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脊髓性肌萎缩症合并严重限制性肺病患者的妊娠情况

Pregnancy in a Patient with Spinal Muscular Atrophy and Severe Restrictive Lung Disease.

作者信息

Alamri Lamia A, Foxx Alexandra M, Dwan Robyn L, Sorabella Laura E, Patel Soha S

机构信息

Department of Obstetrics and Gynecology, University of Colorado Anschutz Medical Campus, Aurora, Colorado.

Department of Obstetrics and Gynecology, University of Michigan Health, Ann Arbor, Michigan.

出版信息

AJP Rep. 2023 Dec 15;13(4):e98-e101. doi: 10.1055/a-2184-0756. eCollection 2023 Jul.

DOI:10.1055/a-2184-0756
PMID:38106965
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10723959/
Abstract

Spinal muscular atrophy (SMA) is a rare autosomal recessive neuromuscular disease that is often associated with chronic respiratory failure. Few cases have described the antepartum and postpartum course in patients with severely compromised respiratory status. We present a case of a 24-year-old nullipara with a history of SMA type II complicated by surgically corrected kyphoscoliosis and severe restrictive lung disease. Her pregnancy was complicated by progressively worsening dyspnea resulting in increased use of noninvasive positive pressure ventilation, ultimately leading to indicated premature delivery at 28 weeks' gestation via cesarean section under general anesthesia. Women with SMA and severe restrictive lung disease are at high risk of premature delivery secondary to worsening respiratory status. A multidisciplinary approach is vital in treating these patients.

摘要

脊髓性肌萎缩症(SMA)是一种罕见的常染色体隐性神经肌肉疾病,常与慢性呼吸衰竭相关。很少有病例描述呼吸状况严重受损患者的产前和产后病程。我们报告一例24岁未孕女性,有II型SMA病史,合并手术矫正的脊柱后凸侧弯和严重的限制性肺病。她的妊娠因进行性加重的呼吸困难而复杂化,导致无创正压通气使用增加,最终在妊娠28周时在全身麻醉下经剖宫产进行了适时早产。患有SMA和严重限制性肺病的女性因呼吸状况恶化而有早产的高风险。多学科方法对治疗这些患者至关重要。

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本文引用的文献

1
Pregnancy outcomes in women with spinal muscular atrophy: A review.脊髓性肌萎缩症女性的妊娠结局:综述。
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Restrictive lung disease in pregnancy.妊娠合并限制性肺疾病。
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Anesthetic management of spinal muscle atrophy type II in a parturient.一名患有II型脊髓性肌萎缩症产妇的麻醉管理
Local Reg Anesth. 2011;4:15-20. doi: 10.2147/LRA.S17089. Epub 2011 Apr 6.
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Management of a pregnancy complicated by type III spinal muscular atrophy.合并III型脊髓性肌萎缩症的妊娠管理
BMJ Case Rep. 2011 Feb 17;2011:bcr1020103402. doi: 10.1136/bcr.10.2010.3402.
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Pregnancy course and outcome in women with hereditary neuromuscular disorders: comparison of obstetric risks in 178 patients.遗传性神经肌肉疾病女性的妊娠过程及结局:178例患者产科风险比较
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