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  • 1
    Electronic Resource
    Electronic Resource
    Latent chronic cholangitis in congenital hepatic fibrosis (1982)
    Springer
    European journal of pediatrics 139 (1982), S. 203-205 
    Details
    ISSN: 1432-1076
    Keywords: Cholangitis ; Child ; Liver disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a five-year-old girl with congenital hepatic fibrosis raised erythrocyte sedimentation rate and serum gammaglobulins were noted for 3 years. Needle liver biopsy showed histological features of cholangitis andKlebsiella oxytoca was cultured from a liver biopsy specimen. Antibiotic treatment resulted in normalization of erythrocyte sedimentation rate and serum gammaglobulins. This suggest that cholangitis can be clinically latent in congenital hepatic fibrosis and should be investigated and treated when unexplained inflammatory signs are present.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01377359
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  • 2
    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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  • 3
    Electronic Resource
    Electronic Resource
    Portal cavernoma in congenital hepatic fibrosis (1994)
    Springer
    Pediatric radiology 24 (1994), S. 61-65 
    Details
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A retrospective pediatric study of 30 angiograms in congenital hepatic fibrosis (CHF) was undertaken to analyze the different aspects of the portal venous system in this disease. Besides the classical angiographic signs (hepatofugal veins and intrahepatic duplications), a hepatopetal venous network was present in ten children, contrasting with the patency of the portal venous system. Analysis of the medical charts for these ten patients showed that the clinical signs and outcome were similar to classical CHF. Liver biopsies were available in six cases and, in addition to the diagnostic features of CHF, demonstrated paucity or absence of portal venules. This unusual association of hepatopetal and hepatofugal collateral veins in an intrahepatic block could be explained by hypoplasia or thrombosis of portal venules. Thus, the association between hepatomegaly and hepatopetal collateral veins without portal obstruction should be suggestive of CHF.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02017666
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  • 4
    Electronic Resource
    Electronic Resource
    Long-term outcome of liver transplantation in patients with glycogen storage disease type Ia (1999)
    Springer
    Journal of inherited metabolic disease 22 (1999), S. 723-732 
    Details
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Liver transplantation may be indicated in patients with GSD type Ia when dietary treatment fails or when hepatic adenomas develop, because they carry a risk of liver cancer or severe intratumoral haemorrhage. Published reports on the results of liver transplantation in patients with GSD Ia include 10 patients and provide little information on long-term outcome. In particular, it is not known whether liver transplantation prevents renal failure due to focal segmental glomerulosclerosis. We report here on 3 patients with GSD Ia in whom liver transplantation was performed at 15, 17 and 23 years of age because of multiple hepatic adenomas in all 3 patients with a fear of malignant transformation, and of poor metabolic balance and severe growth retardationin the youngest one. Renal function was normal in all patients. During the 6–8 years following transplantation, the quality of life has initially greatly improved, with none of the previous dietary restraints and a spectacular increase in height. However, long-term complications included chronic hepatitis C in one patient, gouty attacks in another and focal segmental glomerulosclerosis with progressive renal insufficiency in the third. These results: (1) confirm that liver transplantation restores a normal metabolic balance in patients with GSD Ia, allows catch-up growth and improves the quality of life; (2) suggest that liver transplantation may be considered in teenagers with unresectable multiple adenomas because of a lack of clear-cut criteria to detect malignant transformation early; and (3) suggest that liver transplantation does not prevent focal segmental glomerulosclerosis associated with GSD Ia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1005544117285
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  • 5
    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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  • 6
    Electronic Resource
    Electronic Resource
    Deleted Chromosome 20 from a patient with Alagille syndrome isolated in a cell hybrid through leucine transport selection: study of three candidate genes (1994)
    Springer
    Mammalian genome 5 (1994), S. 663-669 
    Details
    ISSN: 1432-1777
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Alagille syndrome (AGS) is a well-defined genetic entity assigned to the short arm of Chromosome (Chr) 20 by a series of observations of AGS patients associated with microdeletions in this region. By fusing lymphoblastoid cells of an AGS patient that exhibited a microdeletion in the short arm of Chr 20 encompassing bands p11.23 to p12.3 with rodent thermosensitive mutant cells (CHOtsH1-l) deficient in-leucyl-tRNA synthetase, we isolated a somatic cell hybrid segregating the deleted human Chr 20. This hybrid clone, designated NR2, was characterized by several methods, including PCR, with eight pairs of oligonucleotides mapped to Chr 20: D20S5, D20S41, D20S42, D20S56, D20S57, D20S58, adenosine deaminase (ADA), and Prion protein (PRIP); Restriction Fragment Length Polymorphism (RFLP) analyses with four genomic anonymous probes (D20S5, cD3H12, D20S17, D20S18); and fluorescent in situ hybridization (FISH) with total human DNA and D20Z1, a sequence specific to the human Chr 20 centromere, as probes. The NR2 hybrid allowed us to exclude three candidate genes for AGS: hepatic nuclear factor 3 β (HNF3β), paired box 1 (PAX1), and cystatin C (CST3) as shown by their localization outside of the deletion. The NR2 hybrid is a powerful tool for the mapping of new probes of this region, as well as for obtaining new informative probes specific for the deletion by subtractive cloning of the region. Such markers will be useful for linkage analysis and screening of cDNA libraries.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00426072
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