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  • 1
    Electronic Resource
    Electronic Resource
    The role of fibrin tissue adhesives in surgery of haemophilia patients (1998)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 4 (1998), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1998.440443.x
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  • 2
    Electronic Resource
    Electronic Resource
    Functional kinesiology in haemophilia, an area yet to be explored (1998)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 4 (1998), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary. In haemophilic arthropathy there is a progressive limitation of the range of motion (ROM) which may lead to disabilities in the activities of daily living (ADL). In the literature the pathology of haemophilic arthropathy is described extensively, but only one paper describing functional limitations caused by limited range of motion (LOM) in haemophilia was found. The aim of the pilot study was to estimate on theoretical grounds, how many patients with haemophilia might suffer from functional disabilities. Material: ROM of elbows, knees and ankles of 155 Haemophilia A and B patients. Methods: flexion and extension were measured with an ordinary goniometer. The ROM of joints of patients with haemophilia was compared with normal values. Results: 39 of 155 patients had a normal ROM in both elbows; 22 in one elbow; 34 patients had disabilities in ADL with both arms; 14 with one arm; 18 were able to compensate; 89 had no problems; 79 of 155 patients had a near normal ROM of both knees; 38 patients could not ride an ordinary bicycle. Conclusion: only limited data are available concerning the normal ROM needed for individual ADL. Until additional data are available, it is not possible to predict which patients will be disabled in their activities of daily living and individual counselling should be done during the yearly outpatient comprehensive care clinics. Conservative and surgical measures should be taken to ensure elbow flexion of at least 120° and knee flexion of 100° for Western societies. In Asian countries patients with haemophilia need maximum knee flexion and ankle dorsi flexion.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1998.440524.x
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  • 3
    Electronic Resource
    Electronic Resource
    Orthopaedic surgery in haemophilia — The Tel Hashomer experience: a tribute to Professor Henri Horoszowski (1998)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 4 (1998), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1998.00044.x
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    α 1-Acid glycoprotein in gastric cancer juice (1975)
    Springer
    Journal of molecular medicine 53 (1975), S. 139-141 
    Details
    ISSN: 1432-1440
    Keywords: α 1-Acid glycoprotein ; neutralized gastric juice ; gastric cancer ; Sauresα 1-Glykoprotein ; neutralisierter Magensaft ; Magencarcinom
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Magensaft wurdein vivo während einer 3 min Sekretionsperiode mit 80 ml eines58Co-Cyanocobolamin markierten Phosphatpuffers neutralisiert. Der aspiriertein vivo neutralisierte Magensaft (nGJ) wurde in der doppelten Geldiffusion mit Hilfe spezifischer Antiseren auf das Vorkommen von Plasmaproteinen untersucht. Im nGJ wurdeα 1-saures Glykoprotein (AGP) in 36 von 38 Patienten mit Magencarcinomen nachgewiesen. In einer geringeren Häufigkeit fand sich AGP im nGJ von folgenden Krankheitsgruppen: Billroth II-Resektionen (6/15), metaplastische Gastritis (11/52), Ulcus ventriculi (3/24), chronisch atrophische Gastritis (2/26) und chronische Gastritis (3/63). AGP wurde nicht nachgewiesen bei Personen mit Oberflächengastritis (0/38), mit normaler Acidität (0/45) und in der Kontrollgruppe (0/21). Das im nGJ vorhandene AGP war mit „tumorassoziierten Antigenen“ des Intestinaltraktes nicht identisch. AGP zeigte in 7 von 17 untersuchten Proben einen immunologischen Unterschied im Vergleich zu dem Serum-AGP.
    Notes: Summary Gastric juice was neutralized (nGJ)in vivo by 80 ml of a phosphate buffer containing radiolabelled vitamin B12 as dilution indicator. Unprocessed nGJ was analyzed in the double gel diffusion technique for the presence of serum proteins using monospecific antisera.α 1-Acid glycoprotein (AGP) was found in a high incidence (36 out of 38 subjects) in nGJ of gastric cancer patients. AGP was also observed less frequently in nGJ of patients with Billroth II resections (6/15), metaplasia (11/52), gastric ulcer (3/24), chronic atrophic gastritis (2/26) and chronic gastritis (3/63). AGP was absent in the control group (0/21), in patients with surface gastritis (0/38) and in subjects with normal acid secretion (0/45). Immunochemical studies demonstrated no identity of AGP with human “gastrointestinal tumor associated antigens.” In 7 out of 17 AGP positive samples immunochemical differences between gastric and serum AGP were observed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01466718
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  • 5
    Electronic Resource
    Electronic Resource
    Knochenmarktransplantation im Erwachsenenalter bei akuter Leukämie, aplastischer Anämie und paroxysmaler nächtlicher Hämoglobinurie (1983)
    Springer
    Journal of molecular medicine 61 (1983), S. 321-328 
    Details
    ISSN: 1432-1440
    Keywords: Bone-marrow-transplantation ; Acuteleukemia ; Aplastic-anemia ; Paroxysmal-nocturnal-hemoglobinuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Eleven adults have been transplanted for various reasons between July 1979 and July 1982: 2 with aplastic anemia (AA), 1 with paroxysmal nocturnal hemoglobinuria (PNH), 8 with acute leukemia (AL). Four patients suffered from acute lymphocytic leukemia (ALL) and four from acute non-lymphocytic leukemia (ANLL). Two of them were transplanted in relapse, 1 in a partial remission, and 5 in complete remission. All patients were in their late stage of disease. The PNH-patient had an identical twin, 8 patients had an HLA- and MLC compatible sib, 1 an unrelated donor, and 1 was transplanted from his father. Four patients are alive, 2 more than 3 years: 1 with AA and 1 with ALL who was transplanted in relapse. Six patients died of infectious complications (4 of interstitial pneumonia, 1 of a candidasepsis, 1 of acute toxoplasmosis). Patients living more than 3 weeks had a take. Acute graft-versus-host (GvH) disease did not present a major problem. All patients received methotrexate for GvH-prophylaxis, in three instances the marrow was additionally pre-incubated with anti-T-cell globulin.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01485022
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  • 6
    Electronic Resource
    Electronic Resource
    Genetic analysis of the interethnic difference between Chinese and Caucasians in the polymorphic metabolism of debrisoquine and codeine (1991)
    Springer
    European journal of clinical pharmacology 40 (1991), S. 553-556 
    Details
    ISSN: 1432-1041
    Keywords: Genetic polymorphism ; Cytochrome P450 ; drug metabolism ; codeine ; interethnic differences ; Chinese ; debrisoquine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Medicine
    Notes: Summary The Far Eastern and Caucasian populations are strikingly different with respect to the debrisoquine/sparteine hydroxylation polymorphism. The number of poor metabolizers, as defined for Caucasians, is very low among Chinese and Japanese. We investigated the molecular basis for this difference by analysis of the CYP2D6 gene in 115 Chinese subjects, combined with phenotypic classification of codeine and debrisoquine metabolism. A correlation between the rates of metabolism of these two drugs and genotype, as analyzed by RFLP using XbaI, was observed among the Chinese. A high frequency (37%) of alleles indicative of gene insertions (reflected by Xba I 44kb fragments) was recorded in the Chinese, but was not associated with the poor metabolizer phenotype, as it is in Caucasians. PCR amplification of part of the CYP2D6 gene with mutation specific primers for CYP2D6A (29A) and CYP2D6B (29B) allelic variants revealed that the XbaI 44kb fragment in Chinese apparently contains a functional CYP2D6 gene, in contrast to the situation among Caucasians. The results provide a molecular explanation of the interethnic difference in the metabolism of drugs affected by the debrisoquine hydroxylation polymorphism.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00279968
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  • 7
    Electronic Resource
    Electronic Resource
    Acetylation of Serotonin in the Rabbit Pineal Gland: An N-Acetyltransferase with Properties Distinct from NAT1 and NAT2 Is Responsible (1991)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 57 (1991), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Two rabbit arylamine N-acetyltransferases (NAT1 and NAT2, EC 2.3.1.5) have been cloned and characterized recently in this laboratory. They catalyze the acetylation of primary arylamine and hydrazine drugs and other substrates in the liver, including sulfamethazine, ρ-aminosalicylic acid, and ρ-aminobenzoic acid. In the pineal gland, serotonin is metabolized to N-acetylserotonin by an unknown N-acetyltransferase. Similarity of the liver enzymes and the pineal gland arylalkylamine N-acetyltransferase (AA-NAT) has been suggested, because pineal gland homogenates were shown to metabolize arylamine substrates as ρ-phenetidine, aniline, or phenylethylamine, and liver homogenates or partially purified liver enzyme preparations catalyzed the N-acetylation of serotonin. The present study was undertaken to elucidate the possible role of NAT1 or NAT2 in serotonin acetylation in the pineal gland. We transiently expressed rNAT1 and rNAT2 genes in COS cells, studied the kinetics of the enzymes produced with various substrates, and compared these data with activities of rabbit pineal glands and livers. These enzymatic studies were complemented with western blot analysis with antibodies against NAT1 and NAT2. Cross-hybridization of rNAT1 or rNAT2 to the gene for the pineal gland AA-NAT was tested by Southern blot studies of genomic rabbit DNA. Our results indicate that although NAT1 is expressed in the pineal gland, it is not involved in the physiologically important step of N-acetylation of serotonin.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1991.tb08266.x
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  • 8
    Electronic Resource
    Electronic Resource
    Unsolved problems in haemophilia (1998)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 4 (1998), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary. Diagnosis and therapy for the vast majority of haemophiliacs in the world remain beyond their reach. Health care costs for hemophilia replacement products keep rising. Global inequities for financing health care result in most hemophiliacs being undertreated. The transfusion-transmitted disease, factor VIII inhibitors, is common. Its predictability escapes detection. Management has progressed substantially and offers many therapeutic modalities. Programs for prophylaxis or immune tolerance induction are impossible for most patients. Thus, the challenge for haemophiliacs is to attain these goals.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1998.440341.x
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  • 9
    Electronic Resource
    Electronic Resource
    When should prophylactic treatment in patients with haemophilia A and B start?— The German experience (1998)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 4 (1998), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary. Radiological and orthopaedic outcome in severe and moderate haemophilia A and B patients undergoing long-term prophylactic treatment were prospectively investigated focusing on the age of onset of prophylaxis and the number of joint bleedings prior to treatment. We report on 21 patients with severe and moderate haemophilia A and B receiving prophylactic treatment of between 3.1 and 16.1 year's duration. Three patient groups were evaluated according to the age at onset of prophylaxis. In group I prophylactic treatment was initiated in the first 2 years of life. Patients in group II received prophylaxis at the age of 3–6 years. Late-onset or secondary prophylactic treatment was started at the age of 6 years and above in seven patients (group III). All patients received virus-inactivated F VIII or F IX concentrates at dosages of 30–50 IU/kg body weight i.v. three times per week for those with haemophilia A and twice per week for those with haemophilia B. Elbow, knee and ankle joints were investigated at 3–4-yearly intervals according to the radiological and orthopaedic scores recommended by the World Federation of Haemophilia. The total number of joint bleedings before and after start of prophylaxis were recorded in all patients. In group I 7/8 patients had unaffected joints with constant radiological and orthopaedic scores of zero or 1, after a median of 11.25 years of prophylactic treatment. One patient in this group demonstrated mild radiological alterations (score 4). Patients in group II showed neither radiological nor orthopaedic alterations at study entry. Surprisingly, worsening joint scores could be detected despite ongoing prophylaxis after the 3-year interval (median orthopaedic score 4, median radiological score 8). Treatment group III already showed considerable joint damage at study entry with a median radiological score of 11 (0–33) and a median orthopaedic score of 4 (0–11). Despite prophylactic treatment, both radiological (median 19.5, range 2–47) and orthopaedic scores (median 8, range 2–12) deteriorated after 3 years. Prior to onset of prophylaxis, no or only one joint bleeding occurred in treatment group I. In group II, a median of six joint bleeds (range 1–8) was reported before prophylaxis was started. Patients in group III usually experienced a median of more than 10 joint haemorrhages (range 6–10 or more). Under prophylactic treatment the number of joint bleedings decreased significantly in group II and III. However, radiological and orthopaedic scores increased as a sign of progressing osteoarthropathic alterations in patients reporting more than five joint haemorrhages before onset of prophylaxis whereas no joint alterations could be assessed in patients with no or only one joint bleeding episode prior to prophylaxis. Even a small number of joint bleedings seems to cause irreversible osteoarthropathic alterations leading to haemophilic arthropathy. Once apparent, further progression of joint damage could not be arrested despite of prophylactic treatment (groups II and III). In order to prevent haemophilic arthropathy, effective prophylaxis should be started before or at least after the first joint bleeding in severe haemophilia A and B.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1998.440413.x
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  • 10
    Electronic Resource
    Electronic Resource
    Guidelines on treatment of haemophilia in Sweden (1998)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 4 (1998), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1998.4404214.x
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