Mineralocorticoid Deficiency and Treatment in Congenital Adrenal Hyperplasia
Author(s) -
Raja Padidela,
PeterC Hindmarsh
Publication year - 2010
Publication title -
international journal of pediatric endocrinology/international journal of pediatric endocrinology
Language(s) - English
Resource type - Journals
eISSN - 1687-9856
pISSN - 1687-9848
DOI - 10.1186/1687-9856-2010-656925
Subject(s) - medicine , congenital adrenal hyperplasia , pediatrics , mineralocorticoid , intensive care medicine , aldosterone , endocrinology
Approximately 75%–80% of patients with Congenital Adrenal Hyperplasia (CAH) fail to synthesize sufficient mineralocorticoids to maintain salt and water balance. In most instances genotype can predict mineralocorticoid deficiency in CAH. Early recognition and replacement with 9-fludrocortisone and salt supplements will prevent development of potentially lethal salt losing crises. In infancy a relative state of aldosterone resistance exists and replacement dose of 9-fludrocortisone based on body surface area is higher during infancy compared to childhood and adults. Salt supplementation is generally not required after weaning is started. Regular monitoring of blood pressure and measurements of plasma electrolytes and renin are required to prevent complications of under or over dosage.
Open Access