福山型先天性筋ジストロフィー年長患者における臨床経過と各種人工呼吸療法の臨床効果と評価

DOI PubMed 被引用文献2件 参考文献14件

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タイトル別名
  • Fukuyama Type Congenital Muscular Dystrophy in Its Advanced Stage: Effectiveness of Mechanical Ventilation Systems.

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We studied the clinical courses of Fukuyama type congenital muscular dystrophy (FCMD) based on a longterm follow-up of 7 patients. Their peak motor function varied from controlling the head incompletely to standing with support. Six patients had epilepsy, which in some cases was diagnosed with difficulty because of changing seizure types and multiple joint contracture. Six patients underwent endotracheal intubation, and in 5 of them long-term mechanical ventilation began at the age of 15 to 20 years. Three of them underwent trachestomy intermittent positive pressure ventilation (TIPPV), whereas recently 2 patients were treated successfully with nasal intermittent positive pressure ventilation (NIPPV). Mechanical in-exsufflation (MI-E) was effective in clearing airway secretion of 4 patients. With disease progression, the motor, mental, nutritional, cardiorespiratory and swallowing functions of FCMD patients should be assessed to ensure adequate management by NIPPV and MI-E.

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  • 脳と発達

    脳と発達 34 (4), 330-335, 2002

    THE JAPANESE SOCIETY OF CHILD NEUROLOGY

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