Endocrinopathies and cardiopathies in patients with Turner syndrome

Zaidieva Ya.Z., Balan V.E., Serebrennikova K.G.

1) Moscow Regional Research Institute of Obstetrics and Gynecology, Moscow, Russia; 2) Central Clinical Hospital, Russian Academy of Sciences, Moscow, Russia
Ovarian dysgenesis, stunting, and infertility are common characteristics of Turner syndrome (TS). Endocrinopathies and cardiopathies are less common, but can cause significant complications and are a leading cause of death in patients with TS. The prevalence of endocrine abnormalities, including osteoporosis that occurs in almost 60% of patients, hypothyroidism (3.2%) and glucose metabolism disorders (that occur with a variable frequency) are related to the age group. Therapy with estrogens and growth hormones alone or in combination improves bone density in patients with TS. Autoimmune thyroiditis is associated with the X-isochromic phenotype in women with TS in the first and second decades of life. Type 2 diabetes mellitus developing due to Xp haploid gene deficiency and a latent autoimmune β-cell response occurs with higher frequency in TS patients in different age groups. The prevalence of cardiopathies in TS patients is 23%; the most common cardiovascular malformations are coarctation of the aorta and bicuspid aortic valve. The risks of cardiovascular events require ultrasound monitoring throughout a patient’s entire life, during pregnancy in particular. TS patients need interdisciplinary and long-term care. This paper provides an overview of endocrinopathies and cardiopathies in patients with TS.

Keywords

Turner syndrome
estrogen deficiency
endocrinopathies
cardiopathies

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Received 01.10.2019

Accepted 04.10.2019

About the Authors

Yansiyat Z. Zaydieva, MD, Professor, Head of the Department of Gynecological Endocrinology, Moscow Regional Research Institute of Obstetrics and Gynecology, Moscow.
101000, Moscow, ul. Pokrovka, 22A.
Vera E. Balan, MD, professor, head of the outpatient department, Moscow Regional Research Institute of Obstetrics and Gynecology, Russian Federation.
101000, Moscow, st. Pokrovka, 22A.
Klara G. Serebrennikova, MD, professor, supervisor of obstetrics and gynecology of the Central Clinical Hospital of the Russian Academy of Sciences.
Moscow, st. Lithuanian, d.1a.

For citation: Zaidieva Ya.Z., Balan V.E., Serebrennikova K.G.
Endocrinopathies and cardiopathies in patients with Turner syndrome.
Akusherstvo i Ginekologiya/ Obstetrics and gynecology. 2020; 2: 14-20 (In Russian).
https://dx.doi.org/10.18565/aig.2020.2.14-20

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