Weinbaum P J, Cassidy S B, Campbell W A, Rickles F R, Vintzileos A M, Nochimson D J, Tsipouras P
Am J Perinatol. 1987 Apr;4(2):134-7. doi: 10.1055/s-2007-999756.
Ehlers-Danlos syndrome type IV (EDS IV) is a rare but serious disorder of connective tissue that is characterized by the abnormal production or secretion of type III collagen. Experience with pregnant patients exhibiting this syndrome is limited. This report describes the successful management of a pregnancy in a woman with EDS IV that included activity restriction and complete bed rest after 32 weeks, use of 1-desamino-8-D-arginine vasopressin (DDAVP) before delivery to improve coagulation, and cesarean section before the onset of labor. The possibility of prenatal diagnosis based on genetic linkage studies also is discussed.
IV型埃勒斯-当洛综合征（EDS IV）是一种罕见但严重的结缔组织疾病，其特征是III型胶原蛋白产生或分泌异常。对患有该综合征的孕妇的经验有限。本报告描述了一名患有EDS IV的女性成功妊娠的管理情况，包括活动限制以及32周后完全卧床休息、分娩前使用1-去氨基-8-D-精氨酸加压素（DDAVP）改善凝血，以及在临产前进行剖宫产。还讨论了基于基因连锁研究进行产前诊断的可能性。
