Three Cases of Congenital Hepatic Fibrosis with Caroli`s Disease in Three Siblings

Three Cases of Congenital Hepatic Fibrosis with Caroli’s Disease in Three Siblings

Congenital hepatic fibrosis is a relatively rare disease of children and young adults characterized by hard hepatomegaly, portal hypertension with relative preservation of liver function and underlying architecture, and frequent renal involvement. We experienced 3 cases of congenital hepatic fibrosis with Caroli's disease in 3 siblings, whose clinical manifestations were diverse, such as repeat...

Congenital hepatic fibrosis in an aborted calf

An aborted female Holstein foetus with marked generalized anasarca was referred to the Excellence Centre for Ruminant Abortion and Neonatal Mortality, Ferdowsi University of Mashhad. On postmortem examination, red-tinged ascites, pale and firm liver with extreme irregularity and numerous round to oval slightly raised foci on the capsular surface were seen. Histological examination revealed wide...

familial congenital hepatic fibrosis: report of a family with three affected children.

congenital hepatic fibrosis (chf) is a developmental disorder of the biliary system, characterized by defective remodeling of the ductal plate. herein a family of three children, from consanguineous parents, with minor thalassemia is presented who suffered from congenital hepatic fibrosis (chf). prompt diagnosis and appropriate treatment are necessary to avoid further complications in the affec...

APPARENT MINERALOCORTICOID EXCESS IN THREE SIBLINGS

Three siblings (1 boy, 2 girls) with hypertension and hypokalemia are presented, two with low plasma aldosterone and suppressed renin activity and the eldest with a high renin and aldosterone level due to secondary changes in her kidneys. Urinary tetrahydrocortisol (THF) was increased relative to tetrahydrocortisone (THE). Cortisol ring A reduction constant was also lower than normal. Thes...

Aortic aneurysm in three siblings with multiple congenital contractures.