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  • 1
    ISSN: 1432-1076
    Keywords: Acrodermatitis enteropathica ; Small bowel ; Oxyquinoline ; Metabolism of fatty acids ; Paneth cells ; Zinc deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 2 Geschwistern, einem Jungen und einem Mädchen, wurde die Diagnose der Acrodermatitis enteropathica (AE) im Alter von 10 bzw. 6 Wochen gestellt. Bis auf eine Blutsverwandtschaft, die 5 Generationen zurückliegt, ist die Familienanamnese anauffällig. Beide Kinder boten bei Diagnosestellung die für AE typischen Hautveränderungen. Symptome von seiten des Magen-Darm-Kanals waren weniger ausgeprägt und traten mit zunehmendem Alter in den Hintergrund. Bei dem jüngeren Patienten konnten während eines Rezidivs eine Erniedrigung des Serumspiegels der Ölsäure (18:1) sowie eine leichte Vermehrung der Linolsäure (18:2) festgestellt werden, während die Arachidonsäure (20:4) vermindert war. Bei beiden Patienten waren die Serumzinkspiegel mit und ohne Oxychinolinbehandlung deutlich erniedrigt. Eine Substitutionstherapie mit Zinkaspertat brachte weder eine klinische Besserung noch einen wesentlichen Anstieg des Serumzinkspiegels. Durch Gabe von Zinksulfat konnten eine völlige Remission der Hautveränderungen sowie eine weitgehende Normalisierung der Serumzinkspiegel erreicht werden. Untersuchungen der Dünndarmschleimhaut zeigten lichtmikroskopisch keine Besonderheiten. Elektronenmikroskopisch fanden sich in den Enterocyte sogenannte multi-vesicular bodies. Die Paneth-Zellen zeigten teilweise unregelmäßig geformte, inhomogene Strukturen im Cytoplasma. Die pleomorphen Sekretgraula wanen plump und groß und wiesen eine ausgeprägte Heteromorphie ihrer Matrix auf. Da bei Ratten unter zinkarmer Ernährung ähliche ultrastrukturelle Veränderungen bekannt sind, muß durch weitere Untersuchungen geklärt werden, ob die Veränderungen in den Paneth-Zellen typisch für die AE sind. Die adäquate Therapie der AE besteht zur Zeit in der Substitution von Zink. Dadurch wurde die mit toxischen Augenschädigungen belastete Therapie mit Oxychinolinen überflüssig.
    Notes: Abstract Acrodermatitis enteropathica (AE) was diagnosed in 2 siblings, boy and girl, at the age of 10 and 6 weeks. The family history is unremarkable except for consanguinity 5 generations previously. The clinical symptoms of the 2 patients conformed to the known features of AE, the gastrointestinal involvement loosing its significance with increasing age. In one patient in a stage of exacerbation the serum level of oleic acid (18:1) was lowered and of linoleic (18:2) acid slightly increased while that of arachidonic acid was decreased (Fig. 4). In both patients the serum zinc levels were significantly lowered. Under substitution with ZnSO4 the clinical condition improved and the serum zinc levels returned to normal. Histologically the small bowel mucosa was practically normal. Ultrastructural examination of jejunal biopsies revealed rather unspecific changes in the enterocytes in the form of numerous multivesicular bodies. The Paneth cells sometimes contained irregularly formed inhomogeneous structures within their cytoplasm. In addition the secretory granules varied in size and displayed a granular heteromorphic matrix. Frequently they were confluent and formed giant granules.
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 125 (1977), S. 153-162 
    ISSN: 1432-1076
    Keywords: Acrodermatitis enteropathica therapy ; Small bowel ; Zinc malabsorption ; Zinc deficiency ; Paneth cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Als Ursache der klinischen Erscheinungen bei der Akrodermatitis enteropathica (A.E.) muß ein Zinkmangel als Folge einer Zinkmalabsorption angesehen werden. Durch orale Substitution mit Zink lassen sich die Krankheitserscheinungen beseitigen. Es wird über 2 Geschwister mit A.E. berichtet, die lange Zeit mit Oxychinolinpräparaten mit wechselndem Erfolg vorbehandelt waren. Durch orale Substitution mit Zinksulfat in der Dosis von 110–220 mg täglich wurde eine vollständige und anhaltende Remission erzielt. Gleichzeitig normalisierten sich die vorher stark erniedrigten Serumzinkspiegel. Keine so strenge Korrelation bestand zwischen dem Zinkgehalt der Haare und der Art Der Zinksubstitution. — Wie wir in unserer 1. Mitteilung zeigten, lassen die Paneth-Zellen der Dünndarmmukosa bei A.E. ultrastrukturelle Veränderungen in Form von inhomogenen Strukturen des Zytoplasmas, der Bildung von Riesengranula und Einschlüssen erkennen. Unter der Zinksubstitution kommt es zu einer vollkommenen Normalisierung des ultrastrukturellen Bildes der Paneth-Zellen. Die Veränderungen an den Paneth-Zellen können daher nur die Folge, jedoch nicht die Ursache des Zinkmangels bzw. der Zinkmalabsorption sein.
    Notes: Abstract The basic defect in acrodermatitis enteropathica (A.E.) is zinc deficiency caused by zinc malabsorption. The clinical symptoms disappear and serum zinc levels normalize after oral treatment with zinc. A report is given on two siblings suffering from A.E., both treated with oxyquinolines for a long period with changing clinical success. A permanent clinical remission could be achieved by treatment with zinc-sulphate at doses of 110–220 mg daily. The serum zinc levels normalized. The correlation between the zinc concentration of the hair and the kind of therapy was not very close. As we have shown in our first communication, the Paneth cells of the intestinal mucosa display ultrastructural changes in form of an unhomogeneous structure of the cytoplasm, formation of giant granules, and inclusion bodies. The zinc-therapy led to a complete normalization of the pathological changes in the Paneth cells. Thus, the changes in the Paneth cells in A.E. are the result and not the cause of zinc deficiency.
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford BSL : Blackwell Science Ltd
    Clinical & experimental allergy 30 (2000), S. 0 
    ISSN: 1365-2222
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Allergic diseases are more prevalent in affluent countries, which has been attributed to life-style factors. Life-style habits may also differ between socioeconomic (SES) classes. The objective of this paper therefore was to evaluate if SES had an impact on the development of atopic disorders.〈section xml:id="abs1-2"〉〈title type="main"〉MethodsA total of 1314 German children were followed-up in an observational birth cohort study to 6 years of age. Parents filled in questionnaires, and had multi-allergen screening tests for sensitization. Indoor allergen concentrations were determined by ELISA. Children were examined regularly up to 6 years, specific serum IgE values were determined by CAP-Rast-Feia.〈section xml:id="abs1-3"〉〈title type="main"〉ResultsThe risk of aeroallergen sensitization (odds ratio 1.76; 95% CI 1.30–2.37), and the lifetime prevalence of hay fever (2.36; 1.76–3.17), and asthma (1.74; 1.08–2.80), but not of atopic dermatitis (AD: 0.90; 0.54–1.51) was elevated in parents of high compared to low SES. With high SES the risk of smoking in pregnancy (0.35; 0.23–0.51), in the home (0.31; 0.21–0.46), pet ownership (0.37; 0.26–0.55), high mite (0.42; 0.25–0.74), and high cat (0.38; 0.18–0.82) allergen concentration in house dust was reduced, but elevated for breastfeeding over more than 6 months (4.67; 2.9–7.48). In children, even after controlling for other risk factors, only the risk of AD from 3 to 6 years (2.42; 1.42–4.14) was elevated in families with high SES, but not of AD in infancy or of any other atopic disorder.〈section xml:id="abs1-4"〉〈title type="main"〉ConclusionsWhile parents of high SES have a higher prevalence of inhalative allergies, their favourable life-style prevents the development of atopic disorders in their children, except for AD beyond infancy.
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  • 4
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Clinical & experimental allergy 27 (1997), S. 0 
    ISSN: 1365-2222
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background Atopic family history and cord blood IgE have been used as predictors of atopic disease in newborns for about 20 years, but at least for cord blood IgE the sensitivity has been shown to be very low. The objective of this paper was to evaluate whether parental history and cord blood-IgE were more accurate predictors for the appropriate atopic phenotypes in the infants rather than for any atopy.Methods A total of 1314 newborn infants was recruited in six German obstetric departments in 1990 and followed-up for 2 years. Four hundred and ninty-ninc (38%) were at high risk for atopy with at least two first degree atopic family members and/or elevated cord-blood IgE concentrations.Results The cumulative incidence of atopic dermatitis over the first 2 years of life (AD24) amounted to 20. 1%, and there was a significant association with AD history of the mother (OR 2.5, 95%-Cl 1.46–4.26) and of the father (OR 3.53, 95%cC1 1.90–6.54). The cumulative incidence of recurrent wheezing in the first 2 years of life (RW24) amounted to 16.1%, and was positively associated with asthma history (OR 2.11, 95%CI 1.33–3.60) and sensitization history (OR 1.64, 95%C1 1.34–2.36) of the mother, but with neither for the father. RW24 was less prevalent in girls than in boys (OR 0.64. 95%Cl 0.47–0.89). Thirty-one per cent of infants were sensitized (CAP test value 〉 0.35 kU/L) against at least one of nine food or inhalative allergens (S24) and this was signilicantly associatcd with cord blood-IgE value (OR 2.43, 95%C1 1.69–3.49). and sensitization history of the mother (OR 1.64, 95%CI 1.18–2.41). Using multiple logistic regression analysis, the prediction of AD24 by AD of parents, of RW24 by asthma of parents, and of sensitization by cord blood IgE was of low accuracy.Conclusion The predictive capacity of parental history and cord blood IgE is not high enough to recommend them as screening instruments for primary prevention. The majority of atopic manifestations and of sensitization occur in infants with no demonstrable risk at birth.
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  • 5
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Clinical & experimental allergy 20 (1990), S. 0 
    ISSN: 1365-2222
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science, Ltd
    Clinical & experimental allergy 32 (2002), S. 0 
    ISSN: 1365-2222
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background The results of numerous studies on the influence of breastfeeding in the prevention of atopic disorders are often contradictory. One of the most important problems is confounding by other lifestyle factors.Objective The aim of the present study was to analyse the effect of any breastfeeding duration on the prevalence of atopic eczema in the first seven years of life taking into account other risk factors.Methods In an observational birth cohort study 1314 infants born in 1990 were followed-up for seven years. At 3, 6, 12, 18, 24 months and every year thereafter, parents were interviewed and filled in questionnaires, children were examined and blood was taken for in vitro allergy tests. Generalized Estimation Equations (GEE)-models were used to model risk factors for the prevalence of atopic eczema and for confounder adjustmentResults Breastfeeding was carried out for longer if at least one parent had eczema, the mother was older, did not smoke in pregnancy, and the family had a high social status. The prevalence of atopic eczema in the first seven years increased with each year of age (OR 1.05; 95% CI 1.01–1.09 for each year), with each additional month of breastfeeding (1.03; 1.00–1.06 for each additional month), with a history of parental atopic eczema (2.06; 1.38–3.08), and if other atopic signs and symptoms appeared, especially specific sensitization (1.53; 1.25–1.88), and asthma (1.41; 1.07–1.85). Although breastfeeding should be recommended for all infants, it does not prevent eczema in children with a genetic risk.Conclusion Parental eczema is the major risk factor for eczema. But in this study, each month of breastfeeding also increased the risk
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  • 7
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Pediatric allergy and immunology 4 (1993), S. 0 
    ISSN: 1399-3038
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: In order to identify newborns at risk for atopic diseases, we developed a family questionnaire and selected specific answers which were suitable to identify atopic family members. The validity of the questionnaire was evaluated by the Phadiatop lest results of 793 mothers and 353 fathers. As both screening instruments do not measure the same, the Phadiatop test identifies scnsitization to inhalant allergens and the history reflects the clinical manifestation of atopic disease, the agreement between sensitization and manifestation is incomplete. Sensitivity and specificity of the questionnaire screening conditions to reproduce the Phadiotop lest result was 64% and 84% for mothers, and 58% and 88% for fathers, respectively. The relative risk for lifetime prevalence of atopic manifestations in Phadiatop positive over negative mothers was calculated to be 3. 88 (95% confidence interval = 3. 12 to 4. 81), and for Phadiatop positive over negative fathers to amount 4. 84 (95% confidence interval 3. 25 to 7. 23). A few relevant answers of 20 were identified by logistic regression analysis to predict the Phadiatop test result nearly, as well as the total questionnaire.
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  • 8
    ISSN: 1399-3038
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1398-9995
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Objective:  A population study was performed to identify the prevalence of all kinds of adverse reactions to food.Methods:  In a representative cross-sectional survey performed in 1999 and 2000 in Berlin, 13 300 inhabitants of all ages were addressed by questionnaire. This questionnaire was answered by 4093 persons. All respondents mentioning any sign of food intolerance or the existence of allergic diseases (n = 2298) were followed up by telephone and, in case food intolerance could not be ruled out by patient history, were invited to attend to the clinic for personal investigation including double-blind, placebo-controlled food challenge tests (DBPCFC).Results:  The self-reported lifetime prevalence of any adverse reaction to food in the Berlin population (mean age 41 years) was 34.9%. Eight hundred and fourteen individuals were personally investigated according to the guidelines. The point prevalence of adverse reactions to food confirmed by DBPCFC tests in the Berlin population as a mean of all age groups was 3.6% (95% confidence interval [3.0–4.2%]) and 3.7% in the adult population (18–79 years, 95% confidence interval [3.1–4.4.%]). Two and a half percent were IgE-mediated and 1.1% non-IgE-mediated, females were more frequently affected (60.6%). Based on a statistical comparison with available data of adults from the nationwide German Health Survey from 1998, adverse reactions to food in the adult population of Germany (age 18–79) were calculated with 2.6% [2.1–3.2%]).Conclusions:  The study gives for the first time information about the point prevalence of both immunological and nonimmunological adverse reactions to food and underlines the relevance of this issue in public health. The data also show that an individualized stepwise approach including provocation tests is mandatory to confirm the diagnosis.
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  • 10
    ISSN: 1398-9995
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: For screening atopy risk in 6401 (84%) of all infants born during the year 1990 in six obstetric departments of five German cities, cord-blood IgE values were determined with CAP-RAST-FEIA. After cases with elevated IgA values had been excluded, 25 % of the values were above the detection limit of 0.35 kU/1, and 8.5% were above 0.9 kU/1. Boys had significantly higher values than girls (P≤0.001). The distribution of values was significantly different for different nationalities of mothers (P≤0.001). The percentage of elevated values (≥0.9kU/l) increased significantly with the number of close family members with atopic history (P≤0.001). Regarding the atopic history of the father, siblings, and mother separately, only the mother's history had a significant association with the cord-blood IgE class (P≤0.001). The IgE values of 81 twin pairs correlated significantly with a coefficient of r = 0.4909 (P≤0.001). The smoking history of the parents during pregnancy showed an association with cord-blood IgE values (P≤0.02). No significant association could be shown between cord-blood IgE distribution and other variables, i.e., gestational age, birth size, birth modus, Apgar score, cord-blood pH value, neonatal problems, parity, age of the mother, medication during pregnancy, educational level of mother or father, time of year, or obstetric department. It is hypothesized that, in addition to some postpartum contamination or placental transfer of maternal IgE, cord-blood IgE values are also determined by the fetal immunologic reaction to intrauterine exposure to allergens and trigger factors, and by genetic influences.
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