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Case Report

A Case of Congenital Hepatic Fibrosis Accompanied by Renal Tubular Ectasia, Caroli Syndrome and Choledochal Cyst

Bong Seok Choi, Sang Nam Bae, Yong Tak Im, Jae Hong Park, Chang Hoon Lee, Jun Woo Lee

Clin Exp Pediatr. 2002;45(7):923-927. Published online July 15, 2002

Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis...

A Case of Fibromuscular Dysplasia Associated with Hypertensive Encephalopathy

Ju Suk Lee, Sang Nam Bae, Sang Ook Nam, Su Young Kim, Hee Ju Park

Clin Exp Pediatr. 2000;43(5):715-719. Published online May 15, 2000

Fibromuscular dysplasia is the single leading etiology of renovascular hypertension in children. We report an eight-year-old girl who was admitted for generalized tonic seizure with fibromuscular dysplasia of right renal artery. On admission, she presented hypertension and altered mentality. She had suffered from intermittent severe headache for the past year. Renal angiography of right renal artery showed stenosis, beaded pattern...

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