Management of 21-hydroxylase deficiency congenital adrenal hyperplasia: A survey of Canadian paediatric endocrinologists.
BACKGROUND
Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is one of the most common autosomal recessive disorders. Many issues in the management of CAH in children still remain unresolved.


OBJECTIVE
To assess how children with CAH are treated in Canada.


METHODS
Fifty-nine paediatric endocrinologists and postgraduate trainees from across Canada took part in a survey that evaluated four areas of CAH management: type and dose of glucocorticoid therapy, current use of alternative therapies, monitoring of care, and approach/attitude to prenatal diagnosis and treatment of CAH.


CONCLUSIONS
The present survey demonstrated that there is general agreement among paediatric endocrinologists in Canada regarding the management of patients with CAH, which includes very little use of newer antiandrogen therapies. The goal remains to be the optimization of currently available therapy to ensure normal growth and sexual maturation without any evidence of glucocorticoid excess or deficiency. Prenatal diagnosis and management is widely, but infrequently, used.
Stein
Robert
R
Division of Endocrinology, Department of Pediatrics, Children's Hospital of Western Ontario and University of Western Ontario, London.
Wherrett
Diane
D
Daneman
Denis
D
J Clin Endocrinol Metab. 2001 Dec;86(12):5651-7
11739415
Arch Dis Child. 1988 Nov;63(11):1399-404
3060026
J Clin Endocrinol Metab. 1994 Feb;78(2):241-6
8106606
J Clin Endocrinol Metab. 1993 Jun;76(6):1505-10
8501158
Pediatrics. 2002 Feb;109(2):E28
11826238
Am J Med. 1995 Jan 16;98(1A):2S-8S
7825636
Horm Res. 1983;18(4):175-85
6227545
Paediatr Drugs. 2001;3(8):599-611
11577925
J Clin Endocrinol Metab. 2000 Mar;85(3):1114-20
10720048
Arch Dis Child. 1989 Sep;64(9):1235-9
2640553
J Pediatr. 2003 Sep;143(3):402-5
14517528
Arch Pediatr. 1994 Apr;1(4):363-7
7842090
Endocr Rev. 2000 Jun;21(3):245-91
10857554
J Clin Endocrinol Metab. 1978 Mar;46(3):452-8
156192
Horm Res. 2002;58(4):188-95
12324718
Klin Padiatr. 1997 Mar-Apr;209(2):71-7
9113619
Arch Dis Child. 1997 Sep;77(3):214-8
9370898
Horm Res. 1980;13(6):347-57
7196877
J Pediatr. 1979 Jul;95(1):33-7
480011
J Paediatr Child Health. 1995 Jun;31(3):222-7
7669384
Pediatrics. 2000 Oct;106(4):767-73
11015521
J Clin Endocrinol Metab. 1996 Sep;81(9):3180-90
8784066
J Pediatr. 1990 Dec;117(6):892-6
2104527
Acta Paediatr Jpn. 1988;30 Suppl:79-88
3146882
J Pediatr. 2002 Aug;141(2):247-52
12183722
J Pediatr Endocrinol. 1993 Apr-Jun;6(2):173-8
8348221
J Pediatr. 1999 Feb;134(2):185-9
9931527
Bull Johns Hopkins Hosp. 1950 Apr;86(4):249-52
15411555
Endocrinol Metab Clin North Am. 2001 Mar;30(1):31-59, vi
11344938
Adv Pediatr. 1995;42:113-43
8540426