Репозиторий Университета

© 2018 Izdatel'stvo Meditsina. All rights reserved. The problem of childbearing in patients with myasthenia gravis (MG) is extremely important, since the disease most often affects women in the reproductive period. Has not yet been determined neither the prognostic criteria for myasthenia exacerbation during pregnancy, nor the predictors for the development of the transient neonatal myasthenia gravis (TNM). The article analyzes the literature data from the first descriptions of pregnancy in patients with myasthenia and TNM until the present days. The evolution of the concept of the role of a high titer of antibodies to acetylcholine receptors (anti-AChR Ab) in the development of exacerbations of MG in the mother and TNM in her newborn is shown. The role of Ab against γ-subunits of AChR in the development of arthrogryposis and THM is discussed. The importance of planning the pregnancy in myasthenic mothers is emphasized. These observations show that the same woman has either a favorable course of MG with the birth of a healthy child, or has a severe exacerbation until the postpartum myasthenic crisis with the birth of baby with TNM depending on quality of remission before pregnancy. Based on the literature data and own experience, the indisputable role of thymectomy in the prevention of exacerbations of MG and TNM is shown. Own observation of cases of TNM demonstrates the crucial role of neostigmine test in the recognizing of «floppy baby» syndrome. For the first time in Russia, a study of anti-fetal/anti-adult AChR Ab ratio in the umbilical cord blood was conducted. For the first time in the world, a unique case of the birth of a healthy child in a couple of patients with juvenile MG is presented. The husband suffering of the severe refractory MG with elevated titre of anti-AChR Ab up to 20.8 nmol/l and a lot of congenital stigmas of dysembryogenesis. The wife had a mild course of MG. She was carefully prepared for pregnancy by thymectomy and glucocorticoid-therapy and had a stable condition for more than 10 years at the time of pregnancy. The titer of anti-AChR Ab was relatively low (9.0 nmol/L), however, the pool of anti-AChR Ab in the umbilical cord blood mainly contained anti-fetal AChR AB (92%). This example shows that it is the quality of remission of the mother's MG at the time of pregnancy determines the course of the disease during pregnancy and the risks of TNM. This case allows us to consider genetic factors as secondary and once again emphasizes the autoimmune nature of myasthenia gravis.