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[埃勒斯-当洛综合征与妊娠。关于一例病例]

[Ehlers-Danlos syndrome and pregnancy. Apropos of a case].

作者信息

Charvet P Y, Salle B, Rebaud P, Dutruge J, Cordier M P, Pic J C, Touraine J, Berland M, Levrat R, Tourniaire J

机构信息

Service de Gynécologie-Obstétrique, Faculté de Médecine, Lyon Nord, Hôpital Claude-Bernard, Oullins.

出版信息

J Gynecol Obstet Biol Reprod (Paris). 1991;20(1):75-8.

PMID:2019724
Abstract

Ehlers-Danlos Syndrome is a hereditary dysplasia of connective tissue with an abnormality in collagen synthesis. The syndrome consists of increased elasticity and fragility of the skin, increased laxity of the ligaments of the joints and fragility of the blood vessels. It is rarely associated with pregnancy but when it is several problems arise which are demonstrated in our case. Our patient had a type III syndrome (hypermobility of the joints) or a type IV syndrome (echymoses, Sack-Barabas Syndrome). In the literature the following are reported: premature rupture of the membranes with premature delivery, tears, perineal haematomas and type IV ruptures of the great vessels of the uterus. The best way of delivering and anaesthetising the patient are discussed. The prognosis depends above all on the type of the disease (25% of mothers die in type IV disease). Strict rest and Pfannestiel lower segment Caesarean section operation under general anaesthetic allowed us to achieve a favorable outcome for the mother and for her child.

摘要

埃勒斯-当洛综合征是一种遗传性结缔组织发育异常疾病,伴有胶原蛋白合成异常。该综合征表现为皮肤弹性增加和脆弱性增加、关节韧带松弛度增加以及血管脆弱性增加。它很少与妊娠相关,但一旦发生则会出现一些问题,我们的病例就展示了这些情况。我们的患者患有III型综合征(关节活动过度)或IV型综合征(瘀斑、萨克-巴拉巴斯综合征)。文献报道了以下情况:胎膜早破伴早产、撕裂伤、会阴血肿以及子宫大血管IV型破裂。文中讨论了患者分娩和麻醉的最佳方式。预后首先取决于疾病类型(IV型疾病中25%的母亲会死亡)。严格卧床休息以及在全身麻醉下进行耻骨联合上横切口剖宫产手术使我们为母亲和孩子都取得了良好的结局。

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