Abstract
In 22 patients with congenital adrenal hyperplasia (CAH), the effect of treatment on growth was evaluated retrospectively. The degree of control with treatment had generally been assessed by measurements of serum 17-hydroxyprogesterone (17-OHP) and/or urinary pregnanetriol levels. In 20 patients, there were no significant differences in both height SDS for bone age (−2.05 ±1.89 vs −1.85 ±1.30) and height SDS for chronological age (CA) (0.23 ±1.94 vs −0.36 ±1.81) in a mean uninterrupted treatment period of 5.80 ±3.58 years. Further analysis in salt-losers and nonsalt-losers separately showed that height SDS for bone age had decreased in the salt-losers and had increased in the nonsalt-losers. Six patients had signs of early puberty. In 22 patients, there was a negative correlation between the prednisolone dose on one hand and height velocity SDS for CA and bone age velocity on the other. It was also found that the mean serum levels of 17-OHP did not always reflect the degree of long term control measured by auxological parameters. These findings indicate that our treatment in the patients did not result in a significant improvement in height prognosis. We can conclude that growth prognosis in CAH patients can be improved by relying on auxological parameters rather than serum 17-OHP and urinary pregnanetriol levels for monitoring control. Special attention should be paid to salt-losers in this respect.
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References
Huma Z, Crawford C, New MI. Congenital adrenal hyperplasia. In: Brook CGD, ed.Clinical Paediatric Endocrinology. 3rd ed. Oxford; Blackwell Science, 1995.
Migeon CJ. Updating of the treatment of congenital adrenal hyperplasia.J Pediater 1968; 73: 805–806.
Laron Z, Pertzelan A. The comparative effect of 6 a-fluoroprednisolone, 6 a -methylprednisolone, and hydrocortisone on linear growth of children with congenital adrenal virilism and Addison’s disease.J Pediatr 1968; 73: 774–782.
New MI, Ghizzoni L, Speiser PW. Update on congenital adrenal hyperplasia. In: Lifshitz F, edPediatric Endocrinology. 3rd ed. New York; Marcel Dekker, 1996.
Bongiovanni AM, Root AW. The adrenogenital syndrome.N Engl J Med 1963; 268:1283–1289.
Korth-Schutz S. Virdis R, Saenger P, Chow DM, Levine LS, New MI. Serum androgens as a continuing index of adequacy of treatment of congenital adrenal hyperplasia.J Clin Endocrinol Metab 1978; 46:452–458.
Rappaport R, Cornu G, Royer P. Statural growth in congenital adrenal hyperplasia treated with hydrocortisone.J Pediatr 1968; 73: 760–766.
Brook CGD, Zachmann M, Prader A, Murset A. Experience with long-term theraphy in congenital adrenal hyperplasia.J Pediatr 1974; 85:12–19.
Appan S, Hindmarsh PC, Brook CGD. Monitoring treatment in congenital adrenal hyperplasia.Arch Dis Chid 1989; 64:1235–1239.
Greulich WWet al. Radiographic atlas of skeletal development of the hand and wrist 2nd ed, Standford; Standford Uni-versity Press, 1959.
Hung W, August GP, Glasgow AM.Pediatric Endocrinol-ogy. USA; Medical Examination Publishing Co., Inc., 1978:247.
Tanner JM, Whitehouse RH, Takaishi M. Standards from birth to maturity for height, weight velocity and height velocity: British children 1965.Arch Dis Child 1966; 41:613–635.
Tanner JM, Whitehouse RH, Cameron N, Marshall WA, Healy MJR, Golstein H. Effect of human growth hor-mone treatment for 1 to 7 years on growth of 100 chil-dren with growth hormone deficiency, low birth weight inherited smallness, Turner’s syndrome and other com-plaints.Arch Dis Child 1971; 46: 745–782.
Bergstrand CG. Growth in congenital adrenal hyperpla-sia.Acta Paediatr Scand 1966; 55:463–472.
Jaaskelainen J, Voutilainen R. Growth of patients with 21-hydroxylase deficiency: An analysis of the factors influencing adult height.Pediatr Res 1997; 41: 30–33.
Girgis R, Winter JSD. The effects of glucocorticoid replacement theraphy on growth, bone mineral density and bone turnover markers in children with congenital adrenal hyperplasia.J Clin Endocrinol Metab 1997; 82: 3926–3929.
Lippe BM, LaFranchi SH, Lavin N, Parlow A, Coyotuba J, Kaplan SA. Serum 17 oc-hydroxyprogesterone, progesterone, estradiol, and testosterone in the diagnosis and management of congenital adrenal hyperplasia.J Pediatr 1974; 85: 782–787.
Young MC, Robinson JA, Reas GF, Riad-Fahmy D, Hughes, LA. 17 OH progesterone rhythms in congenital adrenal hyperplasia.Arch Dis Child 1988; 63: 617–623.
Sandrini R, Jospe N, Migeon JG. Temporal and individul variations in the dose of glucocorticoid used for the treatment of salt-losing congenital virilizing adrenal hyperplasia due to 21-hydroxylase deficiency.Acta Paediatr Suppl 1993; 388: 56–60.
Rosier A, Levine LS, Schneider B, Novogroder M, New MI. The interrelationship of sodium balance, plasma renin activity and ACTH in congenital adrenal hyperplasia.J Clin Endocrinol Metab 1977; 45: 500–512.
Horner JM, Hintz RL, Luetscher JA. The role of renin and angiotensin in salt-losing, 21-hydroxylase-deficient congenital adrenal hyperplasia.J Clin Endocrinol Metab 1979; 48:776–783.
Siegel SF, Lee PA. Adrenal cortex and medulla. In: Hung W, edClinical Pediatric Endocrinology. St, Louis; Mosby -Year Book, 1992; 179–225.
Winter JSD. Marginal comment: Current approaches to the treatment of congenital adrenal hyperplasia.J Pediatr 1980; 97:81–82.
Gasparini N, Salerno MC, DiMaio S, Argenziano A, Franzese A, Tenore A. Growth pattern in congeni-tal adrenal hyperplasia (CAH) due to 21-OH defi-ciency during the first 36 months of life.Horm Res 1994; 41:111.
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Ercan, O., Hatemi, S., Kutlu, E. et al. Effect of treatment on growth in congenital adrenal hyperplasia. Indian J Pediatr 67, 783–789 (2000). https://doi.org/10.1007/BF02726219
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DOI: https://doi.org/10.1007/BF02726219