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№2' 2019
ENDOCRINOLOGYInternational Medical Journal, Vol. 25., Iss. 2, 2019, P. 65−69.
CLINICAL FEATURES OF PATIENTS WITH SYNDROME OF BIOLOGICALLY INACTIVE GROWTH HORMONE
SI "V. P. Komisarenko Institute of Endocrinology and Metabolism of the National Academy of Medical Sciences of Ukraine", Kyiv, Ukraine
The syndrome of the biologically inactive growth hormone is a new nosological unit, which in 1999 was introduced into the International Classification of the Congenital Growth Hormone Deficiency (KIGS Aetiology Classifi cation System). There is currently no reliable data on the prevalence, peculiarities of anamnesis and clinical course of this pathology. In this study, the clinical features of patients with a biologically inactive growth hormone syndrome were studied based on anamnesis, phenotype, physical and sexual development. The degree of puberty was estimated on the Tanner scale (1962). To confirm the syndrome of a biologically inactive growth hormone, a sensitivity test to the growth hormone, which was positive in all studies, was applied. The bone age of patients was studied using the atlas W. W. Greulich, S. P. Pyle (1993). There is an evidence that patients with the biologically inactive growth hormone syndrome are 12.38 % of patients with low fertility. Pathology in boys is three times more likely than girls. One third of children had cases of low birth rate among the relatives of the first and second lines. The pathological course of pregnancy in mothers was determined in 41.2 % of cases. Most of the surveyed individuals had timely sexual development with an inclination to the early, which began at 10−11 years. Early puberty was recorded in 23.53 % of children aged 8−9. The optimal physiological puberty at the age of 12−14 years was at 24.18 %. Thus, patients with the syndrome of a biologically inactive growth hormone account for the ninth part of the low−grade children who underwent a survey. The frequency of pathology in male children was significantly higher than in females. Such patients have a proportional structure of the body and a typical phenotype, the main manifestations of which are the protruding forehead, saddle−shaped nose, deep−set eyes and hypoplasia of the mandible. During the activation of pubescence, the facial features of patients change, and their phenotype loses its character.
Key words: biologically inactive growth hormone syndrome, frequency, phenotype, physical and sexual development.