Nodular regenerative hyperplasia

Nodular regenerative hyperplasia
Other namesNodular regenerative hyperplasia of the liver
Human liver(normal)
ComplicationsPortal hypertension

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.[citation needed] 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.[citation needed]

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.[1] 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.[2][3] Other drug treatments, such as with oxaliplatin, can also contribute. Medical conditions associated with NRH include cystic fibrosis,[1] common variable immunodeficiency[1][4] and chronic granulomatous disease. Cases may also arise after solid-organ transplantation.[1]

References

  1. ^ a b c d "Nodular Regenerative Hyperplasia". LiverTox: Clinical and Research Information on Drug-Induced Liver Injury. Bethesda, Maryland: National Institute of Diabetes and Digestive and Kidney Diseases. 4 May 2019. PMID 31689031. Retrieved 1 July 2024 – via National Library of Medicine.
  2. ^ Daniel F, Cadranel JF, Seksik P, et al. (May 2005). "Azathioprine induced nodular regenerative hyperplasia in IBD patients". Gastroenterol. Clin. Biol. 29 (5): 600–3. doi:10.1016/S0399-8320(05)82136-0. PMID 15980758.
  3. ^ Vernier-Massouille G, Cosnes J, Lemann M, et al. (October 2007). "Nodular regenerative hyperplasia in patients with inflammatory bowel disease treated with azathioprine". Gut. 56 (10): 1404–9. doi:10.1136/gut.2006.114363. PMC 2000290. PMID 17504943.
  4. ^ Ganapathi L, Cochran RL, Robbins GK, Barmettler S, Holland SM, Ababneh EI (2024). "Case 20-2024: A 73-Year-Old Man with Recurrent Fever and Liver Lesions". The New England Journal of Medicine. 390 (24): 2309–2319. doi:10.1056/NEJMcpc2309383. PMID 38924735.

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