Nodular regenerative hyperplasia

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Nodular regenerative hyperplasia (NRH) is a disease of the liver that is associated with portal hypertension .

In nodular regenerative hyperplasia, extensive benign transformation of the liver parenchyma occurs , and small regenerated nodules develop , while the portal field architecture is preserved. NRH is a rather rare disease and in more advanced cases it leads to non- cirrhosis-related portal hypertension. Systematic population studies of the disease are currently (as of 2016) not yet available and experience is limited to case reports and series.

Pathogenesis

NRH can develop due to autoimmunological, hematological, infectious, neoplastic or drug-induced causes. As a possible cause, NRH can occur as a side effect during therapy with azathioprine . Diabetes mellitus, long-term steroid use and chronic hepatitis also play a role in the development of the disease.

Usually there are no noticeable symptoms in sick patients at first, until portal hypertension or its complications develop in the further course. In the histopathology an accurate diagnosis is possible here by a diffuse micronodular transformation shows no fibrous septa in the liver tissue. An absence of perinuclear collagen tissue distinguishes the NRH normal regenerative nodules that are in a cirrhotic liver form.

therapy

While the initial treatment is limited to treating the underlying disease as a trigger, the therapy must ultimately be aimed at managing the resulting portal hypertension. The prognosis depends on the one hand on the severity of the underlying disease and on the other hand on the avoidance of secondary complications of portal hypertension.

Individual evidence

  1. ^ Daniel F: Azathioprine induced nodular regenerative hyperplasia in IBD patients . In: Gastroenterol. Clin. Biol. Band 29 , no. 5 , May 2005, pp. 600-3 , doi : 10.1016 / S0399-8320 (05) 82136-0 , PMID 15980758 .
  2. ↑ Vernier Massouille G: nodular regenerative hyperplasia in patients with inflammatory bowel disease Treated with azathioprine . In: Good . tape 56 , no. 10 , October 2007, p. 1404-9 , doi : 10.1136 / good 2006.114363 , PMID 17504943 .
  3. ↑ Hartleb M: Nodular regenerative hyperplasia: Evolving concepts on underdiagnosed cause of portal hypertension . In: World Journal of Gastroenterology: WJG . tape 17 , no. 11 , March 21, 2011, p. 1400-1409 , doi : 10.3748 / wjg.v17.i11.1400 , PMID 21472097 .