Aspirantskiy Vestnik PovolzhiyaAspirantskiy Vestnik Povolzhiya2072-23542410-3764Samara State Medical University2451310.17816/2072-2354.2015.0.5-6.176-179Research ArticleCARNEY COMPLEX, ASSOCIATED WITH ENDOGENOUS HYPERCORTISOLISMPRIVALOVY A-KULIKOVL K-TAEVSKAYAA K-SOBOTOVICHV F-ALEXINAE K-VISOKIKHS M-15092015155-617617911032020Copyright © 2015, PRIVALOV Y.A., KULIKOV L.K., TAEVSKAYA A.K., SOBOTOVICH V.F., ALEXINA E.K., VISOKIKH S.M.2015We present a clinical case of endogenous hypercortisolism. After examination, 18-year-old patient was diagnosed with Cushings syndrome, presumably caused by corticosteroma of left adrenal gland. Left laparoscopic adrenalectomy was performed. Pathologohystological conclusion was adrenal pigmented nodular dysplasia. In postoperative period hypercortisolism symptoms regressed. During the 2-year lasting observation of patient no relapse was found. In retrospect, the patient revealed symptoms of a rare hereditary disease - Carney complex. The interest of submitted observation lies in its exceptional rarity. Unilateral adrenalectomy was effective, but the probability of recurrence of Cushing’s syndrome and other manifestations of Carney complex still exists.Карни-комплекспигментная нодулярная дисплазия надпочечникасиндром Иценко-Кушингагиперкортицизмистинный лентигинозаутосомно-доминантный тип наследования[Орлова Е.М., Карева М.А. Карни-комплекс - синдром множественных эндокринных неоплазий // Проблемы эндокринологии. 2012. № 3. 22-30.][Орлова Е.М., Карева М.А., Захарова Е.Ю., Полякова Г.А., Поддубный И.В., Толстов К.Н., Меликян М.А., Калинченко Н.Ю., Удалова Н.В., Петеркова В.А.Три случая Карни-комплекса у детей: клинические и молекулярно-генетические особенности Карни-комплекса у детей (первое описание в России) // Проблемы эндокринологии. 2012. № 5. 50-56.][Carney J.A., Gordon H., Carpenter P.C., Shenoy B.V., Go V.L. The complex of myxomas, spotty pigmentation, and endocrine over activity// Medicine (Baltimore). 1985. 64: 4: 270-283.][Carney J.A., Hruska L.S., Beauchamp G.D., Gordon H. Dominant inheritance of the complex of myxomas, spotty pigmentation and endocrine over activity // Mayo Clin.Proc. 1986. 61: 165-172.][Kirschner L.S., Carney J.A., Pack S.D. et al. Mutations of the gene encoding the protein kinase A type I-alpha regulatory subunit in patients with the Carney complex //Nat. Genet. 2000. 26: 89-92.][Sarlis N.J., Chrousos G.P., Doppman J.L., Carney J.A., Stratakis C.A. Primary pigmented nodular adrenocortical disease: reevaluation of a patient with Сarney complex 27 years after unilateral adrenalectomy //J. Clin.Endocrinol.Metab. 1997. 82: 4: 1274-1278.][StratakisС.A., Kirshner L.S., Carney J.A. Clinical and molecular features of the Carney complex, diagnostic criteria and recommendations for patient evaluation // J. Clin. Endocrinol. Metab. 2001; 86: 9: 4041-4046.]