Problems of hyposomatotropism in the adulthood

Abstract

Orphan diseases are a group of rare congenital or acquired diseases that are characterized by severe, progressive and chronic course, which leads to the formation of degenerative changes in the body, reducing the quality and life expectancy of patients. In Ukraine the problem of orphan diseases and their social support were recognized at the state level only on April 15, 2014 with the adoption of the Law of Ukraine № 1213-VII «On Amendments to the Fundamentals of the Legislation of Ukraine on Health Care for the Prevention and Treatment of Rare (Orphan) Diseases». Nowadays there are up to 8,000 rare diseases in the world, but only 302 of these nosologies have been officially approved in Ukraine and are regulated by the relevant orders of the Ministry of Health of Ukraine. In Ukraine there is a problem with the treatment of orphan diseases due to the lack of the National Register. One of the diseases that is included in the registered list of orphan diseases and requires endocrinological care is somatotropic insufficiency. Somatotropic insufficiency is a heterogeneous group of diseases, the main clinical manifestation of which is a pathological growth retardation caused by impaired secretion or action of somatotropic hormone. To analyze the general incidence of somatotropic insufficiency among children in Vinnytsia and Vinnytsia region and the state of care. Materials and Methods. Database of the register of patients with somatotropic insufficiency of Vinnytsia Regional Clinical Highly Specialized Endocrinology Center of Vinnytsia Regional Council and outpatient cards. Results. According to the register, the number of registered patients aged 4 to 18 with hyposomatotropism is 66, of whom male predominates (58%). Among these patients, the largest number are children of primary school age (45.4%). Regarding the verification of the diagnosis of somatotropic insufficiency, pituitary dwarfism is most common (57.6%). Conclusions. Somatotropic hormone is a protein hormone of the adenohypophysis, which contains 191 amino acid residues and is synthesized by special acidophilic somatotrophic cells. The main biological action of somatotropic hormone in childhood is to ensure growth. Growth is a complex process, such as the metabolism, the impact on the components of growth zones, the interaction on the function of internal organs. There is a question of control over the effects of the absence of somatotropic hormone on the function of the cardiovascular, muscular and musculoskeletal systems in elderly patients without replacement therapy. Organizational issues of the register, providing patients with replacement therapy in adulthood, control of concomitant conditions and complications are relevant and need to address this issue.