeutische Prinzipien. Unzureichende Analgesie kann Morbidität und Mortalität ungünstig beeinflussen. Das Schmerzmaß korreliert nicht mit der Ausdehnung der Verbrennung und wird vom Behandler regelmäßig unterschätzt. In vielen Fällen der Erstversorgung sind Allgemeinanästhesie oder Analgosedierung gerechtfertigt, ähnliches gilt für den Verbandwechsel. Ungenügende Analgesie bei der Erstversorgung führt zu erhöhtem Analgetikaverbrauch und schmerztherapeutischen Misserfolgen bei Folgeeingriffen. Wechselwirkungen zwischen Schmerzen und anderen Symptomen wie Angst, Schlafstörungen oder postraumatischen psychischen Veränderungen sind zu beachten und adäquat zu therapieren. Akute Erkrankungen + Injektionen. Weiter wird auf Schmerzen bei Otitis media, Pharyngitis, Guillain-Barré-Syndrom, Purpura Fulminans und Toxischer Epidermaler Nekrolyse sowie den Einsatz von Lokalanästhetika bei Injektionen eingegangen. Schließlich haben auch nicht pharmakologische Methoden ihren Platz in Schmerztherapie und Prophylaxe.
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  • 77
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    Der Schmerz 14 (2000), S. 333-339 
    ISSN: 1432-2129
    Keywords: Schlüsselwörter Postoperative Schmerztherapie ; Schmerz ; Kinder ; Jugendliche ; Analgetika ; Keywords Postoperative pain therapy ; Pain ; Children ; Adolescents ; Analgesics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Treatment of pain. Undertreatment of postoperative pain in children is a problem in clinical practice. This is due to a lack of both knowledge about age-specific aspects of physiology and pharmacology, and routine pain assessment. For example, the fear of side-effects prevents the adequate usage of opioids. It is of major importance to select a route of drug administration where the child feels comfortable with (avoid intramuscular injections). Non-opioid analgesics. Non-opioid analgesics are recommended for basic pain treatment after minor surgical procedures. Instead of using the whole multitude of drugs available, the doctor should stick to those drugs he is familiar with (acetaminophen, ibuprofen, diclofenac, dipyrone). Opioids. Opioid usage requires individual dose titration and careful monitoring of side-effects (respiratory monitoring, sedation score). The strong opioids piritramide and morphine may advantageously be administered as either continuous, or patient-controlled iv- infusion (PCA). Forms of therapy. In addition to infiltration anesthesia, intraoperatively applied nerve blocks provide excellent pain relief. Epidural analgesia with local anesthetics and/or opioids via a thoracic or lumbar epidural catheter is a therapeutic option after thoracic or abdominal surgery, or after extensive orthopedic or urological interventions. Adjuvant analgesics and nonpharmacologic interventions, i. e. transcutaneous electrical nerve stimulation (TENS), are primarily indicated in patients suffering from neuropathic pain. Conclusion. The establishment of pain services and the comprehensive education of both the nursing and the medical staff should help to improve postoperative pediatric pain therapy.
    Notes: Zusammenfassung Schmerztherapie bei Kindern. Postoperative Schmerzen bei Kindern werden häufig wegen mangelnder physiologischer und pharmakologischer Kenntnisse und des Fehlens einer regelmäßigen standardisierten Schmerzmessung noch immer unzureichend behandelt. Die Angst vor Nebenwirkungen verhindert eine adäquate Opioidtherapie. Bei der Verwendung von Analgetika ist auf eine kindgerechte Applikationsweise (keine i.-m.-Injektionen!) zu achten. Nichtopioidhaltige Analgetika. Nichtopioidhaltige Analgetika (Parazetamol, Ibuprofen, Diclofenac, Metamizol) sind zur Basisschmerztherapie bei kleineren schmerzhaften Eingriffen geeignet. Opioide. Opioide müssen nach Wirkung titriert werden; Nebenwirkungen können nur bei sorgfältigem Monitoring von Atmung und Sedierungsgrad frühzeitig erkannt werden. Opioide (Tramadol, Piritramid, Morphin) können entweder kontinuierlich i. v. oder mittels PCA-Pumpe verabreicht werden. Therapieformen. Neben der Oberflächenanästhesie sind intraoperativ angelegte Nervenblockaden effektive therapeutische Möglichkeiten. Eine lumbale und thorakale Periduralanästhesie mittels Lokalanästhetika und/oder Opioiden bietet sich bei thorakoabdominalen Eingriffen an. Koanalgetika werden vornehmlich bei Nervenschmerzen verwendet und richten sich gegen die schmerzverursachende Pathophysiologie. Optimierung der Schmerztherapie. Die postoperative Schmerztherapie bei Kindern kann durch Einrichtung eines Akutschmerzdiensts und kontinuierliche Weiterbildung von Pflegepersonal und Ärzten optimiert werden.
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  • 78
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    European journal of pediatrics 159 (2000), S. 430-433 
    ISSN: 1432-1076
    Keywords: Key words Alport syndrome ; Angiotensin-converting enzyme inhibitors ; Proteinuria ; Children ; AbbreviationACEi angiotensin-converting enzyme inhibitor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Enalapril, a long-acting inhibitor of angiotensin-converting enzyme, was given for 2 years to seven children with Alport syndrome. Five patients had a classical X-linked form of the disease; two siblings had the autosomal recessive variant. Their age was between 5.15 and 13.75 years when enalapril was started. All patients had haematuria and proteinuria, creatinine clearance was 〉80 ml/min per 1.73 m2 in all, and only one patient was hypertensive. The starting dose of enalapril (0.1 mg/kg body weight per day) was increased progressively according to individual clinical tolerance. The median doses were 0.13, 0.12, 0.21 and 0.29 mg/kg at 6, 12, 18 and 24 months, respectively. Median values of mean blood pressure were 95 mmHg at the start and 84 mmHg after 24 months. Median daily proteinuria decreased from 52 mg/kg to 18 mg/kg at 6 months, 21 mg/kg at 12 months, 12 mg/kg at 18 months and 30 mg/kg at 24 months. Serum creatinine increased over time from a median of 0.64 mg/dl at baseline to 0.77 mg/dl at 24 months. Concomitantly, there was a decrease in GFR from 104 to 83 ml/min per 1.73 m2 at 18 months and an increase again to 95 ml/min per 1.73 m2 at 24 months. Analysis of the individual data showed three patterns: no response (n=2), temporary response (n=2) and sustained response (n=3). Conclusion When given enalapril at the dosages mentioned, Alport patients as a group display a marked reduction in urinary protein excretion with a nadir of 23% of the baseline figure at 18 months, a decrease that cannot be accounted for by the slight decrease in glomerular filtration rate. Although these are preliminary data, it is recommended to try an angiotensin-converting enzyme inhibitor in every paediatric Alport patient with proteinuria.
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  • 79
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    European journal of pediatrics 159 (2000), S. S183 
    ISSN: 1432-1076
    Keywords: Key words Database ; Genetics ; Information services ; Internet ; Mutation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Thanks to the World Wide Web, most results of research in genetics are made available in public databases. At the present time there are resources on genetic diseases, genes and their location, mutations of already cloned genes and on laboratories performing the mutation analysis. The main resources on phenotypes are On-line Mendelian Inheritance in Man (OMIM), Pedbase, GeneClinics, London Dysmorphology Database (LDDB) and Orphanet. The main resources on human genes are, in addition to OMIM, the Genome Database, Genatlas and Genecard. There are also two major sequence databases. All of them can be queried using the OMIM number of the disease. Central databases of mutations, as well as locus specific databases have been created. Their list is maintained at the Human Genome Organisation mutation database initiative website. Several initiative have been taken to integrate all these data and help the clinician to find out quickly what he/she needs. The website of the National Center for Biotechnology Information is the best example of such an effort with sections on diseases, a genome guide, and locus links. Several databases of genetic testing resources have been established. GeneTests is an on-line genetics resource that contains a directory of North American laboratories providing testing for heritable disorders. Orphanet is a similar database on French services which is in the process of becoming a European database. Conclusion Even if clinicians do not have as many services at their disposal as the molecular geneticists, various useful databases already exist and should no longer be ignored in practice.
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  • 80
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    European journal of pediatrics 159 (2000), S. S208 
    ISSN: 1432-1076
    Keywords: Key words Cardiovascular disease ; Cystathionine β-synthase ; Genetics ; Methylenetetrahydrofolate reductase ; Mild hyperhomocysteinaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Over the last decade mild hyperhomocysteinaemia has widely been recognised as a new risk factor for arteriosclerosis and thrombosis. Main regulating enzymes of homocysteine (Hcy) metabolism are cystathionine β-synthase (CBS), methionine synthase and methylenetetrahydrofolate reductase (MTHFR). Early studies on patients with vascular disease described elevated Hcy concentrations after methionine loading and decreased CBS activity, resembling heterozygotes for CBS deficiency. Therefore, heterozygosity for CBS deficiency was proposed as the main cause of mild hyperhomocysteinaemia. However, more recent enzymatic and molecular genetic studies have demonstrated that heterozygosity for CBS deficiency is not or only a very minor cause of mild hyperhomocysteinaemia in vascular disease. We discovered two common genetic causes of mild hyperhomocysteinaemia, the 677C 〉 T and the 1298A 〉 C mutations in the coding region of MTHFR. The 677C 〉 T mutation causes reduced enzyme activity with thermolabile protein properties, elevated Hcy and low-normal or decreased plasma folate levels. The 1298A 〉 C mutation relates also to decreased enzyme activity, but not to thermolabile protein, and Hcy and folate levels are not influenced. However, compound heterozygosity for these two mutations, i.e. individuals with the 677CT/1298AC genotype, have elevated Hcy and decreased plasma folate levels. Gene-enviroment interactions between 677C 〉 T and folate is demonstrated in individuals with the 677TT genotype. Those with low-normal folate have elevated Hcy, whereas those with high-normal folate have normal Hcy concentrations. The elevated Hcy levels due to these mutations can be normalised by administration of folate, but whether folate reduces the risk of cardiovascular disease remains to be established. Conclusion Heterozygosity for cystathionine β-synthase deficiency is a minor cause of hyperhomocysteinaemia. The current data on mutations in the methylenetetrahydrofolate reductase gene do not tell us whether elevated plasma homocysteine plays a causal role in vascular disease. Low cellular vitamin status may be a possible cause and homocysteine may just be a marker for this situation.
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  • 81
    ISSN: 1432-1076
    Keywords: Key words Health-related quality of life ; Health status ; Cross-cultural adaptation ; Children ; Health Utilities Index
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Steady progress in developing effective treatments for childhood cancer and other severe pediatric diseases has established the need to consider the nature and frequency of late physical and psychological effects. The Health Utilities Index Mark 2 and Mark 3 (HUI2/3) systems were developed by Feeny, Furlong, Torrance et al. in Canada. These systems are generic multi-attribute measures of a person's health status and health-related quality of life. The first German version of the Canadian HUI2/3 questionnaire was created in our clinic, following recommended guidelines for cross-cultural adaptation of health-related quality of life measures. The usefulness of the resultant version was investigated using a sample of 142 patients who presented to our oncological outpatients' department for a routine health care visit after completion of treatment. The 15 items of the HUI2/3-questionnaire were answered independently by three groups of assessors – nurses, physicians, and parents or patients. Two additional questions covered ratings of the severity of treatment effects and the specification of these effects. The questionnaire was both easy to use and acceptable to the assessors. Percentage agreement between observers about levels for individual attributes ranged from 56% to 100%, with the lowest agreement on the subjective attributes of emotion, pain and cognition. These results are in accordance with previous studies using the original instrument. HUI2 global utility scores were significantly related to ratings of treatment sequelae, giving support to the discriminant validity of the measure. Conclusion The German version of HUI2/3 is a useful instrument with generally high inter-observer agreement and good suitability for outcome measurement in childhood cancer patients. Further research is needed to assess the usefulness of the instrument in other clinical populations and its sensitivity in longitudinal studies.
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    European journal of pediatrics 159 (2000), S. 507-508 
    ISSN: 1432-1076
    Keywords: Key words Behçet disease ; Children ; Myositis ; Pustulosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 12-year-old boy presented with a limp and findings suggesting localised myositis of his right calf and a working diagnosis of Behçet disease was made. During 3 years of follow-up, he had another three episodes of calf myositis, all responsive to corticosteroids within days. Conclusion A case of recurrent localised myositis as a main manifestation of Behçet disease is reported. The evolution of incomplete Behçet disease, which is common in children, to the full blown form, with the emphasis on muscle involvement and the importance of early diagnosis of Behçet disease, is discussed.
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  • 83
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    European journal of pediatrics 159 (2000), S. 515-519 
    ISSN: 1432-1076
    Keywords: Key words Osteogenesis imperfecta ; Hearing loss ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Osteogenesis imperfecta (OI) is a genetic disorder of connective tissue. Progressive hearing loss is one of the principal symptoms of OI, affecting about 50% of adult patients. Hearing loss may also occur in childhood and results in additional disability in education and psychosocial adaptation and aggravates the physical handicap. This can be avoided by appropriate otological and audiological treatment. In a nationwide search, 254 Finnish patients with OI were identified indicating a prevalence of 4.9/100 000. Of the 60 children, 45 aged between 4 and 16 years accepting to participate the study on hearing, were evaluated by a questionnaire and clinical audiometry. Hearing loss was defined as pure tone average (PTA0.5–2 kHz) more than 20 dB hearing level (HL). A clinical geneticist determined the type of OI among the 45 patients. Two sporadic OI cases with conductive hearing loss were ascertained (4.4%): An 11-year-old girl with type IV OI with a PTA0.5–2 kHz of 35/40 dB HL and a 15-year-old boy with type IV OI with a PTA0.5–2 kHz of 27/18 dB HL. In addition, a 6-year-old girl with familial OI type I had either a congenital sensorineural deafness or early progressive deafness with PTA0.5–2 kHz of 97/103 dB HL, probably of unrelated aetiology. Conclusion Hearing loss in children with osteogenesis imperfecta is less frequent than generally suspected. Nevertheless, it is recommended that audiometry is performed in children with osteogenesis imperfecta even without symptoms of hearing loss at the age of 10 years, and repeated every 3 years thereafter.
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  • 84
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    European journal of pediatrics 159 (2000), S. S35 
    ISSN: 1432-1076
    Keywords: Key words Obesity ; Genetics ; Child ; Nutrient balance ; Energy balance ; Environment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The epidemic diffusion of obesity in industrialised countries has promoted research on the aetiopathogenesis of this disorder. The purpose of this review is to focus mainly on the contribution that European research has made to this field. Available evidence suggests that obesity results from multiple interactions between genes and environment. Parents obesity is the most important risk factor for childhood obesity. Twin, adoption, and family studies indicated that inheritance is able to account for 25% to 40% of inter-individual difference in adiposity. Single gene defects leading to obesity have been discovered in animals and, in some cases, confirmed in humans as congenital leptin deficiency or congenital leptin receptor deficiency. However, in most cases, genes involved in weight gain do not directly cause obesity but they increase the susceptibility to fat gain in subjects exposed to a specific environment. Both genetic and environmental factors promote a positive energy balance which cause obesity. The relative inefficiency of self-adapting energy intake to energy requirements is responsible for fat gain in predisposed individuals. The role of the environment in the development of obesity is suggested by the rapid increase of the prevalence of obesity accompanying the rapid changes in the lifestyle of the population in the second half of this century. Early experiences with food, feeding practices and family food choices affect children's nutritional habits. In particular, the parents are responsible for food availability and accessibility in the home and they affect food preferences of their children. Diet composition, in particular fat intake, influences the development of obesity. The high energy density and palatability of fatty foods as well as their less satiating properties promotes food consumption. TV viewing, an inactivity and food intake promoter, was identified as a relevant risk factor for obesity in children. Sedentarity, i.e. a low physical activity level, is accompanied by a low fat oxidation rate in muscle and a low fat oxidation rate is a risk factor of fat gain or fat re-gain after weight loss. Conclusion Further research is needed to identify new risk factors of childhood obesity, both in the genetic and environmental areas, which may help to develop more effective strategies for the prevention and treatment of obesity.
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    European journal of pediatrics 159 (2000), S. 649-656 
    ISSN: 1432-1076
    Keywords: Key words Antiretroviral therapy ; Children ; AIDS
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The introduction of combination antiretroviral therapy has been associated with a dramatic clinical improvement in children with human immunodeficiency virus infection. However, the uptake of antiretroviral therapy has been variable across Europe. The Paediatric European Network for the Treatment of AIDS Steering Committee has performed a systematic literature review of paediatric antiretroviral therapy trials. An analysis of the evidence base for the commencement and maintenance of antiretroviral therapy was produced. Suggestions for when to commence antiretroviral therapy, which drugs to start with and how to monitor and sequence drug regimens are given. Conclusion The aim of these guidelines is to help in obtaining equity of access to a uniformly high standard of care for children with human immunodeficiency virus infection in all European countries.
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  • 86
    ISSN: 1432-1076
    Keywords: Key words Gentamicin ; Once daily ; Children ; Saliva
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Gentamicin is widely used in paediatric medicine and therapeutic monitoring is mandatory due to the narrow margin of safety. Saliva sampling may be of potential interest, especially in children in whom blood sampling is often difficult. Experience with once daily intravenous administration of aminoglycosides has grown in recent years. Gentamicin levels were measured in serum and saliva of 55 children treated with the drug (5 mg/kg per day), administered intravenously in three different regimens: thrice (n=19), twice (n=18), and once daily (n=18). No correlation was found between serum gentamicin concentrations and saliva levels when the drug was administered twice or thrice daily, however, there was good correlation when the drug was administered once daily (r 2=0.96, P 〈 0.0001). Conclusion In children with uncomplicated infections treated with once daily gentamicin, trough concentrations of the drug can be monitored in saliva.
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  • 87
    ISSN: 1432-1459
    Keywords: Key words Amyotrophic lateral sclerosis ; Genetics ; Presenilin-1 intron 8 polymorphism ; Apoptosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The mechanisms underlying motor neuron degeneration in amyotrophic lateral sclerosis are not fully understood. Recent studies suggest that apoptosis is involved in the abnormal neural death that occurs in this devastating disease. Presenilin-1, a transmembrane protein, seems to be implicated in apoptosis. To determine whether presenilin-1 intron 8 polymorphism has an influence in the course of amyotrophic lateral sclerosis, we examined this polymorphism genotypes in a large group of patients (n=72) with amyotrophic lateral sclerosis and in a random sample of 213 healthy individuals. The results showed a significant difference in genotype (P 〈 0.04) and allele (P 〈 0.03) distribution between patients and controls. These results suggest a possible intervention of presenilin-1 in the pathogenesis of amyotrophic lateral sclerosis.
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  • 88
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    Journal of neurology 247 (2000), S. 81-87 
    ISSN: 1432-1459
    Keywords: Key words Dementia ; Vascular ¶dementia ; Alzheimer’s disease ; Risk factors, stroke ; Genetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This review describes differing profiles of vascular risk factors in different types of dementia. Although vascular risk factors are related to various types of strokes, their independent effect on the occurrence of poststroke dementia appears to be small. Various risk factors have been identified for microangiopathy-related cerebral abnormalities, such as white matter changes and lacunae, which are the core lesions for the development of a vascular dementia syndrome without stroke symptoms. Most consistently, arterial hypertension and diabetes mellitus have been found to be associated with such brain abnormalities. Diastolic blood pressure seems to be of particular importance as recent investigations demonstrate that this factor is related to the course of multiple lacunar strokes and the progression of white matter disease. Epidemiological studies report that various vascular risk factors including arterial hypertension, diabetes mellitus, and atrial fibrillation may also be associated with Alzheimer’s disease. There is also evidence of a direct relationship between Alzheimer’s disease and general atherosclerosis. Further investigations are needed to determine whether these associations are due to the weakness of diagnostic criteria, or whether vascular risk factors indeed modulate the clinical expression of primary degenerative dementia. Common susceptibility genes leading to shared risk factors may be one of the reasons for a higher coincidence of Alzheimer’s disease and vascular dementia than can be expected by chance. A modulatory effect of vascular risk factors in the development of primary degenerative dementia may extend treatment options.
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  • 89
    ISSN: 1432-1459
    Keywords: Key words Parkinson’s disease ; Monoamine oxidase B ; Genetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Monoamine oxidase B (MAOB) metabolises dopamine and activates neurotoxins known to induce parkinsonism in humans and primates. Therefore the MAOB gene (MAOB; Xp15.21–4) is a candidate gene for Parkinson’s disease (PD). Longer length dinucleotide repeat sequences in a highly polymorphic GT repeat region of intron 2 of this gene showed an association with PD in an Australian cohort. We repeated this allele-association study in a population of 176 Chinese PD patients ¶(90 men, 86 women) and 203 age-matched controls (99 men, 104 women). Genomic DNA was extracted from venous blood and the polymerase chain reaction was used to amplify the appropriate regions of the MAOB gene. The length of each (GT) repeat sequence was determined by 5% polyacrylamide denaturing gel electrophoresis. There was no significant difference in allele frequencies of the (GT) repeat allelic variation between patients and controls (χ2 = 2.48; df = 5, P 〈 0.75). Therefore the longer length GT repeat alleles are not associated with PD in this Chinese population. Possible reasons for the discrepancy between Chinese and Australian populations include a different interaction between this genetic factor and environmental factors in the two populations and the possibility that the long length GT repeat alleles may represent a marker mutation, genetically linked to another susceptibility allele in whites but not in Chinese. Methodological differences in the ascertainment of cases and controls in this cohort could also explain the observed differences. Further study is required to determine whether the longer length GT repeat alleles are true susceptibility alleles in PD.
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  • 90
    ISSN: 1432-1459
    Keywords: Key words Progressive ¶supranuclear palsy ; Genetics ; Clinical characteristics ; Parkinsonism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Genetic studies have detected an association between the presence of the τ gene A0 allele and patients with progressive supranuclear palsy (PSP). This study examined whether patients with this polymorphism exhibit distinct demographic or clinical characteristics. We studied 26 patients who fulfilled clinical criteria for the diagnosis of PSP, 20 who had the A0/A0 genotype and 6 who had other genotypes. A questionnaire on demographic data, past medical history, familial history, and initial symptoms was completed as part of the consultation. A complete neurological examination was performed and PSP symptoms were quantified following Golbe’s PSP disability scale. We found a significant difference in the age at onset of PSP symptoms, which was 65.9 ± 5.3 years in the A0/A0 group and 71.2 ± 5.6 in the non-A0/A0 group (P = 0.016). There were no significant differences in the years from disease onset between the two groups. Symptom severity did not differ significantly in patients with the different A0/A0 genotypes. The detection of significantly lower age at onset with the A0/A0 alleles is consistent with the known association of this genotype as a risk factor for PSP. No significant differences were detected in symptom severity between the two groups of patients.
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  • 91
    ISSN: 1432-1459
    Keywords: Key words Multiple sclerosis ; Siblings ; Genetics ; Oligoclonal bands ; Measles
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We found that 19% (9/47) of healthy siblings of patients with clinically definite multiple sclerosis had an intrathecal immunological reaction with two or more 2 CSF-enriched oligoclonal bands (OCBs), in contrast to (4%) (2/50) unrelated healthy controls. Furthermore, in this group of nine healthy sibs the measles CSF IgG antibody titers were higher than that of the other sibs and that of controls. There were also differences in the serum titers for measles IgG antibody, which were higher in the group of all healthy sibs than in healthy volunteers, and (as with CSF titers) higher in the subgroup of healthy sibs with two or more 2 CSF-enriched OCBs than the other sibs. Thus a significant proportion of healthy siblings to MS patients have a partially hyperimmune condition similar to that occurring in MS, which in 19% manifested itself as an OCB reaction, in 9% as increased CSF measles IgG antibody titers, and in 21% as increased serum measles IgG antibody titers, these phenomena tending to occur in the same individuals. This condition is characterized by CSF-enriched OCBs with undefined specificity, although some increased antiviral reactivity is found both in the serum and CSF. While it needs further characterization, a genetic trait interacting with common infections is suggested. The recurrence risk of this condition is approximately five times higher than the 3–4% recurrence risk for manifest MS reported for sibs.
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  • 92
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    Der Hautarzt 51 (2000), S. 95-100 
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Dubowitz-Syndrom ; Atopisches Ekzem ; Monozygote Zwillinge ; Kinder ; Keywords Dubowitz syndrome ; Atopic eczema ; Monozygotic twins ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract Autosomal recessive inheritance, intrauterine growth retardation, short stature, microcephaly, distinct facial dysmorphism, psychomotoric retardation, and often uncharacterized eczematous skin lesions distinguish the rare Dubowitz syndrome. Here a pair of monozygotic twins with Dubowitz syndrome and clear-cut atopic eczema is presented.
    Notes: Zusammenfassung Autosomal rezessiver Erbgang, intrauterine Wachstumsretardierung, Kleinwüchsigkeit, Mikrozephalie, charakteristische Gesichtsdysmorphie, psychomotorischer Entwicklungsrückstand sowie häufig nicht näher charakterisierte ekzematöse Hautveränderungen kennzeichnen das seltene Dubowitz-Syndrom. Wir berichten über monozygote Zwillinge mit Dubowitz-Syndrom und eindeutigem atopischen Ekzem.
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  • 93
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    Child's nervous system 16 (2000), S. 170-175 
    ISSN: 1433-0350
    Keywords: Key words Supratentorial ependymoma ; Children ; Radical surgery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  In view of the widely recognized correlation between extent of surgical resection and length of survival of children with intracranial ependymoma and the statement that total resection is more likely to be achieved in supratentorial than infratentorial primaries, we decided to review our experience with supratentorial ependymomas and the pertinent literature to verify the importance of surgery in treating this subgroup of pediatric ependymal neoplasms. Of 23 patients operated on, 12 are still alive without evidence of disease 72–357 months after surgery (mean 227, median 237 months). One girl treated by surgery alone was lost to follow-up after 234 months when she, and 7 other patients in the series, had already passed the end of the period of risk for recurrence according to Collins’ law. Six surviving patients (2 with subependymoma and 4 with ependymoma) were treated by surgery alone and only 1, the oldest in the series, had to undergo a second operation for recurrence after 10 years. The idea of treating intracranial ependymoma by surgery alone was favored by eminent neurosurgeons in the past and has recently received renewed attention. This was in part the consequence of recognizing that unlike diffuse astrocytoma, in which neoplastic cells can be found up to several centimeters away from the apparent tumor borders, ependymoma has more or less well-defined margins and grows mainly by expansion. Early experience with the policy of electively deferring adjuvant therapy after radiologically controlled total resection of ependymoma seems encouraging, although postoperative MRI does not yet indicate absolute certainty. Close surveillance is recommended. The majority of ependymomas so far treated by surgery alone, with relatively good success, have been supratentorial. In conclusion, on the basis of our experience and a review of the literature we favor a change in attitude to the management of intracranial ependymomas, especially of the cerebrum, with radiologically controlled radical surgery alone followed by close surveillance with periodic MR imaging until the child passes the period of risk for recurrence according to Collins’ law as the initial option. In children less than 3 years old the period of surveillance should be doubled. In case of recurrence, reoperation should be considered first, particularly for supratentorial primaries. Radiotherapy continues to be a major option in malignant ependymoma and unresectable primary or recurrent benign ependymoma.
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  • 94
    ISSN: 1432-2161
    Keywords: Key words Bone mineral density ; Dual energy X-ray absorptiometry ; Children ; Rickets ; X-linked hypophosphatemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Objective. To evaluate the bone mineral status of children being treated for X-linked hypophosphatemia, including potential differences between cortical bone in the radial diaphysis and combined cortical and trabecular bone in the lumbar spine. Design and patients. Forty-four bone mineral evaluations were performed in 11 children and adolescents with X-linked hypophosphatemia. Bone mineral density (BMD) of the lumbar spine and the radial diaphysis were measured by dual X-ray absorptiometry (DXA), second metacarpal cortical thickness was measured on hand radiographs, and these results were expressed as Z-scores (standard deviations from the mean). Results. For the 11 initial examinations, Z-scores (mean±SD) were: radial BMD, –2.73±1.15, lumbar BMD, +1.28±1.53; and cortical thickness, –2.21±0.95. Lumbar BMD Z-scores were significantly greater than those for radial BMD and cortical thickness. On follow-up examinations there was a mild increase in radial BMD and decrease in lumbar BMD. Although these changes were statistically significant, they were quite small and the discordance between radial and lumbar BMD was not corrected. Conclusions. Children and adolescents who are being treated for X-linked hypophosphatemia manifest a bone mineral disorder characterized by decreased BMD in the appendicular skeleton and increased BMD in the lumbar spine. Although current therapy is successful in its anti-rachitic effects, it does not correct this bone mineral disorder and additional therapeutic trials should be considered.
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  • 95
    ISSN: 1432-2277
    Keywords: Key words Liver transplantation ; Children ; Risk factors for survival ; Primary non-function ; Hepatic artery thrombosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Several recipient, donor and operation factors as well as postoperative complications related to patient survival after liver transplantation (LT) in children were studied by univariate and multivariate analyses . In a 13-year period, 103 patients under 15 years of age underwent 120 LT; the mean age was 63 months and 36 % were under 2 years of age. Indications for LT were cholestatic disease in 68 (56 %), metabolic diseases in 18 (14 %), fulminant hepatic failure in 8 (7.5 %), cirrhosis in 7 (5.8 %), and retransplants in 17 (14 %). Whole liver was transplanted in 79 % of cases and partial liver in 21 %. Actuarial survival at 1, 5, and 10 years was 70 %, 61 %, and 57 %, respectively. United Network of Organ Sharing (UNOS) I recipients (RR = 2.7), primary non-function (PNF) (RR = 13.9), and hepatic artery thombosis (HAT) (RR = 3.8) were independent factors for lower patient survival in multivariate analysis. Thus, in our experience, postoperative mortality as a consequence of the patient's condition before transplantation, or complications such as PNF or HAT, are the major causes of decreased survival in pediatric LT.
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  • 96
    ISSN: 1432-2277
    Keywords: Key words Liver transplantation ; Posttransplant lymphoproliferative disease ; Epstein-Barr virus ; Humans ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Epstein-Barr virus (EBV) infection can induce uncontrolled lymphocyte B proliferation in immunosuppressed transplant patients. Monitoring circulating EBV-infected lymphocytes can help in identifying patients at risk of posttransplant lymphoproliferative disease (PTLD). Circulating EBV genome levels were determined in 54 liver transplant pediatric recipients. Ten patients had more than 500 EBV genome/105 peripheral blood lymphocytes (PBL) and exhibited clinical manifestations of EBV infection; three developed PTLD. To treat EBV infection, the level of immunosuppression was reduced and acute rejection developed in 4 patients. Three were treated with steroid and one had to be switched from cyclosporine to tacrolimus. Treatment of acute rejection was associated with increases in circulating EBV genome. None of the patients with less than 500 EBV genome/105 PBL developed PTLD or EBV infection. Monitoring of EBV DNA is useful in the management of EBV infection and PTLD following pediatric liver transplantation. EBV infection should be treated in ways which do not expose patients to the risk of rejection.
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  • 97
    ISSN: 1433-0350
    Keywords: Keywords Intractable epilepsy ; Epilepsy surgery ; Outcome ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Surgical experience with long-term follow-up is limited in childhood intractable epilepsy, compared with adult epilepsy. To assess the role of each presurgical evaluation modality and to identify prognostic factors for favorable seizure control after epilepsy surgery, 38 children with intractable epilepsy who underwent surgery were retrospectively reviewed. Among the available preoperative evaluation modalities, PET and neuropsychological testing showed the highest rates of positive results, whereas MRI was the most concordant with EEG findings. During a follow-up period of at least 12 months, 26 of the 38 patients showed favorable seizure control (Engel classifications I and II). The best seizure control was achieved in patients with a temporal resection and discrete lesion on magnetic resonance imaging. In spite of the invasive study, the less satisfactory results followed an extratemporal resection. We conclude that epilepsy surgery benefits children with intractable epilepsy and that the role of invasive study should be re-established according to the area of resection and presence of discrete lesion on MRI.
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  • 98
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    Child's nervous system 16 (2000), S. 735-741 
    ISSN: 1433-0350
    Keywords: Keywords Glioma ; Children ; Review
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The management and prognosis of gliomas are significantly different in children and in adults. Fortunately, the proportion of gliomas that are malignant is smaller in children than in the adult population. The different types of gliomas encountered in the pediatric population are reviewed, taking account of the most recent contributions on this subject. The importance of considering both localization and histological classification for better definition of the prognostic factors of each subgroup is emphasized. A brief review of the possible causes of gliomas is presented. Unfortunately, the information obtained by molecular and genetic study of these tumors has still not resulted in anything that can help the children suffering from gliomas in a concrete way. Surgery, with all its recent refinements, remains the best treatment for the majority of benign gliomas providing they can be removed without unacceptable sequelae. The role of chemotherapy has emerged recently for the treatment of nonresectable low-grade gliomas, such as hypothalamic- chiasmatic tumors, especially for infants, in whom the adverse effects of radiotherapy can be severe and irreversible. On the eve of the new millennium, there is renewed hope that the problem of malignant gliomas will be solved in the not-too-distant future.
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  • 99
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    Child's nervous system 16 (2000), S. 809-820 
    ISSN: 1433-0350
    Keywords: Keywords Pediatric neurosurgery ; Molecular biology ; Genetics ; Novel therapeutics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The new millennium beckons for novel advances in the diagnosis and treatment of pediatric neurosurgical conditions. Almost every aspect of pediatric neurosurgery has changed over the last decade. Undoubtedly with the application of knowledge in molecular biology to human disease many aspects of neurosurgery, especially neuro-oncology and the field of neuro-developmental anomalies, will change appreciably over the next decade. Overall, the trend in surgery in general and neurosurgery in particular is toward less invasive procedures and possibly non-surgical interventions. This review will briefly cover many of the important areas of pediatric neurosurgery. We will describe the state-of-the-art of our subspecialty and discuss possible future directions.
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  • 100
    ISSN: 1433-0350
    Keywords: Key words Botulism ; Electrophysiology ; Blink reflex ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Electrophysiological studies of a child with presumed botulism showed that the amplitude of the serially and electrically elicited blink reflexes Rl, R2 and R2’ was reduced during recovery. These findings suggest a conduction block of the facial nerves. Other nerve conduction studies and an incremental response to repetitive stimulation demonstrated a block of the presynaptic neuromuscular transmission. Results of the biological tests were negative, but those of electrodiagnosis and clinical examination favored a diagnosis of botulism. A combination of electrically elicited blink reflexes and rapid repetitive stimulation of the peripheral nerves was found to be a sensitive method of assessing the integrity of neuromuscular junctions and the subclinical impairment of muscle nerves.
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