Monitoring treatment in congenital adrenal hyperplasia.

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Monitoring treatment in congenital adrenal hyperplasia.

We report results of monitoring treatment in 41 patients with congenital adrenal hyperplasia controlled over 0.3-13.1 years using standard auxological techniques alone. Doses of glucocorticoid (15-25 mg/m2/day) and mineralocorticoid (0.15 mg/m2/day) replacement were determined initially using biochemical indices and thereafter adjusted according to surface area. Monitoring was solely directed a...

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Monitoring of therapy in congenital adrenal hyperplasia.

BACKGROUND Congenital adrenal hyperplasia is a group of disorders caused by defects in the adrenal steroidogenic pathways. In its most common form, 21-hydroxylase deficiency, patients develop varying degrees of glucocorticoid and mineralocorticoid deficiency as well as androgen excess. Therapy is guided by monitoring clinical parameters as well as adrenal hormone and metabolite concentrations. ...

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Ten patients with congenital adrenal hyperplasia (three males, seven females; aged 12-29 years) had their usual glucocorticoid treatment changed to dexamethasone in three crossover dosage regimens. A starting dose of 5 micrograms/kg/day is suggested but as no one dose regimen resulted in adequate control the timing of the dose must be decided for each patient.

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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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ژورنال

عنوان ژورنال: Archives of Disease in Childhood

سال: 1989

ISSN: 0003-9888,1468-2044

DOI: 10.1136/adc.64.9.1235