Epicardial fat thickness in children with classic congenital adrenal hyperplasia

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Classic congenital adrenal hyperplasia.

Congenital adrenal hyperplasia is attributed to inherited enzyme defects in the adrenal cortex. The classical form results in reduced production of cortisol and aldosterone, accompanied by an increase in production of adrenal cortical androgens. This causes virilisation in girls, adrenocortical failure and early puberty in both sexes. This article describes the genetics, clinical picture, diagn...

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Classic congenital adrenal hyperplasia and puberty.

Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders resulting from deficiency of one of the five enzymes required for synthesis of cortisol in the adrenal cortex. The most common form of the disease is classic 21-hydroxylase deficiency, which is characterized by decreased synthesis of glucocorticoids and often mineralocorticoids, adrenal hyperandrogenism and impaire...

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Classic congenital adrenal hyperplasia: A delayed presentation

Congenital adrenal hyperplasia (CAH) is a rare congenital disorder, which in cases of female genotype may result in virilization. Specific enzyme deficiencies in adrenocorticoid hormones biosynthetic pathway lead to excess androgen production causing virilization. Classic type presents early in infant life as salt losing or simple virilizing type, whereas non classic form presents late at puber...

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A Rationale for Mineralocorticoid Supplementation in Classic Congenital Adrenal Hyperplasia.

most common example of this phenomenon is Cushing syndrome due to high endogenous or exogenous cortisol exposure. Iatrogenic Cushing syndrome, characterized by inhibition of statural growth in children, excess weight gain, metabolic syndrome, and hypertension, may be observed in CAH patients receiving excessive glucocorticoid treatment [6] . This is why it is important to balance the need for s...

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Obesity among children and adolescents with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

OBJECTIVES Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is the most common inherited disorder of adrenal steroid biosynthesis. Patients with the classic form of CAH show androgen excess, with or without salt wasting. There are few studies reporting on higher rates of overweight and obesity among children with CAH. In addition to its role in the regulation of energy bala...

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ژورنال

عنوان ژورنال: Journal of Clinical Research in Pediatric Endocrinology

سال: 2018

ISSN: 1308-5727

DOI: 10.4274/jcrpe.0153