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Nagaeva E.V.
NII detskoĭ éndokrinologii Éndokrinologicheskogo nauchnogo tsentra, Moskva
Shiriaeva T.Iu.
NII detskoĭ éndokrinologii Éndokrinologicheskogo nauchnogo tsentra, Moskva
Peterkova V.A.
Éndokrinologicheskiĭ nauchnyĭ tsentr, Moskva
Bezlepkina O.B.
FGBU "ndokrinologicheskiĭ nauchnyĭ tsentr" Minzdrava Rossii, Moskva
Tiul'pakov A.N.
Éndokrinologicheskiĭ nauchnyĭ tsentr, Moskva
Strebkova N.A.
Institut detskoĭ éndokrinologii Éndokrinologicheskogo nauchnogo tsentra, Moskva
Kiyaev A.V.
Ural State Medical University, Yekaterinburg, Russia
Petriaĭkina E.E.
Morozovskaia detskaia gorodskaia klinicheskaia bol'nitsa
Bashnina E.B.
GBOU DPO "Rossiĭskaia meditsinskaia akademiia poslediplomnogo obrazovaniia" Minzdrava Rossii, Moskva
Malievskiĭ O.A.
Bashkirskiĭ gosudarstvennyĭ meditsinskiĭ universitet, Ufa
Taranushenko T.E.
GBOU VPO "Krasnoiarskiĭ gosudarstvennyĭ meditsinskiĭ universitet im. prof. V.F. Voĭno-Iasenetskogo" Minzdrava Rossii, Krasnoiarsk
Kostrova I.B.
Children’s Republic hospital, Machachkala, Russia
Shapkina L.A.
Pacific State Medical University, Vladivostok, Russia
Dedov I.I.
Endocrinology Research Centre
Russian national consensus. Diagnostics and treatment of hypopituitarism in children and adolescences
Journal: Problems of Endocrinology. 2018;64(6): 402‑411
DOI: 10.14341/probl0091
Views: 10427
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To cite this article:
Nagaeva EV, Shiriaeva TIu, Peterkova VA, et al. . Russian national consensus. Diagnostics and treatment of hypopituitarism in children and adolescences. Problems of Endocrinology.
2018;64(6):402‑411. (In Russ.)
https://doi.org/10.14341/probl0091
Proceedings of the National Consensus reflect the modern Russian and international experience gained in this field. Specific endocrinological examination of a short child should be preceded by exclusion of all other causes of short stature: severe decompensated somatic diseases affecting the growth rate, congenital systemic skeletal diseases, syndromic short stature (all girls with growth retardation require mandatory karyotype analysis, regardless of the presence or absence of phenotypic signs of Turner syndrome), and decompensated endocrine diseases. Specific evaluation of the GH/IGF-I axis is performed in normally-proportioned children with pronounced short stature: if the child's height is < –2.0 SDS; if the difference between the child's height SDS and the mid-parental height SDS exceeds 1.5 SDS; if the growth rate is low. The consensus provides the clear criteria for the diagnosis of GH-deficiency, central hypothyroidism, central hypocorticism, central hypogonadism, diabetes insipidus, and hypoprolactinemia as well as the criteria for their compensation. The dose of somatropin in GH-deficient children and adolescents is 0.025―0.033 mg/kg/day. In total growth hormone deficiency, especially in young children, somatropin therapy should be started with lower doses: 25―50% of the substitution therapy dose, with a gradual increase to the optimal dose for 3―6 months. The dose may be increased to 0.045―0.05 mg/kg/day in children with growth deficiency who enter puberty. In the case of side effects, the dose of somatropin may be reduced (by 30―50%) or temporarily discontinued (depending on severity of the clinical picture) until complete regression of negative symptoms. In the case of optic nerve edema, treatment is temporarily discontinued until the eye fundus condition is fully normalized. If therapy has been temporarily discontinued, the treatment is restarted at smaller doses (50% of the initial dose) with a gradual (within 1―3 months) return to the optimal dose. Growth-promoting therapy with somatropin is discontinued when the following conditions are met: a decrease in the growth rate of less than 2 cm/year during treatment, closure of the epiphyseal growth plates, or at earlier stages (achieving the genetically predicted growth, but not more than 170 cm in girls and 180 cm in boys; desire of the patient and patient's parents/legal representatives who are satisfied with the achieved result of final growth). The re-diagnosis is performed after reaching the final growth, after a 1―3 month gap in somatropin treatment. The re-diagnosis is required in patients with isolated GH-deficiency or patients with 1 (apart from GH-deficiency) tropic deficiency and with a normal IGF-1 level (after somatropin withdrawal) who lack molecular genetic confirmation of the diagnosis. The re-diagnosis is not necessary in patients with two or more (apart from GH-deficiency) tropic deficiencies, acquired hypothalamic-pituitary lesions due to pituitary surgery and irradiation of the hypothalamic-pituitary area (with a low IGF-1 level after somatropin withdrawal), MRI triad, and defects in the GH/IGF-I system gene. If GH-deficiency is confirmed, somatropin therapy is restarted with metabolic doses of 0.01―0.003 mg/kg/day under blood IGF-I level control (measured once in 6 months); the indicator should not exceed the upper reference value for an appropriate age and gender.
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Nagaeva E.V.
NII detskoĭ éndokrinologii Éndokrinologicheskogo nauchnogo tsentra, Moskva
Shiriaeva T.Iu.
NII detskoĭ éndokrinologii Éndokrinologicheskogo nauchnogo tsentra, Moskva
Peterkova V.A.
Éndokrinologicheskiĭ nauchnyĭ tsentr, Moskva
Bezlepkina O.B.
FGBU "ndokrinologicheskiĭ nauchnyĭ tsentr" Minzdrava Rossii, Moskva
Tiul'pakov A.N.
Éndokrinologicheskiĭ nauchnyĭ tsentr, Moskva
Strebkova N.A.
Institut detskoĭ éndokrinologii Éndokrinologicheskogo nauchnogo tsentra, Moskva
Kiyaev A.V.
Ural State Medical University, Yekaterinburg, Russia
Petriaĭkina E.E.
Morozovskaia detskaia gorodskaia klinicheskaia bol'nitsa
Bashnina E.B.
GBOU DPO "Rossiĭskaia meditsinskaia akademiia poslediplomnogo obrazovaniia" Minzdrava Rossii, Moskva
Malievskiĭ O.A.
Bashkirskiĭ gosudarstvennyĭ meditsinskiĭ universitet, Ufa
Taranushenko T.E.
GBOU VPO "Krasnoiarskiĭ gosudarstvennyĭ meditsinskiĭ universitet im. prof. V.F. Voĭno-Iasenetskogo" Minzdrava Rossii, Krasnoiarsk
Kostrova I.B.
Children’s Republic hospital, Machachkala, Russia
Shapkina L.A.
Pacific State Medical University, Vladivostok, Russia
Dedov I.I.
Endocrinology Research Centre
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