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  • 1
    Electronic Resource
    Electronic Resource
    Immunoglobulin deposits in the biliary remnants of extrahepatic biliary atresia: a study by immunoperoxidase staining in 128 infants (1981)
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 5 (1981), S. 0 
    Details
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Bile duct remnants taken from 128 infants during surgery for extrahepatic biliary atresia were paraffin embedded prior to immunoperoxidase staining. Immunoglobulin deposits were found in 44 remnants. They were made up of IgM alone in 25 cases and of IgM and IgG in 19. Deposits were observed along the basement membranes of glandular structures. These findings suggest that extrahepatic bilary atresia might be an acquired and evolving disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2559.1981.tb01779.x
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  • 2
    Electronic Resource
    Electronic Resource
    Glycogen storage disease: recommendations for treatment (1988)
    Springer
    European journal of pediatrics 147 (1988), S. 226-228 
    Details
    ISSN: 1432-1076
    Keywords: Glycogen storage disease ; Glucose-6-phosphatase ; Glucose-6-phosphate translocase ; Debranching enzyme ; Phosphorylase-6-kinase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A workshop was held on “Aspects of treatment of patients with glycogen storage disease” within the framework of the Concerted Action “Inborn errors of metabolism” of the European Communities. Consensus was reached on the main issues of treatment of patients with deficiency of glucose-6-phosphatase, glucose-6-phosphate translocase, debranching enzyme, liver phosphorylase and phosphorylase-b-kinase. The resulting recommendations are reported.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442683
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  • 3
    Electronic Resource
    Electronic Resource
    The Rose Bengal test in neonatal cholestasis: Diagnostic and prognostic value (1981)
    Springer
    European journal of pediatrics 137 (1981), S. 27-29 
    Details
    ISSN: 1432-1076
    Keywords: Neonatal cholestasis ; Rose Bengal test
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract 131I Rose Bengal (131IRB) studies were performed in 73 infants with extrahepatic biliary atresia (EHBA) and in 37 with intrahepatic cholestasis of various origins. Fecal 131IRB excretion of less than 10% (“complete” cholestasis) was observed in EHBA but also in some patients with either paucity of intrahepatic bile ducts (syndromatic type) or with α-1-antitrypsin deficiency. Seventy one 131IRB tests were also performed 3 to 8 weeks postoperatively in children operated on for EHBA. Fecal 131IRB excretion more than 15% was present in 27 out of 34 cases who were later completely jaundice free and in only one out of 37 cases where no bile flow restoration occurred. These results indicate that complete cholestasis in infants can be observed in some types of intrahepatic cholestasis, as well as in EHBA, and show that a postoperative 131IRB test is a reliable means of predicting complete restoration of bile flow in EHBA.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441165
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  • 4
    Electronic Resource
    Electronic Resource
    Infantile Refsum disease: an inherited peroxisomal disorder (1987)
    Springer
    European journal of pediatrics 146 (1987), S. 477-483 
    Details
    ISSN: 1432-1076
    Keywords: Infantile Refsum disease ; Zellweger syndrome ; Neonatal adrenoleukodystrophy ; Peroxisomal disease ; Mental retardation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three patients affected by infantile Refsum disease are described with mental retardation, minor facial dysmorphia, chorioretinopathy, sensorineural hearing deficit, hepatomegaly, failure to thrive and hypocholesterolaemia. Initially, only an accumulation of phytanic acid was thought to be present. More recent findings showed a biochemical profile very similar to that found in classical Zellweger syndrome or neonatal adrenoleukodystrophy. Morphologically typical peroxisomes were absent in the liver. All three disorders are associated with multiple peroxisomal dysfunction. Because of these similarities pertinent clinical data of our three patients are compared with those of reported patients diagnosed as having infantile Refsum disease, neonatal adrenoleukodystrophy or Zellweger syndrome who survived for several years. Attention is drawn to the difference in severity of clinical features, ranging from infantile Refsum's disease to neonatal adrenoleukodystrophy and, finally, to Zellweger syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441598
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  • 5
    Electronic Resource
    Electronic Resource
    Liver adenomas in glycogen storage disease in children (1984)
    Springer
    Pediatric radiology 14 (1984), S. 94-101 
    Details
    ISSN: 1432-1998
    Keywords: Glycogen storage disease ; Adenomas ; Ultrasound ; Angiography ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The authors report the ultrasound and angiographic features of adenomas occurring in children with glycogen storage disease. Seven cases from 83 patients were diagnosed either by ultrasound, preoperative angiography or during surgery. The lesions appear on ultrasound as multiple rounded intrahepatic masses. Their degree of echogenicity as well of vascularity on angiography is highly variable. Ultrasound is the modality of choice in detecting adenomas. No malignant degeneration was observed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01625816
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  • 6
    Electronic Resource
    Electronic Resource
    Comparative use of glucose and fructose in cultured fibroblasts from patients with hereditary fructose intolerance (1987)
    Springer
    Journal of inherited metabolic disease 10 (1987), S. 52-61 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The utilization of fructose and glucose by fibroblast cultures obtained from patients with hereditary fructose intolerance (HFI) was studied in comparison with fibroblast controls. The cell growth, the time course ofd-glucose ord-fructose uptake and the consumption of fructose were similar for both HFI and control cells. Some results showed significant differences between these two cell types: HFI cells consumed less glucose, produced less lactate and contained less glycogen than control cells. Furthermore, significantly less [U-14C]d-glucose and [U-14C]d-fructose was incorporated into lipids in HFI cells than in control cells. The mechanisms responsible for these differences observed between the two cell types are not known.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01799488
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  • 7
    Electronic Resource
    Electronic Resource
    Infantile phytanic acid storage disease, a possible variant of Refsum's disease: Three cases, including ultrastructural studies of the liver (1982)
    Springer
    Journal of inherited metabolic disease 5 (1982), S. 83-90 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three cases of phytanic acid storage disease with symptoms during the first months of life are reported. Hepatomegaly, facial dysmorphia, growth and/or mental retardation and osteopenia were observed in addition to retinitis pigmentosa and neurosensory deafness. The presence of phytanic acid in serum (160–320 µmol/1 (50–100 µg/ml)) was accompanied by hypocholesterolaemia. Electron microscopy showed that a storage material had accumulated in mesenchymal and parenchymal liver cells. Lamellar structures were seen in hepatocytes and other storing cells. These inclusions resembled the structures found in plant chloroplasts containing phytol. Some of the clinical and biological data obtained were consistent with Refsum's disease. However, other characteristics such as mental retardation, hepatomegaly, osteopenia, hypocholesterolaemia and hypoalphalipoproteinaemia, as well as the ultrastructural findings in the liver, suggested that our patients' illness was either a phytanic acid storage disease different from the classical form of Refsum's disease, or a more severe early symptomatic form of Refsum's disease. Early diagnosis by phytanic acid assay and electron microscopic liver examination calls for prescription of a low phytanate diet in the hope of improving the child's condition.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01799998
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  • 8
    Electronic Resource
    Electronic Resource
    Crigler-Najjar type II disease inheritance: A family study (1989)
    Springer
    Journal of inherited metabolic disease 12 (1989), S. 302-306 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The inheritance of Crigler-Najjar type II disease is still contested. Autosomal dominant transmission with incomplete penetrance and autosomal recessive transmission have been proposed. We had the opportunity to study the hepatic activity of bilirubin uridinediphosphate glucuronyltransferase in parents whose first child had been affected by Crigler-Najjar type II disease. The demonstration of reduced activity of glucuronidation in the liver of both parents suggests autosomal recessive inheritance. The second infant of this couple was affected by the same disease and was treated with success by phenobarbital.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01799221
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  • 9
    Electronic Resource
    Electronic Resource
    131I rose bengal: Its use in the evaluation of infantile jaundice (1981)
    Springer
    European journal of nuclear medicine 6 (1981), S. 355-359 
    Details
    ISSN: 1619-7089
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract One-hundred ten 131I-rose bengal studies (RBI) were performed in infants suspected of having biliary atresia. Fecal RBI excretion of less than 10% was observed in 72 of 73 cases of extrahepatic biliary atresia, but also in 10 of 37 cases of intrahepatic cholestasis of various origins. One-hundred twenty-two RBI tests were performed in children operated on for extrahepatic biliary atresia and 71 tests were performed between postsurgical weeks 3 and 8, and 51 tests were done later. Prognostically, early tests show that fecal RBI excretion of more than 15% was observed in 2 of 34 cases who were later completely jaundice-free and in only 1 of 37 cases where no bile flow restoration occurred.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00251337
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