ometendoОжирение и метаболизмObesity and metabolism2071-87132306-5524Endocrinology Research Centre10.14341/2071-8713-4878ometendo-4878Research ArticleСтатьиArticlesОсобенности метаболизма костной тканипри синдроме множественных эндокринныхнеоплазий 1 типаOsobennosti metabolizma kostnoy tkanipri sindrome mnozhestvennykh endokrinnykhneoplaziy 1 tipaRostomyanL Glilit.rostomyan@gmail.comMokryshevaN Genc@endocrincentr.ruTyul'pakovA Nant@endocrincentr.ruVorontsovA Vmrt@endocrincentr.ruKirdyankinaN Onataliya-obraz@yandex.ruRozhinskayaL Yarozh@endocrincentr.ru20091512200964№4 (2009)4246Copyright © Rostomyan L.G., Mokrysheva N.G., Tyul'pakov A.N., Vorontsov A.V., Kirdyankina N.O., Rozhinskaya L.Y., 20092009Rostomyan L.G., Mokrysheva N.G., Tyul'pakov A.N., Vorontsov A.V., Kirdyankina N.O., Rozhinskaya L.Y.Rostomyan L.G., Mokrysheva N.G., Tyul'pakov A.N., Vorontsov A.V., Kirdyankina N.O., Rozhinskaya L.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.omet-endojournals.ru/jour/article/view/4878

Важным аспектом клинической картины синдрома множественных эндокринных неоплазий (МЭН) 1 типа является состояние костного метаболизма и изменение минеральной плотности кости (МПК). Описание клинического случая: представляем клинический случай первичного гиперпаратиреоза (ПГПТ) у пациента с семейной формой синдрома МЭН 1 типа тяжелого течения, дебютировавшего в молодом возрасте и сопровождающегося выраженными изменениями костной ткани. В 16 лет поставлен диагноз «болезнь Иценко-Кушинга». Выявлен выраженный остеопороз (-12% от возрастной нормы) на фоне гиперкортицизма и дефицита половых гормонов . Наблюдалась положительная динамика МПК (+7% по Z-критерию) при ремиссии гиперкортицизма после двух курсов протонотерапии и эффективной заместительной гормональной терапии гипогонадизма. В 19 лет выявлен ПГПТ с выраженным снижением МПК в трех отделах. Через год после эффективного хирургического лечения и назначения антиостеопоротической терапии наблюдалась резко положительная динамика МПК. Таким образом, приведенный клинический случай демонстрирует сочетанное влияние различных факторов на костный метаболизм при синдроме МЭН 1 типа и комплексный подход к их коррекции.

Bone metabolism and changes in bone mineral density (BMD) are very important in patients with multiple endocrine neoplasia (MEN) type 1 syndrome. Case report: we present a clinical case of primary hyperparathyroidism (PHPT) in a patient with a familal MEN 1 syndrome and severe PHPT debuted at a young age w. Pituitary Cushing syndrome was diagnosed in 16 years old patient. He had marked osteoporosis due to hypercorticism and deficiency of sex hormones (-12% from the age norm). There was significant positive dynamics in BMD (+7% by Z-score) after remission of hypercorticism by two courses of radiation treatment and effective hormone replacement therapy of hypogonadism. PHPT with marked decrease in BMD at three sites was revealed at age of 19 years. Obvious increase in BMD was observed during a year after effective surgical treatment and antiosteoporotic therapy. In conclusion, this clinical case demonstrates the combined influence of various factors on bone metabolism in patients with MEN type 1 syndrome, which requires integrated approach to successful therapy.

МЭН 1первичный гиперпарати- реозМПКгиперкортицизмMEN 1primary hyperparathyroidismBMDhypercortisolismboneReferencesДедов И.И., Мельниченко Г.А., Фадеев В.В. Эндокринология. - М., 2000.Дедов И.И., Мельниченко Г.А., Фадеев В.В. Эндокринология. - М., 2000.Лоренс Риггз Б., Л. Джозеф Мелтон III. Остеопороз. - СПб.: ЗАО «Издательство БИНОМ», 2000.Лоренс Риггз Б., Л. Джозеф Мелтон III. Остеопороз. - СПб.: ЗАО «Издательство БИНОМ», 2000.Тинсли Р. Харрисон / Внутренние болезни // Практика - М., 2002.Тинсли Р. Харрисон / Внутренние болезни // Практика - М., 2002.Abdelhadi M., Nordenstrum J. / Bone mineral recovery after parathyroidectomy in patients with primary and renal hyperparathyroidism // J. Clin. Endocrinol. Metab. - 1998. - №83. - Р. 3845.Abdelhadi M., Nordenstrum J. / Bone mineral recovery after parathyroidectomy in patients with primary and renal hyperparathyroidism // J. Clin. Endocrinol. Metab. - 1998. - №83. - Р. 3845.Bilezikian J.P., Brandi M.L., Rubin M., Silverberg S.J. Primary hyperparathyroidism: new concepts in clinical, densitometric and biochemical features // Journal of Internal Medicine. - 2005. - №257. - Р. 6-17.Bilezikian J.P., Brandi M.L., Rubin M., Silverberg S.J. Primary hyperparathyroidism: new concepts in clinical, densitometric and biochemical features // Journal of Internal Medicine. - 2005. - №257. - Р. 6-17.Bilezikian J.P., Aliya A. Khan, John T. Potts, Jr. on behalf of the Third International Workshop on the Management of Asymptomatic Primary Hyperthyroidism. Guidelines for the Management of Asymptomatic Primary Hyperparathyroidism: Summary Statement from the Third International Workshop // J. Clin. Endocrinol. Metab. - 2009. - №94. - Р. 335-9.Bilezikian J.P., Aliya A. Khan, John T. Potts, Jr. on behalf of the Third International Workshop on the Management of Asymptomatic Primary Hyperthyroidism. Guidelines for the Management of Asymptomatic Primary Hyperparathyroidism: Summary Statement from the Third International Workshop // J. Clin. Endocrinol. Metab. - 2009. - №94. - Р. 335-9.Brandi M.L., Gagel R.F., Angeli A., et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2 // J. Clin. Endocrinol. Metab. - 2001. - №86. - Р. 5658-71.Brandi M.L., Gagel R.F., Angeli A., et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2 // J. Clin. Endocrinol. Metab. - 2001. - №86. - Р. 5658-71.Burgess J.R., David R., Greenaway T.M., et al. Osteoporosis in multiple endocrine neoplasia type 1: severity, clinical significance, relationship to primary hyperparathyroidism, and response to parathyroidectomy // Arch. Surg. - 1999. - №134. - Р. 1119-23.Burgess J.R., David R., Greenaway T.M., et al. Osteoporosis in multiple endocrine neoplasia type 1: severity, clinical significance, relationship to primary hyperparathyroidism, and response to parathyroidectomy // Arch. Surg. - 1999. - №134. - Р. 1119-23.Сое F.L., Favrus M.J. (eds). Disorders of Bone and Mineral Metabolism. New York: Raven. - 1992.Сое F.L., Favrus M.J. (eds). Disorders of Bone and Mineral Metabolism. New York: Raven. - 1992.Eller-Vainicher C., Chiodini I., Battista, et al. Sporadic and MEN1-related primary hyperparathyroidism: differences in clinical expression and severity // J. Bone. Miner. Res. - 2009. - V. 24, №8. - Р. 1404-10.Eller-Vainicher C., Chiodini I., Battista, et al. Sporadic and MEN1-related primary hyperparathyroidism: differences in clinical expression and severity // J. Bone. Miner. Res. - 2009. - V. 24, №8. - Р. 1404-10.Finkelstein J.S., Klibanski A., Neer R.M., et al. Increases in bone density during treatment of men with idiopathic hypogonadotropic hypogonadism // J. Clin. Endocrinol. Metab. - 1989. - №69. - Р. 776-83.Finkelstein J.S., Klibanski A., Neer R.M., et al. Increases in bone density during treatment of men with idiopathic hypogonadotropic hypogonadism // J. Clin. Endocrinol. Metab. - 1989. - №69. - Р. 776-83.Gilsanz V. Accumulation of bone mass during childhood and adolescence. In: Orwoll ES, ed. Osteoporosis in men. San Diego, Calif.: Academic. - 1999. - Р. 65-85.Gilsanz V. Accumulation of bone mass during childhood and adolescence. In: Orwoll ES, ed. Osteoporosis in men. San Diego, Calif.: Academic. - 1999. - Р. 65-85.Grey A.B., Evans M.C., Stapleton J.P., et al. Body weight and bone mineral density in postmenopausal women with primary hyperparathyroidism // Ann. Intern. Med. - 1994. - №121. - Р. 745.Grey A.B., Evans M.C., Stapleton J.P., et al. Body weight and bone mineral density in postmenopausal women with primary hyperparathyroidism // Ann. Intern. Med. - 1994. - №121. - Р. 745.Ikeo Y., Sakurai A., Suzuki R., et al. Proliferation-associated expression of the MEN1 gene as revealed by in situ hybridization: possible role of the menin as a negative regulator of cell proliferation under DNA damage // Lab. Invest. - 2000. - №80. - Р. 797-804.Ikeo Y., Sakurai A., Suzuki R., et al. Proliferation-associated expression of the MEN1 gene as revealed by in situ hybridization: possible role of the menin as a negative regulator of cell proliferation under DNA damage // Lab. Invest. - 2000. - №80. - Р. 797-804.Khosla S., Melton L..J., Wermers R.A., et al. Primary hyperparathyroidism and the risk of fracture: a population-based study // J. Bone Miner. Res. - 1999. - №14. - Р. 1700.Khosla S., Melton L..J., Wermers R.A., et al. Primary hyperparathyroidism and the risk of fracture: a population-based study // J. Bone Miner. Res. - 1999. - №14. - Р. 1700.Laura A.L., Suzanne E.S., Jeffrey E.L., et al. Surgical Treatment of Hyperparathyroidism in Patients With Multiple Endocrine Neoplasia Type 1 // Arch. Surg. - 2005. - №140. - Р. 374-82.Laura A.L., Suzanne E.S., Jeffrey E.L., et al. Surgical Treatment of Hyperparathyroidism in Patients With Multiple Endocrine Neoplasia Type 1 // Arch. Surg. - 2005. - №140. - Р. 374-82.LourenЌo J., Toledo R.A., Mackowiak II, et al. Multiple endocrine neoplasia type 1 in Brazil: MEN1 founding mutation, clinical features, and bone mineral density profile // European Journal of Endocrinology. - 2008. - V. 159, №3. - Р. 259-74.LourenЌo J., Toledo R.A., Mackowiak II, et al. Multiple endocrine neoplasia type 1 in Brazil: MEN1 founding mutation, clinical features, and bone mineral density profile // European Journal of Endocrinology. - 2008. - V. 159, №3. - Р. 259-74.Peck W.A. The effects of glucocorticoids on bone cells metabolism and function // Adv. Exp. Med. Biol. - 1984. - №171. - Р. 111-9.Peck W.A. The effects of glucocorticoids on bone cells metabolism and function // Adv. Exp. Med. Biol. - 1984. - №171. - Р. 111-9.Sowa H., Kaji H., Canaff L., et al. Inactivation of menin, the product of the multiple endocrine neoplasia type 1 gene, inhibits the commitment of multipotential mesenchymal stem cells into the osteoblast lineage // Journal of Biological Chemistry. - 2003. - №278. - Р. 21058-69.Sowa H., Kaji H., Canaff L., et al. Inactivation of menin, the product of the multiple endocrine neoplasia type 1 gene, inhibits the commitment of multipotential mesenchymal stem cells into the osteoblast lineage // Journal of Biological Chemistry. - 2003. - №278. - Р. 21058-69.Van Heerden J.A., Grant C.S. Parathyroid. In:. S.I. Schwaetz, G.T. Shires, F.C. Spencer, Daly J.M., A.C. Galloway, J.E. Fisher (Eds.) Principles of surgery // McGrew-Hill, NY. - 1998. - Р. 1694.Van Heerden J.A., Grant C.S. Parathyroid. In:. S.I. Schwaetz, G.T. Shires, F.C. Spencer, Daly J.M., A.C. Galloway, J.E. Fisher (Eds.) Principles of surgery // McGrew-Hill, NY. - 1998. - Р. 1694.

The authors declare that there are no conflicts of interest present.