Congenital hepatic fibrosis.

Hodgson, Hjf; Davies, D.C.; Thompson, R P

doi:10.1136/jcp.29.1.11

Cited by 23 publications

(8 citation statements)

References 17 publications

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“…In the patient described by Hodgson et al (9) with iCHF, renal dysfunction was noted by low creatinine clearance. Lillegard (10) reported encephalopathy in their patients with iCHF.…”

Section: Resultsmentioning

confidence: 96%

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

Srinath¹,

Shneider²

2012

J. pediatr. gastroenterol. nutr.

117

100

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Objectives The published natural history of congenital hepatic fibrosis (CHF) was examined to inform clinical decision making in autosomal recessive polycystic kidney disease (ARPKD). Methods A systematic literature search of the data on CHF, ARPKD, Caroli disease, Caroli syndrome, and type V choledochal cyst was performed to extract data related to portal hypertension, infection, malignancy, mortality, and transplantation. Results Information related to 1230 patients with CHF was extracted from 155 articles. Median and mean age at diagnosis were 2 and 11.2 years, respectively. Median and mean time followed after diagnosis were 5.0 and 7.5 years, respectively (range 0–38 years). Sequelae of portal hypertension (n = 409), cholangitis (n = 152), and malignancy (n = 21) were noted. The nature of the portal hypertension was similar to that in other pediatric conditions (164 with varices, 74 bleeding varices, 81 underwent portosystemic shunting). Documented cholangitis was fatal in 3 of 23 children who were infected after renal transplantation. Twenty-one patients developed hepatobiliary cancer, with the majority having cholangiocarcinoma (n = 19). Cholangiocarcinoma (CCA) was predominant in individuals older than 40 years with either Caroli syndrome or isolated CHF, not ARPKD (median and mean age at CCA diagnosis were 70.3 and 60.1 years, respectively; range 33–75 years). There was a relative paucity of data on combined liver-kidney transplantation. Conclusions Clinical decision making in ARPKD should reflect an understanding of the potential issues emanating from CHF. Accepted pediatric specific approaches to portal hypertension are warranted but must take into consideration the stage of renal insufficiency and potential plans for renal transplantation. Cholangitis is a major issue and necessitates anticipatory guidance and awareness. CCA, although a dreaded complication, does not appear to be a major issue during childhood. The indications for liver and combined liver-kidney transplantation are controversial and warrant further analysis.

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“…In the patient described by Hodgson et al (9) with iCHF, renal dysfunction was noted by low creatinine clearance. Lillegard (10) reported encephalopathy in their patients with iCHF.…”

Section: Resultsmentioning

confidence: 96%

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

Srinath¹,

Shneider²

2012

J. pediatr. gastroenterol. nutr.

117

100

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“…4,48,49 It is a form of ductal plate malformation with characteristic histologic features of broad fibrous bands with fewer than normal portal vein structures and the necklace-like arrangement of nonatretic biliary structures at the periphery of portal tracts/fibrous bands (Figs. 4,48,49 It is a form of ductal plate malformation with characteristic histologic features of broad fibrous bands with fewer than normal portal vein structures and the necklace-like arrangement of nonatretic biliary structures at the periphery of portal tracts/fibrous bands (Figs.…”

Section: Congenital Hepatic Fibrosismentioning

confidence: 99%

“…4,48,49 It is a form of ductal plate malformation with characteristic histologic features of broad fibrous bands with fewer than normal portal vein structures and the necklace-like arrangement of nonatretic biliary structures at the periphery of portal tracts/fibrous bands (Figs. 4,48,49 Portal vein thrombosis and cavernous transformation have been reported with CHF. 4,48,49 Portal vein thrombosis and cavernous transformation have been reported with CHF.…”

Section: Congenital Hepatic Fibrosismentioning

confidence: 99%

“…1 In current practice, a liver biopsy performed with a request of "rule out cirrhosis" implies there is either nodularity noted by imaging or clinically detected features of portal hypertension (pHTN) without a clearly identified etiology. [2][3][4][5][6][7][8][9][10][11][12][13][14] The first 3 often manifest with pHTN before the development of cirrhosis, and thus require awareness among clinicians and pathologists alike. Cirrhosis is by far the leading cause of pHTN in western world.…”

Section: Introductionmentioning

confidence: 99%

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Histopathologic Evaluation of Liver Biopsy for Cirrhosis

Ma¹,

Brunt²

2012

Advances in Anatomic Pathology

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“…Even in the short term, hepatic failure can occur (Kerr et al, 1961;Williams et al, 1964) and it would not be surprising 514 if hepatic function declined after shunt operations. Children with the disorder face other hazards, including uraemia, calculi (Hodgson et al, 1976), and septicaemia (Kerr et al, 1962) from their renal disease, cholangitis from associated anomalies of the bile ducts which include Caroli's anomaly (cystic dilatation) (Murray-Lyon et al, 1972;Mall et al, 1974), choledochous cyst, and gallstones; infections from other portals of entry (Thorburn et al, 1967), and lung fibrosis or emphysema (Williams et al, 1964). Peptic ulcer has featured in some reports (Lorimer et al, 1967) and might be expected to increase after shunt surgery.…”

Section: Personal Note (Dnsk)mentioning

confidence: 99%

Congenital hepatic fibrosis: the long-term prognosis.

Kerr¹,

Okonkwo²,

Choa³

1978

Gut

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SUMMARY The long-term prognosis in congenital hepatic fibrosis has been assessed in 30 patients, 13 treated at Newcastle and 17 from other British centres. Twenty-four patients had been followed-up for more than five years from diagnosis. Shunt operations, performed in 18 patients, were successful in controlling haemorrhage with alow mortality (1/18), low incidence of recurrent haemorrhage (3/18) and portal-systemic encephalopathy (3/18) but with a higher incidence of postoperative jaundice (7/18). About a third of the survivors had some evidence of hepatic dysfunction but none had chronic portal systemic encephalopathy. Intelligence quotient was close to normal but educational achievement and job status were low. Serum albumin fell after shunt surgery and a few patients developed ascites: anaemia and hypersplenism however, were corrected by surgery. Blood ammonia is raised in survivors with congenital hepatic fibrosis and rises further after a normal meal; further observations are needed on cerebral function after several decades of survival.

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Congenital hepatic fibrosis.

Abstract: During 10 years four patients with congenital hepatic fibrosis were seen in a general hospital in London; three presented in adult life. It is suggested that the condition may account for a larger proportion of patients with chronic liver disease than has been thought to be the case.

Cited by 23 publications

References 17 publications

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

Histopathologic Evaluation of Liver Biopsy for Cirrhosis

Congenital hepatic fibrosis: the long-term prognosis.

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