We present a case of a teenage boy with a Rabson-Mendenhall syndrome. There are only few recent publications on the topic of Rabson-Mendenhall syndrome in medical literature. This syndrome appears with a same frequency in both sexes. The disease prevalence is still unknown due to many undiagnosed cases linked with high mortality in early childhood. There are no prenatal screening for this disease till now, but it is clear, that in case of positive diagnostic tests, abortion should be recommended. The typical clinical symptoms of Rabson-Mendenhall syndrome are the following: physical development delay, loss of subcutaneous fat, teeth and nails abnormalities (premature teeth eruption, teeth number doubling, nails thickening). The earliest signs of the syndrome are skin hyperpigmentation and hyperkeratosis at neck, armpits and groin, which are typical for insulin resistance. Children with Rabson-Mendenhall syndrome usually have early manifestation of diabetes mellitus, characterized by labile disease course and frequent ketoacidosis state. The present clinical case might be interesting regarding a long follow-up of the child.
The article is devoted to the analysis of clinical and anthropometric indicators of 38 children and adolescents with pituitary dwarfism in the Stavropol region, receiving replacement therapy with recombinant growth hormone (rGH). The authors demonstrated a high growth stimulating efficacy of the rGR, as well as a positive impact on the level of blood cholesterol in children with somatotropic deficiency. Prognostic factors influencing the effectiveness of treatment were found (chronological age of initiation of therapy, baseline SDS of growth). In particular, it is shown that therapy with preparations of GH in children aged 8 to 10 years allows you to achieve a higher growth-stimulating effect in comparison with patients whose treatment is carried out at a later age. The study of the relationship between the initial deviation of the length of the body and the results of therapy in patients with growth hormone deficiency illustrates that a pronounced lag in growth is a predictive factor provides a more significant effect drugs rGR.
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