nodular regenerative hyperplasia of the liver
MONDO:0018835
Nodular regenerative hyperplasia of the liver is a rare parenchymatous liver disease characterized by diffuse benign transformation of the hepatic parenchyma into multiple small nodules (composed of regenerating hepatocytes) and that is usually asymptomatic but can lead to the development of non-cirrhotic portal hypertension and its complications, including esophageal variceal bleeding, hypersplenism and ascites. It is often associated with rheumatologic, autoimmune, hematologic, and myeloproliferative disorders as well as various immune deficiency states and exposure certain drugs and toxins.
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Nodular regenerative hyperplasia
Nodular regenerative hyperplasia (NRH) is a rare liver disease, characterised by the growth of nodules within the liver, resulting in liver hyperplasia. While in many cases it is asymptomatic and thus goes undetected – or is only discovered incidentally while investigating some other medical condition – in some people it results in non-cirrhotic portal hypertension (NCPH). NCPH is generally less severe than the much more common portal hypertension due to cirrhosis. Complications of NCPH can include jaundice, ascites, splenomegaly, and bleeding esophageal varices. Most people with NRH retain normal liver function – even among the subset who go on to develop NCPH – and liver failure in NRH is uncommon. Only a small proportion of NRH patients will ever require liver transplantation. The causes of NRH are poorly understood, although it is believed to be related to abnormal blood flow in the small vessels of the liver. NRH development may be preceded by an attack of sinusoidal obstructive syndrome. Some cases are known to be caused by treatment with azathioprine, an immunosuppressant drug commonly used to prevent rejection in organ transplantation (especially of the kidneys), and to treat various auto-immune disorders. Other drug treatments, such as with oxaliplatin, can also contribute. Medical conditions associated with NRH include cystic fibrosis, common variable immunodeficiency and chronic granulomatous disease. Cases may also arise after solid-organ transplantation.
Background only. Canonical identity remains MONDO; clinical-trial relationships come from ClinicalTrials.gov.Drugs being studied
Disease identity and terminology: MONDO. Trial relationships: ClinicalTrials.gov.