Аннотация:
Background: Mesenchymal dysplasias or inherited connective tissue diseases are the group of diseases with deficiency of various components of connective tissue. Connective tissue disorders can affect different organs: skeleton, sight organ, skin, lungs, heart. But the most dangerous is vascular wall insufficiency leading to high risk of hemorrhage, especially during pregnancy and delivery due to hemodynamic and hormonal effects on the walls of the modified vessels. Aim: To evaluate the risk of complications during the pregnancy and delivery in patients with mesenchymal dysplasias. Study design: Fifty-six pregnancies in patients with mesenchymal dysplasias, including subclinical forms of diseases: 23 with Marfan syndrome (I group), 22 with Ehlers–Danlos syndrome (II group), and 11 with Osler–Weber–Rendu syndrome (hereditary hemorrhagic telangiectasia) (III group) of the age from 18 to 36. The study included retrospective analysis (for the period from 1993 to 2005) and prospective study.
Ключевые слова:
Aortic dissection in pregnant women; congenital connective tissue pathology; Ehlers–Danlos syndrome; hereditary hemorrhagic telangiectasia; mesenchymal dysplasia
adult; Article; blood clotting disorder; cardiovascular disease; cesarean section; conjunctival hemorrhage; controlled study; Ehlers Danlos syndrome; epistaxis; female; gastrointestinal hemorrhage; hematoma; hemoptysis; high risk population; human; labor; labor complication; major clinical study; Marfan syndrome; mucosa telangiectasia; obstetric delivery; pathogenesis; pregnancy complication; pregnant woman; prenatal care; priority journal; prospective study; Rendu Osler Weber disease; retrospective study; risk assessment; skin telangiectasia; telangiectasia; vagina bleeding; vaginal injury; complication; Ehlers Danlos syndrome; Marfan syndrome; pregnancy; pregnancy complication; Rendu Osler Weber disease; Russian Federation; young adult; Adult; Ehlers-Danlos Syndrome; Female; Humans; Marfan Syndrome; Pregnancy; Pregnancy Complications; Retrospective Studies; Russia; Telangiectasia, Hereditary Hemorrhagic; Young Adult
Makatsariya A. D. Aleksandr Davidovich 1944-
Radetskaya L. S. Lyudmila Sergeevna 1980-
Bitsadze V. O. Viktoriya Omarovna 1970-
Khizroeva D. Kh. Dzhamilya Khizrievna 1973-
Khamani N. M. Nadina Moktarovna 1991-
Makatsariya N. A. Nataliya Aleksandrovna 1984-
Макацария А. Д. Александр Давидович 1944-
Радецкая Л. С. Людмила Сергеевна 1980-
Бицадзе В. О. Виктория Омаровна 1970-
Хизроева Д. Х. Джамиля Хизриевна 1973-
Хамани Н. М. Надина Моктаровна 1991-
Макацария Н. А. Наталия Александровна 1984-
Prenatal care and labor in patients with mesenchimal dysplasias (Marfan syndrome, Ehlers–Danlos syndrome, hereditary hemorrhagic telangiectasia)
Текст визуальный непосредственный
Journal of Maternal-Fetal and Neonatal Medicine
Parthenon Publishing Group
Vol.33, Issue3 P. 373-379
2020
Статья
Aortic dissection in pregnant women congenital connective tissue pathology Ehlers–Danlos syndrome hereditary hemorrhagic telangiectasia mesenchymal dysplasia
adult Article blood clotting disorder cardiovascular disease cesarean section conjunctival hemorrhage controlled study Ehlers Danlos syndrome epistaxis female gastrointestinal hemorrhage hematoma hemoptysis high risk population human labor labor complication major clinical study Marfan syndrome mucosa telangiectasia obstetric delivery pathogenesis pregnancy complication pregnant woman prenatal care priority journal prospective study Rendu Osler Weber disease retrospective study risk assessment skin telangiectasia telangiectasia vagina bleeding vaginal injury complication Ehlers Danlos syndrome Marfan syndrome pregnancy pregnancy complication Rendu Osler Weber disease Russian Federation young adult Adult Ehlers-Danlos Syndrome Female Humans Marfan Syndrome Pregnancy Pregnancy Complications Retrospective Studies Russia Telangiectasia, Hereditary Hemorrhagic Young Adult
Background: Mesenchymal dysplasias or inherited connective tissue diseases are the group of diseases with deficiency of various components of connective tissue. Connective tissue disorders can affect different organs: skeleton, sight organ, skin, lungs, heart. But the most dangerous is vascular wall insufficiency leading to high risk of hemorrhage, especially during pregnancy and delivery due to hemodynamic and hormonal effects on the walls of the modified vessels. Aim: To evaluate the risk of complications during the pregnancy and delivery in patients with mesenchymal dysplasias. Study design: Fifty-six pregnancies in patients with mesenchymal dysplasias, including subclinical forms of diseases: 23 with Marfan syndrome (I group), 22 with Ehlers–Danlos syndrome (II group), and 11 with Osler–Weber–Rendu syndrome (hereditary hemorrhagic telangiectasia) (III group) of the age from 18 to 36. The study included retrospective analysis (for the period from 1993 to 2005) and prospective study.