合并Goodpasture’s综合征孕妇所产新生儿伴发肺大疱一例并文献复习  

One neonate with bullae of lung whose mother diagnosed with Goodpasture＇s syndrome during pregnancy： case report and review of the literatures

作　　者：张乐嘉[1] 丁国芳[1] 王长燕[1] 沈菁[1] 李正红[1] 

机构地区：[1]中国医学科学院北京协和医院儿科,100032

出　　处：《中华围产医学杂志》2015年第7期527-532,共6页Chinese Journal of Perinatal Medicine

摘　　要：目的总结妊娠合并Goodpasture’s综合征的临床特点及其对孕妇及新生儿肺、肾等脏器的可能影响及应对措施。方法北京协和医院2011年8月23日收治1例合并Goodpasture’s综合征孕妇，其所产新生儿伴发肺大疱。总结该病例的临床诊治经过及随访结果，结合医学引文索引检索到的8例妊娠合并Goodpasture’s综合征孕妇的相关资料，总结合并Goodpasture’s综合征孕妇的临床特点及其对新生儿的影响。结果（1）病例资料：孕妇32岁，妊娠30”周，因“发热2周，水肿、胸闷、气促1周，无尿3d”入院。人院后肾功能衰竭进行性加重，于妊娠31“周剖宫产分娩一男婴，出生体重1900g。产后出现咯血，胸部CT示双肺透过度减低，可见多发斑片影。肾脏病理提示新月体性。肾小球肾炎，同时抗肾小球基底膜（glomerular basement membrane，GBM）抗体阳性，诊断Goodpasture’s综合征。并予甲泼尼龙、环磷酰胺、血浆置换、血液透析治疗。新生儿双侧脑室内出血，肾功能一过性异常，抗GBM抗体113．1EU／ml，胸部X射线片及肺部CT提示右肺肺大疱。生后15d患儿呼吸困难及右下肺病变较前加重，予甲泼尼龙治疗，3d后撤离呼吸机。出院6个月后复查抗GBM抗体阴性，肺部CT正常。随访2年，未再出现相关症状及临床表现。（2）文献复习：妊娠期合并Goodpasture’s综合征孕妇临床症状主要为恶性高血压、肾功能衰竭，而呼吸系统症状不明显，治疗上多采用血浆置换、血液透析及糖皮质激素等。其所产新生儿多为早产儿，通过胎盘进入新生儿体内的抗GBM抗体可引起新生儿脑、肾、肺等脏器损害。糖皮质激素治疗，新生儿预后多良好。结论妊娠合并Goodpasture’s综合征常表现为恶性高血压和肾功能衰竭，肾脏病理检查及抗GBM抗体检查可确诊。血浆置换、血液透析及糖皮质激素治疗效果较好。通过�Objective To summarize the clinical features and possible impacts of Goodpasture＇s syndrome in pregnancy on the pulmonary and kidney of the newborn and the mothers. Methods One patient diagnosed Goodpasture＇s syndrome in pregnancy hospitalized in Peking Union Medical College Hospital on August 23 in 2011 delivered a neonate with bullae of lung. And literatures including 8 cases of pregnancy complicated by Goodpasture＇s syndrome worldwide through Medline were reviewed. Results （1） Case report： one 31-year-old women presented with acute renal failure at 30^＋6 weeks of gestation and delivered a male infant with birth weight 1 900 g by caesarean section at 31^＋1 weeks of gestation. Diagnosis was confirmed as Goodpasture＇s syndrome with anti-glomerular basement membrane（GBM） antibodies in serum and renal biopsy after delivery. Then she was treated with methylprednisolone, cyclophosphamide, plasmapheresis and dialysis. The neonate showed the lung buUae in the right middle lobe and bilateral intraventricular hemorrhage but renal fimction was transient normal with anti-GBM as 113.1 EU/ml. The baby was treated by glucocorticoid for twomonths and clinical symptoms were improved. Anti-GBM antibodies and chest CT showed normal. After been followed up for two years, the baby was normal. （2） Literatures review： the main manifestations of Goodpasture＇s syndrome in pregnancy were malignant hypertension and renal failure but respiratory symptoms were not obvious. Treated with plasmapheresis, hematodialysis and glucocorticoid maybe have good effects. Most cases had premature delivery. Neonatal anti-GBM antibodies coming from mothers could result to cerebral, renal and pulmonary injury which could be treated by glucocorticoid. Coneluslons The Clinical features of pregnancy complicating the Goodpasture＇s syndrome are malignant hypertension and renal failure. Diagnosis was depended on positive anti-GBM antibodies and renal pathological changes and treatment were depended on plasmapheresis, hematodialys

关 键 词：抗肾小球基膜疾病 妊娠并发症 婴儿 新生 肺气肿 

分 类 号：R722.1[医药卫生—儿科]