PDF(Pubmed)
Abstract：
BACKGROUND: Congenital fiber-type disproportion (CFTD) is a form of congenital myopathy. CFTD is rare, especially when presenting in patients with critical illnesses. Here, we report a case of CFTD presenting with type II respiratory failure after delivery and provide a review of the literature on CFTD.
METHODS: A 30-year-old woman was admitted to the obstetrics department of our hospital with premature rupture of the fetal membrane and with 7 h of regular contractions. After delivery, the patient experienced a refractory type II respiratory failure. Physical examination along with diagnostic procedures such as electromyography and biopsy confirmed CFTD. Use of invasive ventilator followed by intermittent use of noninvasive ventilator attenuated her symptoms. The patient recovered after ventilator-assisted respiration and was weaned off the noninvasive ventilator on the seventh day postpartum.
CONCLUSIONS: Congenital myopathy should be considered a differential diagnosis for type II respiratory failures that cannot be attributed to other diseases.
METHODS: A 30-year-old woman was admitted to the obstetrics department of our hospital with premature rupture of the fetal membrane and with 7 h of regular contractions. After delivery, the patient experienced a refractory type II respiratory failure. Physical examination along with diagnostic procedures such as electromyography and biopsy confirmed CFTD. Use of invasive ventilator followed by intermittent use of noninvasive ventilator attenuated her symptoms. The patient recovered after ventilator-assisted respiration and was weaned off the noninvasive ventilator on the seventh day postpartum.
CONCLUSIONS: Congenital myopathy should be considered a differential diagnosis for type II respiratory failures that cannot be attributed to other diseases.
摘要：
背景：先天性纤维型不称（CFTD）是一种先天性肌病。CFTD是罕见的，尤其是在患有危重疾病的患者中出现时。这里,我们报告了一例分娩后出现II型呼吸衰竭的CFTD病例,并对CFTD相关文献进行了综述.
方法：我院产科收治1名30岁女性胎膜早破，规律宫缩7h。交货后,患者出现难治性Ⅱ型呼吸衰竭.体格检查以及诸如肌电图和活检等诊断程序证实了CFTD。使用有创呼吸机，然后间歇性使用无创呼吸机减轻了她的症状。患者在呼吸机辅助呼吸后康复，并在产后第七天断奶。
结论：先天性肌病应被视为不能归因于其他疾病的II型呼吸衰竭的鉴别诊断。
方法：我院产科收治1名30岁女性胎膜早破，规律宫缩7h。交货后,患者出现难治性Ⅱ型呼吸衰竭.体格检查以及诸如肌电图和活检等诊断程序证实了CFTD。使用有创呼吸机，然后间歇性使用无创呼吸机减轻了她的症状。患者在呼吸机辅助呼吸后康复，并在产后第七天断奶。
结论：先天性肌病应被视为不能归因于其他疾病的II型呼吸衰竭的鉴别诊断。
