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Neuromuscul Disord. 2013 Nov;23(11):917-21. doi: 10.1016/j.nmd.2013.06.008. Epub 2013 Sep 4.

Fatal hepatic hemorrhage by peliosis hepatis in X-linked myotubular myopathy: a case report.

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  • 1Department of Pediatrics, Uwajima City Hospital, Uwajima, Ehime, Japan. Electronic address: tmotoki@m.ehime-u.ac.jp.

Abstract

We report a 5-year-old boy with X-linked myotubular myopathy complicated by peliosis hepatis. At birth, he was affected with marked generalized muscle hypotonia and weakness, which required permanent ventilatory support, and was bedridden for life. He died of acute fatal hepatic hemorrhage after using a mechanical in-exsufflator. Peliosis hepatis, defined as multiple, variable-sized, cystic blood-filled spaces through the liver parenchyma, was confirmed by autopsy. To avoid fatal hepatic hemorrhage by peliosis hepatis, routine hepatic function tests and abdominal imaging tests should be performed for patients with X-linked myotubular myopathy, especially at the time of using artificial respiration.

Copyright © 2013 Elsevier B.V. All rights reserved.

KEYWORDS:

Hepatic hemorrhage; Mechanical in-exsufflator; Peliosis hepatis; X-linked myotubular myopathy

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