B.A. AZIZOV

Republican specialized scientific and practical medical Center of endocrinology of the Ministry of Health of the Republic of Uzbekistan, 56 Mirzo Ulugbek St., Tashkent, the Republic of Uzbekistan, 100125

Azizov B.A. — junior research associate of the laboratory of endocrine surgery, tel. (99871) 2622557, e-mail: [email protected]; [email protected]

The problem of multiple endocrine neoplasia is still under study and practitioners know little about it. In the described case the unclassical manifestation of the syndrome MEN-I during 10 years manifested by a combination of nodular hyperplasia of the parathyroid glands, functionally active benign pancreatic tumor and bilateral adrenal hyperplasia. Due to distal pancreatectomy concerning insulinoma the patient had a developed pancreatogenic diabetes with relative insulin deficiency.

Key words: Wermer’s syndrome, MEN-I, hyperparathyroid disorder, insulinoma, hyperinsulinism.

REFERENCES

1. Gilyazutdinov I.A., Khasanov R.Sh. Opukholi gormonal’no-zavisimykh i gormonprodutsiruyushchikh organov [Tumor hormone-dependent and hormone-producing organs], 2004. 464 p.
2. Kalinin A.P., Radbil’ O.S., Nurmanbetov D.N. Hormonally active tumors of the pancreas. Problemy endokrinologii, 1986, no. 2, pp. 40-46 (in Russ.).
3. Kalinin A.V. New in the diagnosis and treatment of neuroendocrine neoplasms of the gastrointestinal tract and pancreas, Rossiyskiy zhurnal gastroenterologii, gepatologii, koloproktologii, 1997, no. 5 (in Russ.).
4. Gitel’ E.P., Fadeev V.V. Diagnosis and treatment of endocrine tumors gastroenteropankreaticheskih. Problemy endokrinologii, 1996, vol. 42, no. 6, pp. 34-40 (in Russ.).
5. Gibril F., Schumann M., Pace A. et al. Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. Medicine, 2004, vol. 83, rr. 43-83.
6. Langer P., Cupisti K., Bartsch D.K. et al. Adrenal involvement in multiple endocrine neoplasia type 1. World J Surg., 2002, vol. 26, rr. 891-896.
7. Marini F. et al. Multiple endocrine neoplasia type 1. Orphanet. J.Rare. Dis., 2006, vol. 2, pp. 1-38.
8. Brandi M.L. et al. Multiple endocrine neoplasia type 1. Rev. Endocr. Metabolic. Disord., 2000, vol.1, pp. 275-282.
9. Krages W. et al. Clinical and molecular diagnosis of multiple endocrine neoplasia type 1. Langenbecks Arch. Surg., 2002, vol. 386, pp. 547-552.
10. Pinskiy S.B., Kalinin A.P., Beloborodov V.A. Redkie opukholi i zabolevaniya shchitovidnoy zhelezy [Rare tumors and thyroid disease]. Irkutsk, 1999. Pp. 86-105.
11. Gibril F., Schumann M., Pace A., Jensen R.T. 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, vol. 83 (1), rr. 43-83.
12. Agarwal S.K., Lee Burns A., Sukhodolets K.E., Kennedy P.A., Obungu V.H. et al. Molecular pathology of the MEN1 gene. Ann N Y Acad Sci., 2004, vol. 1014, rr. 189-198.