Familial occurrence of nodular regenerative hyperplasia of the liver: a report on three families

Abstract

Background/Aims-Nodular regenerative hyperplasia of the liver is a histological lesion usually associated with systemic diseases, haematological malignancies, or drugs. Its prognosis depends on portal hypertension, which usually is well tolerated and requires medical management only. Patients-Three unrelated families, in which two sibling adult male patients presented with nodular regenerative hyperplasia of the liver, were studied. Methods-Complete clinical charts and liver biopsy specimens were available for all patients. In addition, explanted livers were available for examination for the two transplanted patients. Results-There was no evidence of any of the various clinical situations known to be associated with nodular regenerative hyperplasia of the liver. Portal hypertension was severe, requiring surgical treatment in two cases. Renal lesions were present in three patients. In two patients, progressive evolution to liver atrophy and hepatic failure, associated with renal failure, led to combined liver and renal transplantation. Conclusions-This report describes the existence of familial cases of nodular regenerative hyperplasia of the liver, occurring without underlying or associated systemic disease, characterised by a poor clinical course and often associated with progressive renal failure. (Gut 1999;45:289-294)

Orthotopic Liver Transplantation for Idiopathic Portal Hypertension: Indications and Outcome

“…Management and outcome of OLT are given in Table IV. Four patients have been the subject of a previous partial report (15).…”

“…The median age at time of diagnosis was 38.5 years (range 17-59). In four patients there was a familial history of IPH (15). The circumstances of diagnosis were: gastrointestinal bleeding in four cases, incidental endoscopic diagnosis of esophageal varices in three cases and leucothrombocytopenia in one case.…”

Nodular regenerative hyperplasia: Not all nodules are created equal

“…26 Rare cases of hepatic and renal failure necessitating orthotopic liver transplantation and renal transplantation have also been reported. 16 NRH has also been reported to occur after liver transplantation. It is not known whether NRH is a reversible process once the presumed cause is removed, such as might occur with stopping a drug.…”

“…There have been three familial cases of nodular regenerative hyperplasia reported in the literature. 16 …”

“…Experience with orthotopic liver transplantation in NRH is very limited. 16,39,40 Lastly, there are case reports of hepatocellular carcinoma occurring in NRH. 41,42 There are theoretical reasons why NRH might be a risk factor for hepatocellular carcinoma.…”