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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http://dx.doi.org/10.14341/probl13102 | DOI Listing |
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9762434 | PMC |
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.
View Article and Find Full Text PDFZh Vopr Neirokhir Im N N Burdenko
April 1998
Fifty three consecutive patients with Cushing's disease were operated on in the Neurosurgical Institute from 1987 to 1995. In all cases the diagnosis was based on clinical and laboratory data, as well as radiographic images. Ten patients with false-negative CT scans had MRI confirming the presence of microadenomas less than 5 mm in size.
View Article and Find Full Text PDFThe authors studied the informative value of various diagnostic methods: ultrasonic examination (USE), computed tomography (CT), magneto-resonance tomography (MRT), phlebography with separate collection of blood from the inferior vena cava and its hormonal investigation in Itsenko-Cushing's disease (52 cases). All patient underwent operation, and because of this the changes detected in the adrenals by various diagnostic methods of examination were compared with the morphological findings. Three patients were subjected to bilateral adrenalectomy.
View Article and Find Full Text PDFProbl Endokrinol (Mosk)
May 1994
Nineteen patients with Itsenko-Cushing's disease aged 13 to 18 were examined during an active stage of the disease and during a remission after proton exposure of the hypophysis. Nine normal subjects were controls. Adenohypophyseal tropic hormones and hydrocortisone were radioimmunoassayed before and after administration of nakom, a dopaminergic agent.
View Article and Find Full Text PDFThe paper summarizes the results od several studies of the daily rhythms of steroid hormones in patients with ACTH-dependent Itsenko-Cushing's disease (CD) and congenital adrenal hyperplasia (late-onset forms) (CAH). Normal daily rhythms of adrenal C21- and C19-steroids and ACTH were observed in 23.5% of CD patients.
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