Literature DB >> 19675839

Management of 21-hydroxylase deficiency congenital adrenal hyperplasia: A survey of Canadian paediatric endocrinologists.

Robert Stein1, Diane Wherrett, Denis Daneman.   

Abstract

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. RESULTS AND
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.

Entities:  

Keywords:  21-hydroxylase deficiency; Congenital adrenal hyperplasia

Year:  2005        PMID: 19675839      PMCID: PMC2722970     

Source DB:  PubMed          Journal:  Paediatr Child Health        ISSN: 1205-7088            Impact factor:   2.253


  30 in total

1.  The use of adrenalectomy as a treatment for congenital adrenal hyperplasia.

Authors:  J J Van Wyk; D F Gunther; E M Ritzén; A Wedell; G B Cutler; C J Migeon; M I New
Journal:  J Clin Endocrinol Metab       Date:  1996-09       Impact factor: 5.958

2.  Cortisol production rate in childhood and adolescence.

Authors:  B L Linder; N V Esteban; A L Yergey; J C Winterer; D L Loriaux; F Cassorla
Journal:  J Pediatr       Date:  1990-12       Impact factor: 4.406

3.  Growth and final height in classical and nonclassical 21-hydroxylase deficiency.

Authors:  M I New; J M Gertner; P W Speiser; P del Balzo
Journal:  Acta Paediatr Jpn       Date:  1988

4.  Estimation of daily cortisol production and clearance rates in normal pubertal males by deconvolution analysis.

Authors:  J R Kerrigan; J D Veldhuis; S A Leyo; A Iranmanesh; A D Rogol
Journal:  J Clin Endocrinol Metab       Date:  1993-06       Impact factor: 5.958

5.  [Final height in 69 patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency].

Authors:  M David; M Sempé; M Blanc; M Nicolino; M G Forest; Y Morel
Journal:  Arch Pediatr       Date:  1994-04       Impact factor: 1.180

Review 6.  Clinical review 54: Genetics, diagnosis, and management of 21-hydroxylase deficiency.

Authors:  W L Miller
Journal:  J Clin Endocrinol Metab       Date:  1994-02       Impact factor: 5.958

7.  Treatment and disease effects on short-term growth and adult height in children and adolescents with 21-hydroxylase deficiency.

Authors:  B P Hauffa; A Winter; H Stolecke
Journal:  Klin Padiatr       Date:  1997 Mar-Apr       Impact factor: 1.349

8.  Auxological and biochemical parameters in assessing treatment of infants and toddlers with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Authors:  S Einaudi; R Lala; A Corrias; P Matarazzo; S Pagliardini; C de Sanctis
Journal:  J Pediatr Endocrinol       Date:  1993 Apr-Jun

Review 9.  Steroid 21-hydroxylase deficiency (congenital adrenal hyperplasia).

Authors:  M I New
Journal:  Am J Med       Date:  1995-01-16       Impact factor: 4.965

10.  Adrenal 21-hydroxylase deficiency in childhood: 25 years' experience.

Authors:  Y J Lim; J A Batch; G L Warne
Journal:  J Paediatr Child Health       Date:  1995-06       Impact factor: 1.954

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