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  • 1
    Electronic Resource
    Electronic Resource
    Geographic distribution and origin of CFTR mutations in Germany (1996)
    Springer
    Human genetics 〈Berlin〉 97 (1996), S. 727-731 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract The geographic distribution and origin of CFTR mutations in Germany was evaluated in 658 three-generation families with cystic fibrosis (CF). Fifty different mutations were detected on 1305 parental CF chromosomes from 22 European countries and overseas. The major mutation ΔF508 was identified on 71.5% of all CF chromosomes, followed by R553X (1.8%), N1303K (1.3%), G542X (1.1%), G551D (0.8%) and R347P (0.8%). According to the grandparents’ birthplace, 74% of CF chromosomes had their origin in Germany; the ΔF508 percentage was 77%, 75%, 70% and 62% in northern, southern, western and eastern Germany, respectively. Ten or more mutant alleles in the investigated CF gene pool originated from Austria, the Czech Republic, Poland, Russia, Turkey and the Ukraine. This widespread geographic origin of CFTR mutations in today’s Germany reflects the many demographic changes and migrations in Central Europe during the 20th century.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004390050128
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Cisapride reduces postoperative gastrocaecal transit time after cardiac surgery in children (1996)
    Springer
    Intensive care medicine 22 (1996), S. 977-980 
    Details
    ISSN: 1432-1238
    Keywords: Key words Enteral nutrition ; Critical illness ; Prokinetic drugs
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Objective: To investigate the influence of the prokinetic drug cisapride on gastrocaecal transit time (GCTT) in children after open heart surgery. Design: Prospective, randomized and controlled study. Setting: Interdisciplinary paediatric intensive care unit in a tertiary-care children‘s hospital. Patient: Twenty-one children with a median age of 6.2 years on day 1 after uncomplicated open heart surgery for isolated septal defects, acquired mitral or aortic valve disease or tetralogy of Fallot. Control group consisting of 10 healthy children with a median age of 8.1 years. Interventions: Ten children were randomized to receive cisapride 0.2 mg/kg body weight, 30 min prior to measurement of GCTT. Measurements and results: GCTT was measured using hydrogen breath testing with a test solution containing lactulose and mannitol (0.4 g/kg and 0.1 g/kg body weight respectively). GCTT was markedly delayed in all patients compared to the control group. Within 8 h 8/10 patients in the treatment group versus 4/11 patients in the non-cisapride group achieved gastrocaecal transit. No adverse side-effects were observed. Conclusions: Cisapride accelerates gastrocaecal transit after open heart surgery in children. In intensive care patients on inotropic support or opioid medication, it may facilitate the earlier reintroduction of enteral feeding.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001340050198
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Cisapride reduces postoperative gastrocaecal transit time after cardiac surgery in children (1996)
    Springer
    Intensive care medicine 22 (1996), S. 977-980 
    Details
    ISSN: 1432-1238
    Keywords: Enteral nutrition ; Critical illness ; Prokinetic drugs
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Objective To investigate the influence of the prokinetic drug cisapride on gastrocaecal transit time (GCTT) in children after open heart surgery. Design Prospective, randomized and controlled study. Setting Interdisciplinary paediatric intensive care unit in a tertiary-care children's hospital. Patient Twenty-one children with a median age of 6.2 years on day 1 after uncomplicated open heart surgery for isolated septal defects, acquired mitral or aortic valve disease or tetralogy of Fallot. Control group consisting of 10 healthy children with a median age of 8.1 years. Interventions Ten children were randomized to receive cisapride 0.2 mg/kg body weight, 30 min prior to measurement of GCTT. Measurements and results GCTT was measured using hydrogen breath testing with a test solution containing lactulose and mannitol (0.4 g/kg and 0.1 g/kg body weight respectively). GCTT was markedly delayed in all patients compared to the control group. Within 8 h 8/10 patients in the treatment group versus 4/11 patients in the noncisapride group achieved gastrocaecal transit. No adverse side-effects were observed. Conclusions Cisapride accelerates gastrocaecal transit after open heart surgery in children. In intensive care patients on inotropic support or opioid medication, it may facilitate the earlier reintroduction of enteral feeding.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02044127
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    Paper (German National Licenses)
    Fulltext
  • 4
    Electronic Resource
    Electronic Resource
    Geographic distribution and origin of CFTR mutations in Germany (1996)
    Springer
    Human genetics 〈Berlin〉 97 (1996), S. 727-731 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract The geographic distribution and origin of CFTR mutations in Germany was evaluated in 658 three-generation families with cystic fibrosis (CF). Fifty different mutations were detected on 1305 parental CF chromosomes from 22 European countries and overseas. The major mutation ΔF508 was identified on 71.5% of all CF chromosomes, followed by R553X (1.8%), N1303K (1.3%), G542X (1.1%), G551D (0.8%) and R347P (0.8%). According to the grandparents' birthplace, 74% of CF chromosomes had their origin in Germany; the ΔF508 percentage was 77%, 75%, 70% and 62% in northern, southern, western and eastern Germany, respectively. Ten or more mutant alleles in the investigated CF gene pool originated from Austria, the Czech Republic, Poland, Russia, Turkey and the Ukraine. This widespread geographic origin of CFTR mutations in today's Germany reflects the many demographic changes and migrations in Central Europe during the 20th century.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02346181
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Intestinal adaptation in short-bowel syndrome (1989)
    Springer
    European journal of pediatrics 148 (1989), S. 294-299 
    Details
    ISSN: 1432-1076
    Keywords: Short bowel syndrome ; Intestinal adaptation ; Trophic substances
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract After massive resection of the small intestine the remannt mucosa has an important capacity to enlarge the absorptive surface for the digestion, hydrolysis and absorption of nutrients. This intestinal adaptation is achieved by the interaction of various factors. Oral nutrients together with pancreatic biliary secretions stimulate the mucosa to become hyperplastic. Secondary to these luminal factors hormones play an important role in the adaptive process. Among the hormones, enteroglucagon is the most important growth promoting agent together with other growth factors such as epidermal growth factor, prostaglandin E2 and human growth hormone analogues, e.g. plerocercoid growth factor from the plerocercoid larvae of the tapeworm Spirometra mansonoides. The intestinal enterocyte is the target of these factors and within the cell the synthesis of polyamines, which are responsible for rapid growth, is the most essential step for the development of hyperplasia after resection. The rate limiting enzyme for polyamine synthesis ornithine decarboxylase (ODC) reacts to trophic stimuli with an increased activity. Thereafter rapid accumulation of tissue polyamines occurs. Blockade of ODC by specific inhibitors is accompanied by absence of intestinal hyperplasia after resection. Therefore it is concluded that ODC plays a key role in the intestinal adaptation of the remnant small bowel. To start and enhance intestinal hyperplasia after resection in patients with short bowel syndrome introduction of oral nutrition as soon as possible after operation is very important. On account of gastric acid hypersecretion the use of H2 receptor blocking agents is recommended. A decreased intestinal transit time is treated with loperamide. Adequate nutritional support by enteral and (home) parenteral feeding is the prerequisite for the initiation and development of intestinal adaption in short bowel syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00444117
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    Paper (German National Licenses)
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  • 6
    Electronic Resource
    Electronic Resource
    IgG, IgA and IgE gliadin antibody determinations as screening test for untreated coeliac disease in children, a multicentre study (1989)
    Springer
    European journal of pediatrics 148 (1989), S. 496-502 
    Details
    ISSN: 1432-1076
    Keywords: Diagnostic value ; Gliadin IgG, IgA, and IgE antibody determinations ; Coeliac disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The diagnostic value of gliadin IgG, IgA and IgE antibody (AB) determinations using the fluorescent immunosorbent test was examined in 586 children with malabsorptive disorders and/or failure to thrive. All patients underwent jejunal biopsy and were on a gluten-containing diet. IgG AB were found in all patients (331/331) with untreated coeliac disease (CD) in our study, but IgA AB in only 295/331 (89%). Therefore a screening test based only on IgA AB determinations is not recommended. By contrast, 203 (80%) of 255 children with other malabsorptive disorders had no gliadin AB, 43 (16.5%) had only IgG AB and only 9 (3.5%) had IgG and IgA AB. IgE AB proved to be of no additional value as a diagnostic tool because they were found in a quarter of the children without CD. Statistical evaluation of combined IgG and IgA AB determination showed at least 96% sensitivity and a specificity of 97%. The subjective (“Bayesian”) probability that an actual patient with a given AB test result has CD, is considered: a patient very probably has CD in the case of positive IgG and IgA AB, and no CD in the case of a negative AB result. In the case of negative IgA AB but positive IgG AB the physician's judgement (“prior probability”) influences the (“posterior”) probability of CD for an actual patient. In contrast to IgG AB, IgA AB decline rapidly after the introduction of a gluten-free diet and may be used for diet control after diagnosis. Antibodies against cow's milk proteins, though present in 72% of the 331 patients with CD, are of no therapeutic significance in CD and are of no value for its diagnosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441541
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    Paper (German National Licenses)
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  • 7
    Electronic Resource
    Electronic Resource
    Prenatal diagnosis of cystic fibrosis: False negative result with the 4-methylumbelliferyl-p-guanidinobenzoate assay for proteases in amniotic fluid (1982)
    Springer
    European journal of pediatrics 139 (1982), S. 35-38 
    Details
    ISSN: 1432-1076
    Keywords: Cystic fibrosis ; Prenatal diagnosis ; MUGB-reactive protease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The promising method for the prenatal detection of cystic fibrosis (CF), developed by Naldler's group [6,7], has recently attracted considerable interest but there is a lack of confirmatory reports from other laboratories. We have established the assay for methylumbelliferyl-guanidinobenzoatereactive proteases (MUGB assay) and used it, together with isoelectric focusing and gel filtration, to screen retrospectively 84 second and third trimester amniotic fluids, one of which was known to be from a CF pregnancy. The control values for MUGB-reactive protease were 4.25±1.36 and 6.12±1.04 nmoles/mg protein (mean±1 SD) for second and third trimester fluids, respectively, compared to 5.64 nmoles/mg protein for the third trimester CF fluid. Gel filtration resolved two peaks of MUGB-reactive protease of approximately 50 000 and 10 000 molecular weight from all samples. Insufficient CF fluid remained for isoelectric focusing and the results obtained with normal amniotic fluids were inconclusive due to a highly variable background staining. Thus, with two methods a false-negative result has been obtained, suggesting that further refinement of these techniques may be required before they can be routinely applied for the prenatal diagnosis of CF.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442076
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    Paper (German National Licenses)
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  • 8
    Electronic Resource
    Electronic Resource
    Megaloblastic anaemia in one of monozygous twins breast fed by their vegetarian mother (1986)
    Springer
    European journal of pediatrics 145 (1986), S. 570-571 
    Details
    ISSN: 1432-1076
    Keywords: Alimentary vitamin B12 deficiency ; Vegetarianism ; Megaloblastic anaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Megaloblastic anaemia in infancy is uncommon in western countries. We describe a case of an exclusively breast-fed monozygous twin with severe vitamin B12 deficiency with haematologic and neurologic abnormalities. Treatment with vitamin B12 resulted in a rapid haematological and clinical improvement.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02429070
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    Paper (German National Licenses)
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  • 9
    Electronic Resource
    Electronic Resource
    Crohn disease with sclerosing cholangitis and liver cirrhosis in adolescence (1991)
    Springer
    European journal of pediatrics 150 (1991), S. 557-559 
    Details
    ISSN: 1432-1076
    Keywords: Sclerosing cholangitis ; Crohn disease ; Liver cirrhosis ; Adolescence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 17-year-old patient suffering from Crohn disease (CD) and liver cirrhosis is presented. At an advanced stage of the disease, he died of a concomitant urosepsis. Autopsy showed that the liver cirrhosis was caused by sclerosing cholangitis. This very rare complication of CD in adolescence is discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02072206
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    Paper (German National Licenses)
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  • 10
    Electronic Resource
    Electronic Resource
    A 15-year-old girl with a large lumbosacral abscess (1999)
    Springer
    European journal of pediatrics 158 (1999), S. 1003-1004 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310051267
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