Congenital hepatic fibrosis in Jamaican children.

Polycystic disease of kidney and liver presenting in childhood.

“…Cases 6 and 7 in the series of Jamaican children with congenital hepatic fibrosis described by Thorburn, Miller, and Bras (1967) are sibs, and both appear to fit into the infantile group.…”

Congenital hepatic fibrosis: the long-term prognosis.

“…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.…”

“…If it were a dominant (Sweetnam and Sykes, 1961) it should have appeared in successive generations. The few studies of karyotype have been normal (Thorburn et al, 1967 Most accounts of congenital hepatic fibrosis stress the well-preserved hepatic function, the good prognosis if bleeding from varices can be controlled, and the suitability of the patients for shunt surgery. However, the published information on long-term survival and rehabilitation is scant.…”

“…If it were a dominant (Sweetnam and Sykes, 1961) it should have appeared in successive generations. The few studies of karyotype have been normal (Thorburn et al, 1967).…”