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Problemy endokrinologii · 2022

[A clinical Case and brief literature review of Icenko-Cushing's Disease in a pediatric patient with atypical onset of the disease].

СтатьяОбзорНазвание и аннотация: Оригинал на русском
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Tiulpakov M A, Bezlepkina O B, Nagaeva E V, Azizian V N, Lapshina A M

Аннотация

Itsenko-Cushing's disease is a rare, multisystem disease characterized by the presence of endogenous central hypercortisolism due to an ACTH-secreting brain tumor. The frequency of Itsenko-Cushing's disease in adulthood is 0.7-2.4 per 1 million population, and only 10% of all cases occur in childhood. The age of onset of the disease in children is on average 12.0-14.8 years. A typical manifestation of the disease in children, along with obesity and arterial hypertension, is a decrease in growth rates. The gold standard for diagnosing central hypercortisolism is MRI of the brain, however, the effectiveness of this method in children is only 50%. The main method of treatment is neurosurgical transnasal transsphenoidal removal of endosellar pituitary adenoma, which makes it possible to achieve remission in more than 65% of cases. This article describes a clinical case of Itsenko-Cushing's disease in a 6.5-year-old child with obesity, arterial hypertension, atypically «high» stature, average velocity and non-visualizable corticotropinoma. The article presents the stages of diagnostic search, the complexity of differential diagnosis and surgical treatment, the results of follow-up after the treatment and a brief review of the literature.

Предметные рубрики MeSH

ИП Урсолов Владлен Сергеевич

ИНН 500118482352 · ОГРНИП 314500117700049

Реквизиты и контакты