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Non-alcoholic steatohepatitis

Orpha number ORPHA33271
Prevalence of rare diseases >1 / 1000
Inheritance
  • Sporadic
Age of onset Adulthood
ICD 10 code
  • K76.0
MIM number
Synonym(s) NASH syndrome

Summary

Patients with chronic, moderately elevated liver enzyme concentrations without a diagnosis after a clinical, biochemical and radiological work-up are likely to suffer from non-alcoholic steatohepatitis or NASH. This possibility is further supported by the presence of obesity, hyperglycemia and hyperechogenic hepatic parenchyma. The diagnosis can be definitively made only by histological examination of a liver biopsy containing lesions suggestive of ethanol intake in a patient known to consume less than 40 g of alcohol/week. NASH is a common disease, with a prevalence around 1% of the general population similar to that of hepatitis C. The natural history remains to be studied, but it is not necessarily benign: cryptogenic cirrhosis in patients is a substantial number of probably end-stage NASH. The treatment is to lose weight for the overweight patients, to correct the biochemical abnormalities like hyperglycemia and hyperlipidemia and, if necessary to remove excess iron. Vitamin E and/or ursodeoxycholic acid may be helpful, but such therapies should be prescribed within clinical trials as their efficacies remain uncertain. Small trials have shown benefits of betaine and of a thiazolidinedione

Expert reviewer(s)

  • Pr Jean-François DUFOUR

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Orphanet version 4.7.11 - Last updated: 2012-05-22