Elhers-Danlos IV型患者妊娠期出现肾动脉瘤破裂(法)

作     者：Tassart S. Bernard P Debieve F. 李跃萍 

作者机构：D' epartement d'Obsté trique Hpital Universitaire Saint- Luc Université Catholique de Louvain (UCL) 10 avenue Hippocrate 1200 Bruxelles Belgium 

出 版 物：《世界核心医学期刊文摘（妇产科学分册）》 (Core Journal in Obstetrics/Gynecology)

年 卷 期：2006年第2卷第11期

页      面：51-52页

学科分类：1002[医学-临床医学] 100211[医学-妇产科学] 10[医学] 

主　　题：动脉瘤破裂 血管破裂 子宫破裂 产后出血 会阴裂伤 产前诊断 经典型 产科医师 个人史 

摘      要：Recall the clinical signs and personal and family medical history suggestive of Elhers-Danlos syndrome, mainly type IV. Review of literature and presentation of a clinical case observed in a female patient with Elhers-Danlos disease type IV who developed dissection of a renal artery during pregnancy. The prevalence of the syndrome is increasing, reaching, according to the latest publications 1/5000 births. It is important to determine the type of syndrome to assess prognosis. In the classic type and in the hyper mobile type, pregnancy is generally well tolerated although certain complications linked to connective tissue dysfunction such as pelvic instability, premature delivery, bleeding postpartum and perineal lacerations, may develop. Ehlers-Danlos syndrome can be associated with serious and even fatal complications for these patients: vascular dissection or uterine rupture. It is important for obstetricians to be aware of the clinical signs and symptoms suggestive of Elhers-Danlos syndrome in a pregnant patient and to know the diagnostic possibilities and potential risks. If a type IV syndrome is diagnosed, it is very important to inform the patient about the potential implications for pregnancy and maternal health, as well as the risk of transmission to the child and the possibility of antenatal diagnosis.