Congenital Hepatic Fibrosis-Is it a Single Clinical Entity?

Murray‐Lyon, Iain M.; Ockenden, B. G.; Williams, R

doi:10.1016/s0016-5085(73)80138-6

Cited by 66 publications

(16 citation statements)

References 9 publications

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“…There seems every reason to support and extend the belief8 that there is a family of interlocked and overlapping hepatobiliary fibrocystic disordersa view held in part for many years on the Liver Unit. 79 …”

Section: Resultsmentioning

confidence: 99%

Cystic disease of the liver and biliary tract.

Forbes

Murray‐Lyon

1991

Gut

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Section: Resultsmentioning

confidence: 99%

Cystic disease of the liver and biliary tract.

Forbes

Murray‐Lyon

1991

Gut

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“…Patients with infantile-type polycystic kidney disease may develop large cortical and medullary cysts resembling those found in APCKD (Lieberman et al, 1971) so a family history is crucial for a certain diagnosis of APCKD in association with CHF. Murray-Lyon et al, (1973) have drawn attention to the various combinations of hepatic and renal fibrocystic lesions and suggested that CHF should not be regarded as a distinct entity.…”

Section: Discussionmentioning

confidence: 99%

Congenital hepatic fibrosis and adult-type autosomal dominant polycystic kidney disease in a child

Lee¹,

Paes²

1985

Postgraduate Medical Journal

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“…This is cited as evidence for a subgrouping of hepatic fibropolycystic disease on genetic grounds. A further theory suggests the involvement of mutant alleles with intermediate forms of the disease being due to compound heterozygotes (Murray-Lyon, Ockenden and Williams, 1973). The kidneys of case 1 were more reminiscent of adult disease, i.e.…”

Section: Discussionmentioning

confidence: 99%

“…This man, although clinically having juvenile disease, did not suffer variceal haemorrhage despite his long standing portal hypertension and his renal failure did not become obvious until his early forties. Late onset variceal haemorrhage or rcnal failure is recognized in juvenile disease (Murray-Lyon et al, 1973).…”

Section: Discussionmentioning

confidence: 99%

Juvenile hepatic fibropolycystic disease and other abnormalities

Carty

Jones

1978

Postgraduate Medical Journal

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SummaryTwo cases are described of hepatic fibropolycystic disease (Brunt, 1973 The liver had a smooth capsule and was firm to the knife. The cut surface had a mosaic appearance with brown areas of liver tissue being broken up by white bands of fibrous tissue. There were no obvious cysts.On microscopy, the findings were typical of congenital hepatic fibrosis with strands of fibrous tissue within the portal tracts. The bile ducts appeared to be proliferating and there was some evidence of biliary stasis (Fig. 2).The capsules of the kidneys were adherent. The cortices measured up to 5 mm in thickness and were sponge-like. The cysts in the kidneys were up to 2 cm in diameter and some contained green pus and necrotic debris. On histology in the cortices there was fibrosis around the glomeruli which were sparse (Fig. 3). There were many cysts lined with flattened epithelium, and some of these contained casts. In the smaller right kidney there were areas of scarring, consistent with chronic pyelonephritis.The sister was not investigated by the authors but when aged 21 years she was said still to be suffering from hepatosplenomegaly. A barium meal had not shown oesophageal varices. Her kidneys were not palpable and her blood urea was 6.4 mmol/l. Intravenous pyelography had not been performed.Both the patient's parents were healthy into old age, and his children have been thoroughly investigated and appear free of renal or hepatic disease. Case 2This 27-year-old man was found to have enlarged kidneys when he was 3 months old. Shortly afterwards he developed pyloric stenosis and Rammstedt's operation was performed. There followed surgery for craniostenosis at 18 months, at which time both his liver and spleen were palpable.Routine biochemistry revealed a blood urea of 11.2 mmol/l.He remained moderately well until the age of 14 years when he had a haematemesis from oesophageal varices. During surgery to construct a portocaval

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Congenital Hepatic Fibrosis-Is it a Single Clinical Entity?

Cited by 66 publications

References 9 publications

Cystic disease of the liver and biliary tract.

Cystic disease of the liver and biliary tract.

Congenital hepatic fibrosis and adult-type autosomal dominant polycystic kidney disease in a child

Juvenile hepatic fibropolycystic disease and other abnormalities

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