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Болезнь Иценко-Кушинга у ребенка с нетипичным дебютом болезни. Клинический случай с кратким обзором литературы
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 childhoo...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Endocrinology Research Centre
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9762434/ https://www.ncbi.nlm.nih.gov/pubmed/36104970 http://dx.doi.org/10.14341/probl13102 |
Sumario: | 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. |
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