The materials of the National Consensus reflect the modern domestic and international experience on this issue. Before conducting a specialized endocrinological examination of a short child, all other causes of short stature should be excluded: severe somatic diseases in a state of decompensation that can affect growth velocity, congenital systemic skeletal diseases, syndromic short stature (all girls with growth retardation require a mandatory study of karyotype, depending on the presence or absence of phenotypic signs of Turner syndrome), endocrine diseases in decompensation. A specialized examination of the state of GH-IGF-I axis is carried out when the proportionally folded child has pronounced short stature: if the child’s height is < –2.0 SDS, if the difference between the child’s height SDS and child’s midparental height SDS exceeds 1.5 SDS and/or a low growth velocity. The consensus reflects clear criteria for the diagnosis of GH-deficiency, central hypothyroidism, central hypocorticosolism, central hypogonadism, diabetes insipidus, hypoprolactinemia, and also the criteria for their compensation. The dose of somatropin with GH-deficiency in children and adolescents is 0.025–0.033 mg/kg/day. With total somatotropic insufficiency, especially in young children, it is advisable to start therapy with somatropin from lower doses: 25–50% of the substitution, gradually increasing it within 3–6 months to optimal. In children with a growth deficit when entering puberty, the dose may be increased to 0.045–0.05 mg/kg/day. With the development of side effects, the dose of somatropin can be reduced (by 30–50%), or temporarily canceled (depending on the severity of the clinical picture) until the complete disappearance of undesirable symptoms. With swelling of the optic nerve, treatment is temporarily stopped until the picture of the fundus of the eye fully normalizes. If therapy has been temporarily discontinued, treatment is resumed in smaller doses (50% of the initial) with a gradual (within 1–3 months) return to the optimum. GH treatment at pediatric doses not continue beyond attainment of a growth velocity below 2–2.5 cm/year, closure of the epiphyseal growth zones, or earlier, when: the achievement of genetically predicted height, but not more than 170 cm in girls, 180 cm in boys, the patient’s desire and his parents / legal representatives satisfied with the achieved result of the final height. Re-evaluation of the somatotropic axis is carried out after reaching the adult height, after 1–3 months GH therapy will be discontinued. Patients with isolated GH-deficiency or patients with 1 (besides GH) pituitary hormone deficiencies in the presence of a normal IGF-1 level (against the background of somatropin withdrawal) and not having molecular genetic confirmation of the diagnosis need re- evaluation. Patients with two or more (besides GH) pituitary hormone deficiencies, acquired hypothalamic-pituitary lesions due to operations on the pituitary and irradiation of the hypothalamic-pituitary area (if the IGF-1 level is low against somatropin withdrawal), specific pituitary/ hypothalamic structural defect on MRI, gene defects of the GH-IGF-I system do not need re- evaluation. If GH deficiency is confirmed, treatment with somatropin is resumed at metabolic doses of 0.01—0.003 mg/kg/day under the control of the IGF-I level in the blood (measurement 1 time in 6 months), the indicator should not exceed the upper limit of the reference value for the corresponding age and floor.
Central precocious puberty occupies an important place in the practice of a pediatric endocrinologist. If the patient reveals signs of premature sexual development, the diagnostic search is aimed at eliminating the tumor origin of both false (peripheral) and gonadotropin-dependent, or central, precocious puberty, as well as gonadotropin-independent forms of premature sexual development. Oncological alertness is important in the work of not only a pediatric endocrinologist, but also a pediatrician. In the treatment of all non-tumor forms of central precocious puberty, drugs of the group of analogues of gonadotropin-releasing hormone are used, which allows to stop the progression of sexual development, reduce the rate of bone maturation and, thereby, increase the final growth of the child. The most common idiopathic variant of central precocious puberty. The article presents a clinical case of observing a patient with an idiopathic variant of central premature sexual development during therapy with a drug from the group of analogues of gonadotropin releasing hormone of prolonged action. The classical course of the idiopathic variant of central precocious puberty with typical diagnostic difficulties in the onset of the disease, good compensation against the background of therapy with a drug from the group of agonists of gonadotropin-releasing hormone and normal puberty 612 months after cancellation of the therapy is demonstrated. The latter is explained by the proven reversibility of the effects of this group of drugs. The description of this clinical case, in the authors opinion, should be of interest to doctors at the local pediatricians and pediatricians working in the medical care departments for children in educational institutions.
A female patient with congenital hypopituitarism is followed up at the Children’s Endocrinology Centre (St. Petersburg, Russia). Growth hormone deficiency was confirmed by the diagnostic stimulation test; the maximum peak value of growth hormone was 8.3 ng/ml. At the moment of diagnosis, the growth deficit was –3.9 SDS. MRI showed the «empty Turkish saddle», the heterogeneous structure of the pituitary gland. No dysfunction of the other endocrine glands was identified. Bone age lagged behind the chronological age and was 9 years. The somatogenic causes of growth delay and chromosomal abnormalities were ruled out. Molecular genetic testing of the genes associated with hypopituitarism revealed no mutations. Growth hormone therapy was started in a daily dose of 0.033 mg/kg body weight. Two months after the growth hormone therapy had been started, the patient was admitted to the Surgical Department with the symptoms of «acute abdomen». The growth hormone therapy was suspended. The patient was diagnosed with Crohn’s disease upon further examination. After surgical treatment and prescription of specific therapy with Remicade, treatment with growth hormone was resumed after the 6-month break. Now the patient is receiving replacement therapy with growth hormone and permanent therapy of the concomitant Crohn’s disease.
Central precocious puberty (CPP) occupies an important place in the practice of pediatric endocrinologist. In the treatment of all forms of CPP, there are used drugs of GnRH (gonadotropin-releasing hormone) agonists group, whose pharmacological effect of is based on desensitization of the pituitary gland to the stimulating effect of GnRH. Therapy with agonist of gonadotropin-releasing hormone allows to stop the progression of sexual development, reduce the rate of bone maturation and, thereby, increase the final growth of the child. The article demonstrates the structure of the dispensary group of patients with CPP who were treated with the agonists GnRH of prolonged action. There has been conducted the analysis of the observation results of patients with idiopathic CPP who received 3.75 mg Triptorelin therapy in the standard regimen once every 28 days and transferred to Tryptorelin 11.25 mg once every 3 months, as well as patients with different forms of CPP with a newly established diagnosis. The presented results of treatment with 11.25 mg Triptorelin drugs by intramuscular injection in a regimen of 1 time in 3 months in comparison with the results of treatment with 3.75 mg of Triptorelin patients in the regimen of intramuscular injections once every 28 days in patients with CPP showed their effectiveness. Preparations of the agonists GnRH group of prolonged action inhibit the development of secondary sexual characteristics, lead to a decrease in the size of the internal genitalia in female and external genitalia in male and reduce the progression of bone age. It was also noted that reducing the frequency of injections of drugs of this group from 1 time in 28 days to 1 time in 3 months positively affects the emotional state of children receiving this treatment for a long period (3-6 years).
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