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  • 11
    Electronic Resource
    Electronic Resource
    Radiation sensitivity of bloom's syndrome lymphocytes during S and G"2 phases
    Amsterdam : Elsevier
    Cancer Genetics and Cytogenetics 16 (1985), S. 131-136 
    Details
    ISSN: 0165-4608
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0165-4608(85)90006-8
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  • 12
    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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  • 13
    Electronic Resource
    Electronic Resource
    Long-term results of surgical treatment of biliary atresia (1978)
    Springer
    World journal of surgery 2 (1978), S. 589-593 
    Details
    ISSN: 1432-2323
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Résumé Nous présentons, par une série de 111 cas d'atrésie biliaire dite “non corrigible”, les résultats à longue échéance de l'opération de Kasai. L'écoulement de bile a été rétabli pendant au moins un an, de façon plus ou moins satisfaisante, chez 34 malades. Presque tous ont encore des signes de cholostase, des canaux biliaires intrahépatiques à morphologie anormale, une fibrose hépatique persistante ou de l'hypertension portale. Malgré ces altérations hépatiques, de nombreux malades sont capables de mener une vie normale.
    Notes: Abstract The long-term results of surgical treatment of biliary atresia are presented from a series of 111 “noncorrectable” cases treated by the Kasai procedure. Thirty-four patients displayed some degree of restoration of bile flow for at least 1 year after surgery. Almost all of them still have some manifestations of cholestasis, abnormal intrahepatic bile ducts, persistent hepatic fibrosis, or portal hypertension. Despite this continuous liver dysfunction, many of these patients are able to carry on an almost normal life.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01556053
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  • 14
    Electronic Resource
    Electronic Resource
    Cardiomyopathy in glycogen-storage disease type III: Clinical and echographic study of 18 patients (1991)
    Springer
    Pediatric cardiology 12 (1991), S. 161-163 
    Details
    ISSN: 1432-1971
    Keywords: Glycogen-storage disease ; Amylo-1,6-glucosidase ; Cardiomyopathy ; Echocardiography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Cardiac examinations were performed on 18 patients with glycogen-storage disease (GSD) type III. Clinical examination was always normal and the electrocardiograms revealed nonspecific data. Similarly, serum muscular enzyme activities were not useful in indicating the presence of cardiomyopathy. Echocardiographic evidence of myocardiopathy was found in five of the 16 children studied (mean age, 9.5 years). Echocardiographic parameters remained stable during the follow-up period (at least 3 years). The other 11 children had no echocardiographic evidence of cardiomyopathy. No relationship was found between peripheral myopathy and cardiomyopathy. All patients with GSD type III should be regularly investigated by echocardiography in respect of their cardiac muscle status.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02238523
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  • 15
    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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  • 16
    Electronic Resource
    Electronic Resource
    The long-term outcome of patients with glycogen storage diseases (1990)
    Springer
    Journal of inherited metabolic disease 13 (1990), S. 411-418 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In this retrospective study from five centres, 139 patients over 10 years of age with glycogen storage disease types I, III, VI and IX are described. Almost half of the patients with glycogen storage disease type Ia had retarded growth and most had hyperlipidaemia. One-third of the patients had adenomas, although none of these showed malignant transformations. With increasing age the growth, liver size and hyperlipidaemia of patients with glycogen storage disease type III improve. However, there was a high incidence of myopathy and cardiomyopathy. Patients with glycogen storage disease types VI and IX had a normal growth pattern after childhood. Hepatomegaly and hyperchole-sterolaemia, however, were still present in half of the patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01799498
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  • 17
    Electronic Resource
    Electronic Resource
    Clinical presentation of metabolic liver disease (1991)
    Springer
    Journal of inherited metabolic disease 14 (1991), S. 526-530 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Some clinical clues should alert paediatricians to the possibility of metabolic liver diseases. They can be classified into three categories: (i) Manifestations due to hepatocellular necrosis, acute or subacute, which can reveal galactosaemia, hereditary fructose intolerance, tyrosinaemia type I, Wilson disease andα 1-antitrypsin deficiency. Symptoms and signs suggestive of Reye syndrome should lead to a study of fatty acid oxidation and urea cycle enzymes. All these manifestations may necessitate a rapid diagnosis and treatment when liver dysfunction is severe. (ii) Cholestatic jaundice can revealα 1-antitrypsin deficiency, Byler's disease, cystic fibrosis, Niemann-Pick disease and some disorders of peroxisome biogenesis. (iii) Hepatomegaly can reveal disorders with liver damage but also storage diseases such as glycogen storage diseases, cholesteryl ester storage disease and, when associated with splenomegaly, lysosomal storage diseases. Appropriate investigations for recognizing all these entities are proposed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01797922
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  • 18
    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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  • 19
    Electronic Resource
    Electronic Resource
    In vivo 13C-NMR evaluation of glycogen content in a patient with glycogen storage disease (1992)
    Springer
    Journal of inherited metabolic disease 15 (1992), S. 723-726 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Glycogen storage disease was suspected in a 10-month-old boy. Initial technical problems did not permit the determination of the precise enzyme deficiency, and type VI glycogen storage disease was only diagnosed at the age of 2 years. In the mean time, natural abundance13C nuclear magnetic resonance evaluation of muscular and hepatic glycogen content indicated normal muscular glycogen and increased hepatic glycogen in our patient, a finding which strongly argued for the diagnosis of type VI glycogen storage disease. Even though the use of nuclear magnetic resonance might seem, in this situation, a somewhat circuitous means of reaching the diagnosis, it appears that nuclear magnetic resonance could provide a useful tool for a non-invasive diagnosis of glycogen storage diseases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01800013
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  • 20
    Electronic Resource
    Electronic Resource
    Tolerance to prolonged fasting in two children with type I glycogen storage disease (1993)
    Springer
    Journal of inherited metabolic disease 16 (1993), S. 1044-1045 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00711524
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