{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,30]],"date-time":"2026-03-30T15:34:41Z","timestamp":1774884881844,"version":"3.50.1"},"reference-count":26,"publisher":"Endocrinology Research Centre","issue":"4","license":[{"start":{"date-parts":[[2022,5,14]],"date-time":"2022-05-14T00:00:00Z","timestamp":1652486400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.probl-endojournals.ru\/jour\/about\/editorialPolicies#openAccessPolicy"}],"content-domain":{"domain":["www.probl-endojournals.ru"],"crossmark-restriction":true},"short-container-title":["Probl Endokrinol (Mosk)"],"abstract":"<jats:p>Itsenko\u2013Cushing\u2019s disease is a rare, multisystem disease characterized by the presence of endogenous central hypercortisolism due to an ACTH-secreting brain tumor. The frequency of Itsenko-Cushing\u2019s disease in adulthood is 0.7\u20132.4 per 1 million population, and only 10% of all cases occur in childhood. The age of onset of the disease in children is on average 12.0\u201314.8 years. A typical manifestation of the disease in children, along with obesity and arterial hypertension, is a decrease in growth rates. The gold standard for diagnosing central hypercortisolism is MRI of the brain, however, the effectiveness of this method in children is only 50%. The main method of treatment is neurosurgical transnasal transsphenoidal removal of endosellar pituitary adenoma, which makes it possible to achieve remission in more than 65% of cases. This article describes a clinical case of Itsenko\u2013Cushing\u2019s disease in a 6.5-year-old child with obesity, arterial hypertension, atypically \u00abhigh\u00bb stature, average velocity and non-visualizable corticotropinoma. The article presents the stages of diagnostic search, the complexity of differential diagnosis and surgical treatment, the results of follow-up after the treatment and a brief review of the literature.<\/jats:p>","DOI":"10.14341\/probl13102","type":"journal-article","created":{"date-parts":[[2022,9,15]],"date-time":"2022-09-15T05:34:44Z","timestamp":1663220084000},"page":"92-101","update-policy":"https:\/\/doi.org\/10.14341\/crossmarkpolicy2018","source":"Crossref","is-referenced-by-count":2,"title":["A clinical Case and brief literature review of Icenko-Cushing\u2019s Disease in a pediatric patient with atypical onset of the disease"],"prefix":"10.14341","volume":"68","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8159-8769","authenticated-orcid":true,"given":"M. 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Problems of Endocrinology. 2020;66(6):39-49. (In Russ.). doi: https:\/\/doi.org\/10.14341\/probl12676","DOI":"10.14341\/probl12676"},{"key":"ref4","doi-asserted-by":"crossref","unstructured":"Nayak S, Dabadghao P, Dixit P, et al. Cushing\u2019s disease in children: a review. Neurol India. 2020;68(Supplement):S52-S65. doi: https:\/\/doi.org\/10.4103\/0028-3886.287677","DOI":"10.4103\/0028-3886.287677"},{"key":"ref5","doi-asserted-by":"crossref","unstructured":"Stratakis CA. An update on Cushing syndrome in pediatrics. Ann Endocrinol (Paris). 2018;79(3):125-131. doi: https:\/\/doi.org\/10.1016\/j.ando.2018.03.010","DOI":"10.1016\/j.ando.2018.03.010"},{"key":"ref6","doi-asserted-by":"crossref","unstructured":"Storr HL, Savage MO. Management of endocrine disease: Paediatric Cushing\u2019s disease. Eur J Endocrinol. 2015;173(1):R35-R45. doi: https:\/\/doi.org\/10.1530\/EJE-15-0013","DOI":"10.1530\/EJE-15-0013"},{"key":"ref7","doi-asserted-by":"crossref","unstructured":"Pivonello R, De Leo M, Cozzolino A, Colao A. The treatment of Cushing\u2019s disease. Endocr Rev. 2015;36(4):385-486. doi: https:\/\/doi.org\/10.1210\/er.2013-1048","DOI":"10.1210\/er.2013-1048"},{"key":"ref8","unstructured":"Solntseva AV. Giperkortitsizm u detei. Uchebno-metodicheskoe posobie. \u2014 Minsk: BGMU; 2020. P. 19. (In Russ.)."},{"key":"ref9","doi-asserted-by":"crossref","unstructured":"Storr HL, Chan LF, Grossman AB, Savage MO. Paediatric Cushing\u2019s syndrome: epidemiology, investigation and therapeutic advances. Trends Endocrinol Metab. 2007;18(4):167-174. doi: https:\/\/doi.org\/10.1016\/j.tem.2007.03.005","DOI":"10.1016\/j.tem.2007.03.005"},{"key":"ref10","doi-asserted-by":"crossref","unstructured":"Pappachan JM, Hariman C, Edavalath M, et al. Cushing\u2019s syndrome: a practical approach to diagnosis and differential diagnoses. J Clin Pathol. 2017;70(4):350-359. doi: https:\/\/doi.org\/10.1136\/jclinpath-2016-203933","DOI":"10.1136\/jclinpath-2016-203933"},{"key":"ref11","unstructured":"Dedov II, Peterkova VA. Rukovodstvo po detskoi endokrinologii. Moscow: UNIVERSUM PABLIShING; 2006. P. 117-131. (In Russ.)."},{"key":"ref12","doi-asserted-by":"crossref","unstructured":"Melnichenko GA, Dedov II, Belaya ZE, et al. Cushing\u2019s disease: the clinical features, diagnostics, differential diagnostics, and methods of treatment. Problems of Endocrinology. 2015;61(2):55-77. (In Russ.). doi: https:\/\/doi.org\/10.14341\/probl201561255-77","DOI":"10.14341\/probl201561255-77"},{"key":"ref13","doi-asserted-by":"crossref","unstructured":"Ferrigno R, Hasenmajer V, Caiulo S, et al. Paediatric Cushing\u2019s disease: Epidemiology, pathogenesis, clinical management and outcome. Rev Endocr Metab Disord. 2021;22(4):817-835. doi: https:\/\/doi.org\/10.1007\/s11154-021-09626-4","DOI":"10.1007\/s11154-021-09626-4"},{"key":"ref14","doi-asserted-by":"crossref","unstructured":"Sitkin II, Malygina AA, Belaya ZE, et al. Inferior petrosal sinus sampling in differential diagnosis of ACTH-dependent hypercortisolism. Endocrine Surgery. 2018;12(2):89-95. (In Russ.). doi: https:\/\/doi.org\/10.14341\/serg9752","DOI":"10.14341\/serg9752"},{"key":"ref15","doi-asserted-by":"crossref","unstructured":"Juul KV, Bichet DG, N\u00f8rgaard JP. Desmopressin duration of antidiuretic action in patients with central diabetes insipidus. Endocrine. 2011;40(1):67-74. doi: https:\/\/doi.org\/10.1007\/s12020-011-9492-z","DOI":"10.1007\/s12020-011-9492-z"},{"key":"ref16","doi-asserted-by":"crossref","unstructured":"Juul KV, Erichsen L, Robertson GL. Temporal delays and individual variation in antidiuretic response to desmopressin. Am J Physiol Renal Physiol. 2013;304(3):F268-278. doi: https:\/\/doi.org\/10.1152\/ajprenal.00502.2012","DOI":"10.1152\/ajprenal.00502.2012"},{"key":"ref17","doi-asserted-by":"crossref","unstructured":"Allolio B, Gunther RW, Benker G, et al. A multihormonal response to corticotrophin-releasing hormone in inferior petrosal sinus blood of patients with Cushing\u2019s disease. J Clin Endocrinol Metabol. 1990;71(5):1195-1201. doi: https:\/\/doi.org\/10.1210\/jcem-71-5-1195","DOI":"10.1210\/jcem-71-5-1195"},{"key":"ref18","doi-asserted-by":"crossref","unstructured":"McNally PG, Bolia A, Absalom SR, et al. Preliminary observations using endocrine markers of pituitary venous dilution during bilateral simultaneous inferior petrosal sinus catheterization in Cushing\u2019s syndrome: is combined CRF and TRH stimulation of value? Clin Endocrinol (Oxford). 1993;39(6):681-686. doi: https:\/\/doi.org\/10.1111\/j.1365-2265.1993.tb02427.x","DOI":"10.1111\/j.1365-2265.1993.tb02427.x"},{"key":"ref19","doi-asserted-by":"crossref","unstructured":"Findling JW, Kehoe ME, Raff H. Identifi cation of patients with Cushing\u2019s disease with negative pituitary adrenocorticotropin gradients during inferior petrosal sinus sampling: prolactin as an index of pituitary venous effluent. J Clin Endocrinol Metabol. 2004;89(12):6005-6009. doi: https:\/\/doi.org\/10.1210\/jc.2004-1378","DOI":"10.1210\/jc.2004-1378"},{"key":"ref20","doi-asserted-by":"crossref","unstructured":"Mulligan GB, Eray E, Faiman C, et al. Reduction of false negative results in inferior petrosal sinus sampling with simultaneous prolactin and corticotrophin measurement. Endocrinol Pract. 2011;17(1):33-40. doi: https:\/\/doi.org\/10.4158\/EP10067.OR","DOI":"10.4158\/EP10067.OR"},{"key":"ref21","doi-asserted-by":"crossref","unstructured":"Heaney AP, Melmed S. Molecular targets in pituitary tumors. Nat Rev Cancer. 2004;4(4):285-295. doi: https:\/\/doi.org\/10.1038\/nrc1320","DOI":"10.1038\/nrc1320"},{"key":"ref22","doi-asserted-by":"crossref","unstructured":"Sharma ST, Raff H, Nieman LK. Prolactin as a marker of successful catheterization during IPPS in patients with ACTH-dependent Cushing\u2019s syndrome. J Clin Endocrinol Metabol. 2011;96(12):3687-3694. doi: https:\/\/doi.org\/10.1210\/jc.2011-2149","DOI":"10.1210\/jc.2011-2149"},{"key":"ref23","doi-asserted-by":"crossref","unstructured":"Belaia ZE, Rozhinskaia LI, Mel\u2019nichenko GA, et al. The role of prolactin gradient and normalized ACTH\/prolactin ratio in the improvement of sensitivity and specificity of selective blood sampling from inferior petrosal sinuses for differential diagnostics of ACTH-dependent hypercorticism. Problems of Endocrinology. 2013;59(4):3-10. (In Russ.). doi: https:\/\/doi.org\/10.14341\/probl20135943-10","DOI":"10.14341\/probl20135943-10"},{"key":"ref24","doi-asserted-by":"crossref","unstructured":"Sitkin II, Malygina AA, Belaya ZE, et al. Inferior petrosal sinus sampling in differential diagnosis of ACTH-dependent hypercortisolism. Endocrine Surgery. 2018;12(2):89-95. (In Russ.). doi: https:\/\/doi.org\/10.14341\/serg9752","DOI":"10.14341\/serg9752"},{"key":"ref25","unstructured":"Strebkova NA, Udalova NV. Bolezn\u2019 Itsenko-Kushinga u detei i podrostkov. Farmateka. 2009;3:48-52. (In Russ.)."},{"key":"ref26","doi-asserted-by":"crossref","unstructured":"Chen S, Chen K, Lu L, et al. The effects of sampling lateralization on bilateral inferior petrosal sinus sampling and desmopressin stimulation test for pediatric Cushing\u2019s disease. 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