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Alagille Syndrome
Journal Scan

Should patients with inherited liver diseases be screened for congenital cytomegalovirus?

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In this case series, 3 patients, each with either progressive familial intrahepatic cholestasis type IV, alpha 1 anti-trypsin deficiency, or Alagille syndrome, presented to the authors institution with a congenital cytomegalovirus (cCMV) infection and treated with valgancilovir. The patients with progressive familial intrahepatic cholestasis type IV and alpha 1 anti-trypsin deficiency patients were subsequently treated with suppressive doses. After 15-24 month follow-up, the patents with progressive familial intrahepatic cholestasis type IV and alpha 1 anti-trypsin deficiency had developed severe liver failure and the patient with Alagille syndrome had ongoing cholestatic disease with stable synthetic function.

The authors concluded that cCMV infection may have significantly contributed to the course of the inherited liver disease, potentially compromising the liver more.

The authors recommended “screening patients with inherited liver disease for cCMV, and considering anti-viral treatment with valganciclovir to delay hepatic disease progression.”

Reference
Swed-Tobia R, Kassis I, Weiss K, et al. Concomitant congenital CMV infection and inherited liver diseases. Eur J Med Genet. 2021;104249. doi: 10.1016/j.ejmg.2021.104249. Epub ahead of print. PMID: 34023521.

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