CASE REPORT Nodular regenerative hyperplasia, portal vein thrombosis, and avascular hip necrosis due to hyperhomocysteinaemia
نویسندگان
چکیده
A male patient with portal hypertension, portal vein thrombosis, spontaneous splenorenal shunt formation, and encephalopathy, thought to have post-hepatitis B cirrhosis, is described. His condition deteriorated and necessitated liver transplantation. In the explant liver, nodular regenerative hyperplasia with pronounced vascular lesions both in portal venules and in arterioles was found instead of classical cirrhosis. Two years post-transplant he developed bilateral ischaemic femur head necrosis. The three disorders (portal vein thrombosis, nodular regenerative hyperplasia, and ischaemic hip necrosis) seemed to be due to a common vasculopathy induced by hyperhomocyteinaemia. Genetic studies showed that he carried a mutation in the gene encoding for formation of methylenetetrahydrofolate reductase. Treatment with folic acid combined with pyridoxine (vitamin B6) and cyanocobalamin (vitamin B12) normalised his serum homocysteine levels.
منابع مشابه
Nodular regenerative hyperplasia, portal vein thrombosis, and avascular hip necrosis due to hyperhomocysteinaemia.
A male patient with portal hypertension, portal vein thrombosis, spontaneous splenorenal shunt formation, and encephalopathy, thought to have post-hepatitis B cirrhosis, is described. His condition deteriorated and necessitated liver transplantation. In the explant liver, nodular regenerative hyperplasia with pronounced vascular lesions both in portal venules and in arterioles was found instead...
متن کاملSuccessful Treatment of Small Intestinal Bleeding in a Crohn's Patient with Noncirrhotic Portal Hypertension by Transjugular Portosystemic Shunt Placement and Infliximab Treatment
Small intestinal bleeding in Crohn's disease patients with noncirrhotic portal hypertension and partial portal and superior mesenteric vein thrombosis is a life-threatening event. Here, a case is reported in which treatment with azathioprine may have resulted in nodular regenerative hyperplasia, portal hypertension and portal vein thrombosis. The 56-year-old patient with Crohn's disease develop...
متن کاملDevelopment of multiple focal nodular hyperplasia lesions after portocaval shunting. A case report.
It has been postulated that altered hepatic blood flow, particularly reduced portal flow, is responsible for the induction of hyperplasia of liver cells and nodule formation. This report describes the case of a 31-year old female patient developing multiple focal nodular hyperplasia (FNH) lesions two years after portocaval shunting and extended right hemihepatectomy due to the suspicion of a ma...
متن کاملPortal Hypertension, Nodular Regenerative Hyperplasia of the Liver, and Obstructive Portal Venopathy due to Metastatic Breast Cancer
Nodular regenerative hyperplasia (NRH) of the liver is associated with noncirrhotic portal hypertension, rheumatologic and hematologic disorders, administration of certain drugs, and other underlying conditions. This report describes a 64-year-old man with clinically presumed cirrhosis who presented to our institution with coffee-ground emesis, esophageal varices, ascites, and encephalopathy. E...
متن کاملVariceal bleeding in an adolescent with HIV diagnosed with hepatoportal sclerosis and nodular regenerative hyperplasia.
340 due to elevated portal cirrhotic and noncirrh V ariceal bleeding venous pressure may result from both otic processes. Etiologies of portal hypertension in the absence of cirrhosis include portal vein thrombosis, nodular regenerative hyperplasia (NRH), hepatoportal sclerosis (HPS), incomplete septal cirrhosis, schistosomiasis, and vasculitides (1). HPS is also known as Banti syndrome (2), id...
متن کامل