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August 1985, Vol 139, No. 8 >
Article | August 1985

Growth and Pubertal Development in Patients With Congenital Adrenal Hyperplasia due to 11-β-Hydroxylase Deficiency FREE

Zeev Hochberg, MD; Jakob Schechter, MD; Abraham Benderly, MD; Esther Leiberman, MD; Ariel Rosler, MD
[+] Author Affiliations

Reprint requests to Endocrine Laboratory, Rambam Medical Center, PO Box 9602, Haifa 31096, Israel (Dr Hochberg).


Am J Dis Child. 1985;139(8):771-776. doi:10.1001/archpedi.1985.02140100033020.
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• To evaluate growth and pubertal development in patients with congenital adrenal hyperplasia due to 11-β-hydroxylase deficiency, data were collected retrospectively from the charts of 24 patients, 15 girls and nine boys. Growth before diagnosis was characterized by a rapid acceleration in the second half of the first year of life, with rapid advancement of bone age. Final height was severely compromised in all the patients, regardless of age at diagnosis and quality of therapeutic control. Onset of puberty was precocious in male patients and normal in female patients. In this respect, treatment with hydrocortisone acetate proved to be superior to cortisone acetate or prednisone.

(AJDC 1985;139:771-776)

REFERENCES

1 +
Eberlein WH, Bongiovanni AM:  Congenital adrenal hyperplasia with hypertension: Unusual steroid pattern in blood and urine . J Clin Endocrinol Metabol 1955;;15:1531-1539.
2 +
Zachmann M, Tassinari D, Prader A:  Clinical and biochemical variability of congenital adrenal hyperplasia due to 11-β-hydroxylase deficiency: A study of 25 patients . J Clin Endocrinol Metabol 1983;;56:222-230.
3 +
Rosler A, Leiberman E:  Enzymatic defects of steroidogenesis: 11-β-hydroxylase deficiency, congenital adrenal hyperplasia , in Laron Z (ed): Adrenal Diseases in Childhood . Basel, Switzerland, S Karger, 1984;, vol 13: Pediatric and Adolescent Endocardiology Series, pp 47-71.
4 +
Rosier A, Leiberman E, Sack J, et al:  Clinical variability of congenital adrenal hyperplasia due to 11-hydroxylase deficiency . Horm Res 1982;;16:133-141.
5 +
Zadik Z, Kahana L, Kaufman H, et al:  Salt loss in hypertensive form of congenital adrenal hyperplasia (11-hydroxylase deficiency) . J Clin Endocrinol Metabol 1984;;58:384-391.
6 +
Hochberg Z, Benderly A, Kahana L, et al:  Sodium balance in glucocorticoid-treated patients with 11-hydroxylase deficiency , abstracted. Pediatr Res 1984;;18:116.
7 +
Tanner JM, Whitehouse RH, Takaishi M:  Standards from birth to maturity for height, weight, height velocity, and weight velocity: British children, 1965 . Arch Dis Child 1966;;41:454-471, 613-635.
8 +
Laron Z:  Somatic growth of children in warm climate . Nordic Council Arct Med Res Rep 1976;;14:15-24.
9 +
Polgi A:  Ethnic differences in adulthood growth attainment of first-generation Israelis and in their babies' birth weights . Hum Biol 1984;;56:355-364.
10 +
Marshall WA, Tanner JM:  Variations in pattern of pubertal changes in girls . Arch Dis Child 1969;;44:291-303.
11 +
Marshall WA, Tanner JM:  Variations in pattern of pubertal changes in boys . Arch Dis Child 1970;;45:13-24.
12 +
Bailey CC, Komrower GM:  Growth and skeletal maturation in congenital adrenal hyperplasia: Review of 20 cases . Arch Dis Child 1974;; 49:4-7.
13 +
Raiti S, Newns GH:  Linear growth in treated congenital adrenal hyperplasia . Arch Dis Child 1971;;46:376-378.
14 +
Rappaport R, Gornu G, Royer P:  Statural growth in congenital adrenal hyperplasia treated with hydrocortisone . J Pediatr 1968;;73:760-766.
15 +
Rappaport R, Bouthreuil E, Marti-Henneberg C, et al:  Linear growth rate, bone maturation, and growth hormone secretion in prepubertal children with congenital adrenal hyperplasia . Acta Paediatr Scand 1973;;62:513-519.
16 +
Sperling MA, Kenny FM, Schutt-Aine JC, et al:  Linear growth and growth hormonal responsiveness in treated congenital adrenal hyperplasia . AJDC 1971;;122:408-418.
17 +
Hochberg Z, Benderly A, Zadik Z:  Salt loss in congenital adrenal hyperplasia due to 11-β-hydroxylase deficiency . Arch Dis Child 1984;;59:1092-1094.
18 +
Rayyis SS, Horton R:  Effect of angiotensin II on adrenal and pituitary function in man . J Clin Endocrinol Metabol 1971;;32:539-546.
19 +
Steele MK, Negro-Vilar A, McCann SM:  Effect of angiotensin II on in vivo and in vitro release of anterior pituitary hormone in the female rat . Endocrinology 1981;;109:893-899.
20 +
Steele MK, McCann SM, Negro-Vilar A:  Modulation by dopamine and estradiol of the central effects of angiotensin II on anterior pituitary hormone release . Endocrinology 1982;;111: 722-729.
21 +
Spinedi E, Negro-Vilar A:  Angiotensin II increases ACTH release in the absence of endogenous arginine vasopressin . Life Sci 1984;;34: 721-724.
22 +
Rosler A, Levine LS, Schneider B, et al:  Interrelationship of sodium balance, plasma renin activity, and ACTH in congenital adrenal hyperplasia . J Clin Endocrinol Metabol 1977;; 45:500-512.
23 +
Richman RA, Hochberg Z, Streeten DHP: Effect of mineralocorticoid on the required dosage in congenital adrenal hyperplasia, abstracted. Proceedings of the 61st annual meeting of the Endocrine Society, Anaheim, Calif, 1979, p 232.
24 +
Laron Z, Pertzelan A:  The comparative effects of 6-α-fluoroprednisolone, 6-α-methylprednisolone, and hydrocortisone on linear growth of children with congenital adrenal virilism and Addison's disease . J Pediatr 1968;;73: 774-782.

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References

1 +
Eberlein WH, Bongiovanni AM:  Congenital adrenal hyperplasia with hypertension: Unusual steroid pattern in blood and urine . J Clin Endocrinol Metabol 1955;;15:1531-1539.
2 +
Zachmann M, Tassinari D, Prader A:  Clinical and biochemical variability of congenital adrenal hyperplasia due to 11-β-hydroxylase deficiency: A study of 25 patients . J Clin Endocrinol Metabol 1983;;56:222-230.
3 +
Rosler A, Leiberman E:  Enzymatic defects of steroidogenesis: 11-β-hydroxylase deficiency, congenital adrenal hyperplasia , in Laron Z (ed): Adrenal Diseases in Childhood . Basel, Switzerland, S Karger, 1984;, vol 13: Pediatric and Adolescent Endocardiology Series, pp 47-71.
4 +
Rosier A, Leiberman E, Sack J, et al:  Clinical variability of congenital adrenal hyperplasia due to 11-hydroxylase deficiency . Horm Res 1982;;16:133-141.
5 +
Zadik Z, Kahana L, Kaufman H, et al:  Salt loss in hypertensive form of congenital adrenal hyperplasia (11-hydroxylase deficiency) . J Clin Endocrinol Metabol 1984;;58:384-391.
6 +
Hochberg Z, Benderly A, Kahana L, et al:  Sodium balance in glucocorticoid-treated patients with 11-hydroxylase deficiency , abstracted. Pediatr Res 1984;;18:116.
7 +
Tanner JM, Whitehouse RH, Takaishi M:  Standards from birth to maturity for height, weight, height velocity, and weight velocity: British children, 1965 . Arch Dis Child 1966;;41:454-471, 613-635.
8 +
Laron Z:  Somatic growth of children in warm climate . Nordic Council Arct Med Res Rep 1976;;14:15-24.
9 +
Polgi A:  Ethnic differences in adulthood growth attainment of first-generation Israelis and in their babies' birth weights . Hum Biol 1984;;56:355-364.
10 +
Marshall WA, Tanner JM:  Variations in pattern of pubertal changes in girls . Arch Dis Child 1969;;44:291-303.
11 +
Marshall WA, Tanner JM:  Variations in pattern of pubertal changes in boys . Arch Dis Child 1970;;45:13-24.
12 +
Bailey CC, Komrower GM:  Growth and skeletal maturation in congenital adrenal hyperplasia: Review of 20 cases . Arch Dis Child 1974;; 49:4-7.
13 +
Raiti S, Newns GH:  Linear growth in treated congenital adrenal hyperplasia . Arch Dis Child 1971;;46:376-378.
14 +
Rappaport R, Gornu G, Royer P:  Statural growth in congenital adrenal hyperplasia treated with hydrocortisone . J Pediatr 1968;;73:760-766.
15 +
Rappaport R, Bouthreuil E, Marti-Henneberg C, et al:  Linear growth rate, bone maturation, and growth hormone secretion in prepubertal children with congenital adrenal hyperplasia . Acta Paediatr Scand 1973;;62:513-519.
16 +
Sperling MA, Kenny FM, Schutt-Aine JC, et al:  Linear growth and growth hormonal responsiveness in treated congenital adrenal hyperplasia . AJDC 1971;;122:408-418.
17 +
Hochberg Z, Benderly A, Zadik Z:  Salt loss in congenital adrenal hyperplasia due to 11-β-hydroxylase deficiency . Arch Dis Child 1984;;59:1092-1094.
18 +
Rayyis SS, Horton R:  Effect of angiotensin II on adrenal and pituitary function in man . J Clin Endocrinol Metabol 1971;;32:539-546.
19 +
Steele MK, Negro-Vilar A, McCann SM:  Effect of angiotensin II on in vivo and in vitro release of anterior pituitary hormone in the female rat . Endocrinology 1981;;109:893-899.
20 +
Steele MK, McCann SM, Negro-Vilar A:  Modulation by dopamine and estradiol of the central effects of angiotensin II on anterior pituitary hormone release . Endocrinology 1982;;111: 722-729.
21 +
Spinedi E, Negro-Vilar A:  Angiotensin II increases ACTH release in the absence of endogenous arginine vasopressin . Life Sci 1984;;34: 721-724.
22 +
Rosler A, Levine LS, Schneider B, et al:  Interrelationship of sodium balance, plasma renin activity, and ACTH in congenital adrenal hyperplasia . J Clin Endocrinol Metabol 1977;; 45:500-512.
23 +
Richman RA, Hochberg Z, Streeten DHP: Effect of mineralocorticoid on the required dosage in congenital adrenal hyperplasia, abstracted. Proceedings of the 61st annual meeting of the Endocrine Society, Anaheim, Calif, 1979, p 232.
24 +
Laron Z, Pertzelan A:  The comparative effects of 6-α-fluoroprednisolone, 6-α-methylprednisolone, and hydrocortisone on linear growth of children with congenital adrenal virilism and Addison's disease . J Pediatr 1968;;73: 774-782.

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