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Osobennosti metabolizma kostnoy tkanipri sindrome mnozhestvennykh endokrinnykhneoplaziy 1 tipa
Author(s) -
Liliya Rostomyan,
Н. Г. Мокрышева,
A. N. Tyulpakov,
А В Воронцов,
Nataliya Kirdyankina,
Rozhinskaya L Ya
Publication year - 2009
Publication title -
ožirenie i metabolizm
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.154
H-Index - 5
eISSN - 2306-5524
pISSN - 2071-8713
DOI - 10.14341/2071-8713-4878
Subject(s) - medicine , bone mineral , osteoporosis , primary hyperparathyroidism , bone remodeling , endocrinology , hormone , hormone replacement therapy (female to male) , hyperparathyroidism , endocrine system , pediatrics , testosterone (patch)
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 MEN1 syndrome and severe PHPT debuted at a young age w. Pituitary Cushing syndrome was diagnosed in 16 years old patient. He had markedosteoporosis due to hypercorticism and deficiency of sex hormones (-12% from the age norm). There was significant positive dynamicsin BMD (+7% by Z-score) after remission of hypercorticism by two courses of radiation treatment and effective hormone replacement therapyof hypogonadism. PHPT with marked decrease in BMD at three sites was revealed at age of 19 years. Obvious increase in BMD was observedduring a year after effective surgical treatment and antiosteoporotic therapy. In conclusion, this clinical case demonstrates the combinedinfluence of various factors on bone metabolism in patients with MEN type 1 syndrome, which requires integrated approach to successfultherapy.

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