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一名7个月大婴儿出现严重低氧血症和肺动脉高压:肺泡毛细血管发育不良的晚期表现

Profound hypoxemia and pulmonary hypertension in a 7-month-old infant: late presentation of alveolar capillary dysplasia.

作者信息

Ahmed Sheikh, Ackerman Veda, Faught Philip, Langston Claire

机构信息

Department of Pediatric Critical Care, , James Whitcomb Riley Hospital for Children, Indianapolis, IN, USA.

出版信息

Pediatr Crit Care Med. 2008 Nov;9(6):e43-6. doi: 10.1097/PCC.0b013e31818e383e.

Abstract

OBJECTIVE

To report a case of an oldest previously asymptomatic infant diagnosed with alveolar capillary dysplasia who lived a relatively normal life until 7 months of age.

DESIGN

Descriptive case report.

SETTING

Intensive care unit of a tertiary care children's hospital.

PATIENT

Seven-month-old female infant with profound hypoxemia and pulmonary hypertension.

CONCLUSION

Alveolar capillary dysplasia should be considered with a high index of suspicion in an infant who presents with pulmonary hypertension beyond the neonatal period and for which no anatomical cause can be found. Early consideration of open lung biopsy may prevent using costly, invasive, and probably ineffective procedures such as extracorporeal membrane oxygenation.

摘要

目的

报告一例此前无症状的最年长婴儿被诊断为肺泡毛细血管发育不良的病例,该婴儿在7个月大之前生活相对正常。

设计

描述性病例报告。

地点

一家三级儿童专科医院的重症监护病房。

患者

一名患有严重低氧血症和肺动脉高压的7个月大女婴。

结论

对于新生儿期后出现肺动脉高压且找不到解剖学病因的婴儿,应高度怀疑肺泡毛细血管发育不良。早期考虑进行开胸肺活检可避免采用如体外膜肺氧合等昂贵、有创且可能无效的治疗手段。

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