Factors determining final height in congenital adrenal hyperplasia.
Congenital adrenal hyperplasia (CAH) is a family of adrenal disorders whereby cortisol biosynthesis is impaired or abolished. In most cases, CAH is caused by a deficiency of the enzyme 21-hydroxylase causing a massive build up of adrenal precursors. In addition to prenatal virilisation of genetic females and postnatal virilisation of both males and females, short adult stature is characteristic of the disorder. The inadequate final height in CAH patients is often attributed to overtreatment with glucocorticoid replacement and poor control of adrenal androgen levels. In a recent study, the use of growth hormone alone and in combination with gonadotropin releasing hormone analogue in children with CAH and poor predicted final height was found to decrease height deficit after one and two years of treatment.
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Department of Pediatrics, New York-Presbyterian Hospital and the Weill Medical College of Cornell University, NY 10021, USA. minew@mail.med.cornell.edu
D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology*; D001827:Body Height / Q000187:drug effects / Q000235:genetics / Q000502:physiology*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male