中华围产医学杂志
中華圍產醫學雜誌
중화위산의학잡지
CHINESE JOURNAL OF PERINATAL MEDICINE
2015年
7期
527-532
,共6页
张乐嘉%丁国芳%王长燕%沈菁%李正红
張樂嘉%丁國芳%王長燕%瀋菁%李正紅
장악가%정국방%왕장연%침정%리정홍
抗肾小球基膜疾病%妊娠并发症%婴儿,新生%肺气肿
抗腎小毬基膜疾病%妊娠併髮癥%嬰兒,新生%肺氣腫
항신소구기막질병%임신병발증%영인,신생%폐기종
Anti-glomerular basement membrane disease%Pregnancy complications%Infant,newborn%Pulmonary emphysema
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+6周，因“发热2周，水肿、胸闷、气促1周，无尿3 d”入院。入院后肾功能衰竭进行性加重，于妊娠31+1周剖宫产分娩一男婴，出生体重1900 g。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113.1 EU/ml，胸部X射线片及肺部CT提示右肺肺大疱。生后15 d患儿呼吸困难及右下肺病变较前加重，予甲泼尼龙治疗，3 d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，必要时可予糖皮质激素治疗。
目的：總結妊娠閤併Goodpasture's綜閤徵的臨床特點及其對孕婦及新生兒肺、腎等髒器的可能影響及應對措施。方法北京協和醫院2011年8月23日收治1例閤併Goodpasture's綜閤徵孕婦，其所產新生兒伴髮肺大皰。總結該病例的臨床診治經過及隨訪結果，結閤醫學引文索引檢索到的8例妊娠閤併Goodpasture's綜閤徵孕婦的相關資料，總結閤併Goodpasture's綜閤徵孕婦的臨床特點及其對新生兒的影響。結果（1）病例資料：孕婦32歲，妊娠30+6週，因“髮熱2週，水腫、胸悶、氣促1週，無尿3 d”入院。入院後腎功能衰竭進行性加重，于妊娠31+1週剖宮產分娩一男嬰，齣生體重1900 g。產後齣現咯血，胸部CT示雙肺透過度減低，可見多髮斑片影。腎髒病理提示新月體性腎小毬腎炎，同時抗腎小毬基底膜（glomerular basement membrane， GBM）抗體暘性，診斷Goodpasture's綜閤徵。併予甲潑尼龍、環燐酰胺、血漿置換、血液透析治療。新生兒雙側腦室內齣血，腎功能一過性異常，抗GBM抗體113.1 EU/ml，胸部X射線片及肺部CT提示右肺肺大皰。生後15 d患兒呼吸睏難及右下肺病變較前加重，予甲潑尼龍治療，3 d後撤離呼吸機。齣院6箇月後複查抗GBM抗體陰性，肺部CT正常。隨訪2年，未再齣現相關癥狀及臨床錶現。（2）文獻複習：妊娠期閤併Goodpasture's綜閤徵孕婦臨床癥狀主要為噁性高血壓、腎功能衰竭，而呼吸繫統癥狀不明顯，治療上多採用血漿置換、血液透析及糖皮質激素等。其所產新生兒多為早產兒，通過胎盤進入新生兒體內的抗GBM抗體可引起新生兒腦、腎、肺等髒器損害。糖皮質激素治療，新生兒預後多良好。結論妊娠閤併Goodpasture's綜閤徵常錶現為噁性高血壓和腎功能衰竭，腎髒病理檢查及抗GBM抗體檢查可確診。血漿置換、血液透析及糖皮質激素治療效果較好。通過胎盤進入新生兒體內的抗GBM抗體可引起新生兒腦、腎、肺等髒器損害，必要時可予糖皮質激素治療。
목적：총결임신합병Goodpasture's종합정적림상특점급기대잉부급신생인폐、신등장기적가능영향급응대조시。방법북경협화의원2011년8월23일수치1례합병Goodpasture's종합정잉부，기소산신생인반발폐대포。총결해병례적림상진치경과급수방결과，결합의학인문색인검색도적8례임신합병Goodpasture's종합정잉부적상관자료，총결합병Goodpasture's종합정잉부적림상특점급기대신생인적영향。결과（1）병례자료：잉부32세，임신30+6주，인“발열2주，수종、흉민、기촉1주，무뇨3 d”입원。입원후신공능쇠갈진행성가중，우임신31+1주부궁산분면일남영，출생체중1900 g。산후출현각혈，흉부CT시쌍폐투과도감저，가견다발반편영。신장병리제시신월체성신소구신염，동시항신소구기저막（glomerular basement membrane， GBM）항체양성，진단Goodpasture's종합정。병여갑발니룡、배린선알、혈장치환、혈액투석치료。신생인쌍측뇌실내출혈，신공능일과성이상，항GBM항체113.1 EU/ml，흉부X사선편급폐부CT제시우폐폐대포。생후15 d환인호흡곤난급우하폐병변교전가중，여갑발니룡치료，3 d후철리호흡궤。출원6개월후복사항GBM항체음성，폐부CT정상。수방2년，미재출현상관증상급림상표현。（2）문헌복습：임신기합병Goodpasture's종합정잉부림상증상주요위악성고혈압、신공능쇠갈，이호흡계통증상불명현，치료상다채용혈장치환、혈액투석급당피질격소등。기소산신생인다위조산인，통과태반진입신생인체내적항GBM항체가인기신생인뇌、신、폐등장기손해。당피질격소치료，신생인예후다량호。결론임신합병Goodpasture's종합정상표현위악성고혈압화신공능쇠갈，신장병리검사급항GBM항체검사가학진。혈장치환、혈액투석급당피질격소치료효과교호。통과태반진입신생인체내적항GBM항체가인기신생인뇌、신、폐등장기손해，필요시가여당피질격소치료。
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 bullae in the right middle lobe and bilateral intraventricular hemorrhage but renal function was transient normal with anti-GBM as 113.1 EU/ml. The baby was treated by glucocorticoid for two months 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. Conclusions 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, hematodialysis and glucocorticoid. Neonatal cerebral, renal and pulmonary injury resulting from anti-GBM antibodies coming from mothers should be followed up, and glucocorticoid should be taken if necessary.
