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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...

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Autores principales: Тюльпаков, М. А., Безлепкина, О. Б., Нагаева, Е. В., Азизян, В. Н., Лапшина, А. М.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Endocrinology Research Centre 2022
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
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author Тюльпаков, М. А.
Безлепкина, О. Б.
Нагаева, Е. В.
Азизян, В. Н.
Лапшина, А. М.
author_facet Тюльпаков, М. А.
Безлепкина, О. Б.
Нагаева, Е. В.
Азизян, В. Н.
Лапшина, А. М.
author_sort Тюльпаков, М. А.
collection PubMed
description 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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spelling pubmed-97624342023-01-06 Болезнь Иценко-Кушинга у ребенка с нетипичным дебютом болезни. Клинический случай с кратким обзором литературы Тюльпаков, М. А. Безлепкина, О. Б. Нагаева, Е. В. Азизян, В. Н. Лапшина, А. М. Probl Endokrinol (Mosk) Research Article 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. Endocrinology Research Centre 2022-05-14 /pmc/articles/PMC9762434/ /pubmed/36104970 http://dx.doi.org/10.14341/probl13102 Text en Copyright © Endocrinology Research Centre, 2022 https://creativecommons.org/licenses/by/4.0/This work is licensed under a Creative Commons Attribution 4.0 License.
spellingShingle Research Article
Тюльпаков, М. А.
Безлепкина, О. Б.
Нагаева, Е. В.
Азизян, В. Н.
Лапшина, А. М.
Болезнь Иценко-Кушинга у ребенка с нетипичным дебютом болезни. Клинический случай с кратким обзором литературы
title Болезнь Иценко-Кушинга у ребенка с нетипичным дебютом болезни. Клинический случай с кратким обзором литературы
title_full Болезнь Иценко-Кушинга у ребенка с нетипичным дебютом болезни. Клинический случай с кратким обзором литературы
title_fullStr Болезнь Иценко-Кушинга у ребенка с нетипичным дебютом болезни. Клинический случай с кратким обзором литературы
title_full_unstemmed Болезнь Иценко-Кушинга у ребенка с нетипичным дебютом болезни. Клинический случай с кратким обзором литературы
title_short Болезнь Иценко-Кушинга у ребенка с нетипичным дебютом болезни. Клинический случай с кратким обзором литературы
title_sort болезнь иценко-кушинга у ребенка с нетипичным дебютом болезни. клинический случай с кратким обзором литературы
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9762434/
https://www.ncbi.nlm.nih.gov/pubmed/36104970
http://dx.doi.org/10.14341/probl13102
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