References

problendo

Проблемы Эндокринологии

Problems of Endocrinology

0375-96602308-1430

Endocrinology Research Centre

10.14341/probl201258322-30

problendo-4685

Research Article

Статьи

Articles

Карни-комплекс - синдром множественных эндокринных неоплазий

Carney complex - multiple endocrine neoplasia syndrome

Orlova

E M

elizaveta.orlova@mail.ru

Kareva

M A

2012

15062012

583

ТОМ 58, №3 (2012)

2230

2012

Orlova E.M., Kareva M.A.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://www.probl-endojournals.ru/jour/article/view/4685

Карни-комплекс - редкий наследственный синдром с аутосомно-доминантным типом наследования, характеризующийся множественными неоплазиями, в том числе органов эндокринной системы. Типичными проявлениями этого синдрома являются микронодулярная пигментная дисплазия надпочечников, лентигиноз, миксомы сердца и кожи, крупноклеточные сертолиомы, а также ряд других неоплазий. На сегодняшний день в мире описано всего несколько сотен таких пациентов. Представлен обзор современных сведений о Карни-комплексе.

Carney complex is a rare hereditary syndrome characterized by an autosomal-dominant mode of inheritance and associated with multiple neoplasias affecting endocrine organs. The typical manifestations of this syndrome include pigmented micronodular adrenal dysplasia, lentiginosis, heart and skin myxomas, giant cell sertoliomas, and some other neoplasias. To date, a few hundred patients with this pathology have been described worldwide. A review of the available data about Carney complex is presented.

Карни-комплекспигментная нодулярная надпочечниковая дисплазияген PRKAR1Aлентигинозкрупноклеточная сертолиома

Carney complexpigmented nodular adrenal dysplasiaPRKAR1A genelentiginosisgiant cell sertoliomas

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