Original ArticlesHeight outcome in congenital adrenal hyperplasia caused by 21-hydroxylase deficiency: A meta-analysis☆,☆☆
Section snippets
Retrospective Review
After institutional review board approval was obtained, charts from patients with 21-hydroxylase deficiency aged 5 years or older who were followed up at the James Whitcomb Riley Hospital for Children from 1978 to 1998 were reviewed. Age at diagnosis, target heights based on calculation of mid-parental heights, and FHs were examined. Because newborn screening for CAH has not yet been implemented in our state, all patients were identified by clinical presentation. Patients in whom the diagnosis
Retrospective Review
Charts from 65 patients (29 males and 36 females) with 21-hydroxylase deficiency were examined. Of these, 23 had completed linear growth and 42 were still growing. The time of diagnosis was “early” in 43 patients, and “late” in 22. Compliance was judged to be “good” in 28 patients. Typical follow-up consisted of pediatric endocrine clinic visits at 3- to 6-month intervals. Adjustments in glucocorticoid and mineralocorticoid doses were made on the basis of both auxological and biochemical
Discussion
CAH refers to a group of autosomal recessive disorders caused by deficiency of an enzyme involved in cortisol biosynthesis. The most commonly affected enzyme is 21-hydroxylase. Achievement of optimal growth in children with CAH is a well-recognized challenge in the treatment of this disorder because of an inability to adequately suppress corticotropin stimulation without simultaneously incurring the deleterious effects on growth of glucocorticoid overtreatment.8 The belief that adult height in
References (32)
- et al.
Experience with long-term therapy in congenital adrenal hyperplasia
J Pediatr
(1974) - et al.
Glucocorticoid treatment of girls with congenital adrenal hyperplasia: effects on height, sexual maturation and fertility
J Pediatr
(1977) - et al.
Clinical longitudinal standards for height and height velocity for North American children
J Pediatr
(1985) - et al.
Linear growth and pubertal development in treated congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Clin Endocrinol
(1977) - et al.
Accrescimento e sviluppo puberale nel maschio con sindrome adreno-genitale
Pediatr Med Chir
(1989) - et al.
Adult height and fertility in men with congenital virilizing adrenal hyperplasia
N Engl J Med
(1978) - et al.
Taille définitive chez 69 sujets atteints d’hyperplasie congénitale des surrénales par déficit en 21-hydroxylase
Arch Pediatr
(1994) - et al.
Growth and final height in classical and nonclassical 21-hydroxylase deficiency
J Endocrinol Invest
(1989) - et al.
The effect of treatment of final height in classical congenital adrenal hyperplasia (CAH)
Acta Endocrinol
(1986) - et al.
New approaches to the treatment of congenital adrenal hyperplasia
JAMA
(1997)
Therapeutic controversies: the use of adrenalectomy as a treatment for congenital adrenal hyperplasia
J Clin Endocrinol Metab
Adult height in women with early-treated congenital adrenal hyperplasia (21-hydroxylase type): relation to body mass index in earlier childhood
Acta Paediatr
Growth of patients with 21-hydroxylase deficiency: an analysis of the factors influencing adult height
Pediatr Res
Patterns of growth from birth to maturity in infants and children with congenital adrenal hyperplasia
Acta Endocrinol
Radiographic atlas of skeletal development of the hand and wrist
Longer term outcome in females with congenital adrenal hyperplasia (CAH): the Cardiff experience
Clin Endocrinol
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Reprint requests: Erica A. Eugster, MD, Clinical Assistant Professor of Pediatrics, Pediatric Endocrinology/Diabetology, Riley Hospital No. 5984, 702 Barnhill Dr, Indianapolis, IN 46202.