Fatal hepatic hemorrhage by peliosis hepatis in X-linked myotubular myopathy: a case report.
Neuromuscul Disord
; 23(11): 917-21, 2013 Nov.
Article
em En
| MEDLINE
| ID: mdl-24011703
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.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Temas:
Geral
Base de dados:
MEDLINE
Assunto principal:
Peliose Hepática
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Insuflação
/
Miopatias Congênitas Estruturais
/
Hemorragia
Tipo de estudo:
Diagnostic_studies
Limite:
Child, preschool
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Humans
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Male
Idioma:
En
Revista:
Neuromuscul Disord
Assunto da revista:
NEUROLOGIA
Ano de publicação:
2013
Tipo de documento:
Article