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Alagille Syndrome
Journal Scan

Does congenital cytomegalovirus infection cause hepatic disease progression in patients with inherited liver diseases?

Posted on August 26, 2021

In this case report, 3 patients with progressive familial intrahepatic cholestasis (PFIC) type IV, alpha 1 anti-trypsin deficiency (A1ATD), or Alagille syndrome (ALGS) and diagnosed with congenital cytomegalovirus (cCMV) infection were followed. Patients were treated with valgancilovir for symptomatic cCMV infection for 6 to 12 months. The patients with PFIC and A1ATD followed valgancilovir treatment with suppressive dosing. At the follow-period (15-24 months), the patients with PFIC and A1ATD had developed severe liver failure. The patient with ALGS had ongoing cholestatic disease with stable synthetic function.

The authors suggested that cCMV infection may significantly contribute to the course of the inherited liver disease, “possibly leading to further compromise of the liver,” and that patients with inherited liver diseases be screened for cCMV and undergo anti-viral treatment 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;64(8):104249. doi: 10.1016/j.ejmg.2021.104249. Epub 2021 May 20. PMID: 34023521.

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