ometendoОжирение и метаболизмObesity and metabolism2071-87132306-5524Endocrinology Research Centre10.14341/2071-8713-5129ometendo-5129Research ArticleСтатьиArticlesКлинический случай: синдром множественной эндокринной неоплазии типа 1 (МЭН 1)Clinical case: multiple endocrine neoplasia type 1 (MEN 1)LipatenkovaA Klipatenkova@gmail.comDzeranovaL Kdzeranovalk@yandex.ruPigarovaE Akpigarova@gmail.comRozhinskayaL Yarozh@endocrincentr.ruKochatkovA Vkochatkov@mail.ru20121512201294№4 (2012)4447Copyright © Lipatenkova A.K., Dzeranova L.K., Pigarova E.A., Rozhinskaya L.Y., Kochatkov A.V., 20122012Lipatenkova A.K., Dzeranova L.K., Pigarova E.A., Rozhinskaya L.Y., Kochatkov A.V.Lipatenkova A.K., Dzeranova L.K., Pigarova E.A., Rozhinskaya L.Y., Kochatkov A.V.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/5129

Синдром множественной эндокринной неоплазии типа 1 (МЭН 1 типа, синдром Вермера) – группа гетерогенных наследственных заболеваний, в основе патогенеза которого лежит гиперплазия или опухолевая трансформация нескольких эндокринных желез. МЭН 1 отличается вариабельностью сочетания различных клинических проявлений, в литературе описано более 20 различных комбинаций эндокринных и метаболических нарушений в рамках синдрома. Мы представляем описание клинического случая синдрома Вермера, особенностью которого является неклассическая манифестация заболевания с пролактин-секретирующей макроаденомы гипофиза, последовательное присоединение гиперпаратиреоза и гипогликемического синдрома вследствие гиперпроинсулинемии.

Multiple endocrine neoplasia syndrome type 1 (MEN1, Wermer syndrome) – group o а heterogeneous inherited deseases, caused by hyperlasia or neoplasia of several endocrine glands. The phenotype of MEN1 is broad, and over 20 different combinations of endocrine and non-endocrine metabolic manifestations have been described. This case demonstrates multiple formations of endocrine organs, starting non-classical with macroprolactonoma resistant to dopamine agonists therapy, other endocrine disorders developed gradually eventually: hyperparathyreoidism and hypoglycemia caused by pancreas lesions, produced proinsulin in high levels.

синдром ВермераМЭН 1пролактиномапролактингиперпаратиреозинсулиномапроинсулинWermer syndromeMEN 1prolactinomaprolactinhyperparathyroidisminsulinomaproinsulinReferencesGiraud S, Zhang CX, Serova-Sinilnikova O, Wautot V, Salandre J, Buisson N, Waterlot C, Bauters C, Porchet N, Aubert JP, Emy P, Cadiot G, Delemer B, Chabre O, Niccoli P, Leprat F, Duron F, Emperauger B, Cougard P, Goudet P, Sarfati E, Riou JP, Guichard S, Rodier M, Meyrier A, Caron P, Vantyghem MC, Assayag M, Peix JL, Pugeat M, Rohmer V, Vallotton M, Lenoir G, Gaudray P, Proye C, Conte-Devolx B, Chanson P, Shugart YY, Goldgar D, Murat A, Calender A. Germ-line mutation analysis in patients with multiple endocrine neoplasia type 1 and related disorders. Am J HumGenet 1998;63:455–467.Giraud S, Zhang CX, Serova-Sinilnikova O, Wautot V, Salandre J, Buisson N, Waterlot C, Bauters C, Porchet N, Aubert JP, Emy P, Cadiot G, Delemer B, Chabre O, Niccoli P, Leprat F, Duron F, Emperauger B, Cougard P, Goudet P, Sarfati E, Riou JP, Guichard S, Rodier M, Meyrier A, Caron P, Vantyghem MC, Assayag M, Peix JL, Pugeat M, Rohmer V, Vallotton M, Lenoir G, Gaudray P, Proye C, Conte-Devolx B, Chanson P, Shugart YY, Goldgar D, Murat A, Calender A. Germ-line mutation analysis in patients with multiple endocrine neoplasia type 1 and related disorders. Am J HumGenet 1998;63:455–467.White ML, Doherty GM. Multiple endocrine neoplasia. Surgical Oncology Clinics of North America 2008;17:439–459.White ML, Doherty GM. Multiple endocrine neoplasia. Surgical Oncology Clinics of North America 2008;17:439–459.Skarulis MC. Clinical expression of MEN1 at NIH. AnnInternMed. 1998;129:484–494.Skarulis MC. Clinical expression of MEN1 at NIH. AnnInternMed. 1998;129:484–494.Gibril F, Schumann M, Pace A, Jensen RT. Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. Medicine (Baltimore) 2004;83(1):43–83.Gibril F, Schumann M, Pace A, Jensen RT. Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. Medicine (Baltimore) 2004;83(1):43–83.Agarwal SK, Lee Burns A, Sukhodolets KE, Kennedy PA, Obungu VH, Hickman AB, Mullendore ME, Whitten I, Skarulis MC, Simonds WF, Mateo C, Crabtree JS, Scacheri PC, Ji Y, Novotny EA, Garrett-Beal L, Ward JM, Libutti SK, Richard Alexander H, Cerrato A, Parisi MJ. Santa Anna-A S, Oliver B, Chandrasekharappa SC, Collins FS, Spiegel AM, Marx SJ. Molecular pathology of the MEN1 gene. Ann N Y Acad Sci 2004;1014:189–198.Agarwal SK, Lee Burns A, Sukhodolets KE, Kennedy PA, Obungu VH, Hickman AB, Mullendore ME, Whitten I, Skarulis MC, Simonds WF, Mateo C, Crabtree JS, Scacheri PC, Ji Y, Novotny EA, Garrett-Beal L, Ward JM, Libutti SK, Richard Alexander H, Cerrato A, Parisi MJ. Santa Anna-A S, Oliver B, Chandrasekharappa SC, Collins FS, Spiegel AM, Marx SJ. Molecular pathology of the MEN1 gene. Ann N Y Acad Sci 2004;1014:189–198.Marx SJ. Molecular genetics of multiple endocrine neoplasia types 1 and 2. NatRevCancer 2005;5(5):367–375.Marx SJ. Molecular genetics of multiple endocrine neoplasia types 1 and 2. NatRevCancer 2005;5(5):367–375.Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, Conte-Devolx, Falchetti BA, Gheri RG, Libroia A, Lips CJ, Lombardi G, Mannelli M, Pacini F, Ponder BA, Raue F, Skogseid B, Tamburrano G, Thakker RV, Thompson NW, Tomassetti P, Tonelli F,Wells SA, Jr., Marx SJ. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin. EndocrinolMetab 2001;86:5658–5671.Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, Conte-Devolx, Falchetti BA, Gheri RG, Libroia A, Lips CJ, Lombardi G, Mannelli M, Pacini F, Ponder BA, Raue F, Skogseid B, Tamburrano G, Thakker RV, Thompson NW, Tomassetti P, Tonelli F,Wells SA, Jr., Marx SJ. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin. EndocrinolMetab 2001;86:5658–5671.Madeira I, Terris B, Voss M. et al. Prognostic factors in patients with endocrine tumors of the duodenopancreaticarea. Gut 1998;43:422–427.Madeira I, Terris B, Voss M. et al. Prognostic factors in patients with endocrine tumors of the duodenopancreaticarea. Gut 1998;43:422–427.

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