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Pediatr Endocrinol Rev. 2012 Nov;10(1):164-73.

Update on treatment strategies for optimization of final adult height in children with congenital adrenal hyperplasia.

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1
Division of Pediatric Endocrinology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN, USA. javed.asma@mayo.edu

Abstract

Congenital adrenal hyperplasia (CAH) is one of the most common autosomal recessive disorders, caused by deficiency of an enzyme involved in adrenal synthesis of cortisol. Due to lack of feedback from cortisol, an elevation in ACTH occurs, shifting precursors of steroidogenesis into androgen synthesis. Both the disorder itself due to excess androgens and replacement with glucocorticoids can compromise final adult height. Also, unpredictable progression to precocious puberty in some patients can further compromise height. The achievement of normal growth remains the ultimate goal of treatment. This review will first examine the evidence behind deficits in adult height in CAH and implicated factors behind such compromise. The primary goal of the review is to identify therapies to optimize height in CAH. This will include variations in 'standard' medical therapy and recent single and combination therapies with growth hormone, GnRH analogs, aromatase inhibitors and anti androgens to optimize final height in CAH.

PMID:
23469393
[Indexed for MEDLINE]
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