Pregravid preparation and management of pregnancy in patients with connective tissue dysplasia

Гинекология

Yu E Dobrokhotova¹  E I Borovkova¹ 

[1] N.I.Pirogov Russian National Research Medical University of the Ministry of Health of the Russian Federation;

关键词: connective tissue dysplasia;    pregravid preparation;    pregnancy;    magnesium;    marfan syndrome;    ehlers-danlos syndrome;    aortic aneurysm;    premature birth;   

DOI  :  

来源: DOAJ

【 摘 要 】

Connective tissue dysplasia is a genetically determined condition characterized by a violation of the structural organization and synthesis of the main components of connective tissue. The prevalence of non-syndromic forms is extremely high and to 46.6-72.0% is due to cofactor deficiency of magnesium. Typical obstetric complications in Ehlers-Danlos syndrome are bleeding, early formation of isthmicocervical insufficiciency, premature and rapid delivery, delayed fetal growth, symphysiopathy, rupture of the pubic joint, rupture of the perineum, spontaneous ruptures of the skin, vagina and internal organs, postpartum bleeding and incompetence of the pelvic floor muscles, bladder, urethra and rectum. Marfan syndrome in 40% of cases develop premature rupture of membranes, premature birth and miscarriage at an early age. Maternal mortality is 0.74 per 100 000 births and is associated with lamination (1%) or rupture of the aortic aneurysm (3%). With nonsynthetic forms of connective tissue dysplasia at the stage of pregravid preparation and throughout pregnancy, the appointment of magnesium preparations is indicated.

【 授权许可】

Unknown