Nodular regenerative hyperplasia (NRH) is a
rare liver disease, characterised by the growth of
nodules within the liver, resulting in
liver
The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it ...
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
Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, and end-stage liver disease, is the impaired liver function caused by the formation of scar tissue known as fibrosis due to damage caused by liver disease. Damage causes tissue rep ...
. Complications of NCPH can include
jaundice,
ascites
Ascites is the abnormal build-up of fluid in the abdomen. Technically, it is more than 25 ml of fluid in the peritoneal cavity, although volumes greater than one liter may occur. Symptoms may include increased abdominal size, increased weight, a ...
,
splenomegaly
Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulatin ...
, 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 liver. Some cases are known to be caused by treatment with
azathioprine
Azathioprine (AZA), sold under the brand name Imuran, among others, is an immunosuppressive medication. It is used in rheumatoid arthritis, granulomatosis with polyangiitis, Crohn's disease, ulcerative colitis, and systemic lupus erythematosus, ...
, an immunosuppressant drug commonly used to prevent rejection in organ transplantation (especially of the kidneys), and to treat various auto-immune disorders.
References
External links
Hepatology
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