Nodular regenerative hyperplasia
|Nodular regenerative hyperplasia|
|Other names: Nodular regenerative hyperplasia of the liver|
Nodular regenerative hyperplasia (NRH) is a rare liver condition characterized by a widespread benign transformation of the hepatic parenchyma into small regenerative nodules. NRH may lead to the development of non-cirrhotic portal hypertension. There are no published systematic population studies on NRH and our current knowledge is limited to case reports and case series. NRH may develop via autoimmune, hematological, infectious, neoplastic, vascular, or drug-related causes. It is associated with rheumatoid arthritis, Felty syndrome, myeloproliferative disorders, hereditary hemorrhagic telangiectasia (HHT, Osler–Weber–Rendu syndrome), liver, kidney and bone marrow transplantation, cytotoxic drugs like azathioprine, mercaptopurine, thioguanine, antiretroviral drugs for HIV like didanosine and vitamin A. There is also association between NRH and platinum-based drugs, such as chemotherapy with oxaliplatin. The disease is usually asymptomatic, slowly or non-progressive unless complications of portal hypertension develop. Accurate diagnosis is made by histopathology, which demonstrates diffuse micronodular transformation without fibrous septa. Lack of perinuclear collagen tissue distinguishes NRH from typical regenerative nodules in the cirrhotic liver. While the initial treatment is to address the underlying disease, ultimately the therapy is directed to the management of portal hypertension. The prognosis of NRH depends on both the severity of the underlying illness and the prevention of secondary complications of portal hypertension. In this review we detail the epidemiology, pathogenesis, diagnosis, management, and prognosis of NRH. Feathery degeneration is seen in PBC It can be a complication of azathioprine therapy.
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