Literature DB >> 2640553

Monitoring treatment in congenital adrenal hyperplasia.

S Appan1, P C Hindmarsh, C G Brook.   

Abstract

We report results of monitoring treatment in 41 patients with congenital adrenal hyperplasia controlled over 0.3-13.1 years using standard auxological techniques alone. Doses of glucocorticoid (15-25 mg/m2/day) and mineralocorticoid (0.15 mg/m2/day) replacement were determined initially using biochemical indices and thereafter adjusted according to surface area. Monitoring was solely directed at maintaining a 50th centile height velocity for chronological age. Of 41 patients, 32 were referred after the newborn period. Nearly half of these patients were either overtreated or undertreated before their referral. Of the nine treated from birth, all but one were in good control and only two have had a second hospital admission. Present height standard deviation scores (SDS) for chronological age range from -1.60 to -0.26. Height SDS for bone age were compared with midparental heights in 33 patients: 15 treated with early emphasis on growth had a height prognosis exceeding midparental values; patients who had experienced appreciable prior overtreatment or undertreatment fared less well. In the long term management of congenital adrenal hyperplasia correction of salt loss is of primary importance. Doses of glucocorticoid required in addition to mineralcorticoid replacement should be continuously assessed and adjusted to maintain a normal growth velocity. This is most conveniently achieved by standardising replacement doses on surface area.

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Year:  1989        PMID: 2640553      PMCID: PMC1792744          DOI: 10.1136/adc.64.9.1235

Source DB:  PubMed          Journal:  Arch Dis Child        ISSN: 0003-9888            Impact factor:   3.791


  12 in total

1.  The interrelationship of sodium balance, plasma renin activity and ACTH in congenital adrenal hyperplasia.

Authors:  A Rösler; L S Levine; B Schneider; M Novogroder; M I New
Journal:  J Clin Endocrinol Metab       Date:  1977-09       Impact factor: 5.958

2.  Variable efficacy of glucocorticoids in congenital adrenal hyperplasia.

Authors:  J W Hansen; D L Loriaux
Journal:  Pediatrics       Date:  1976-06       Impact factor: 7.124

Review 3.  Theoretical approaches to estimation of plasma renin activity: a review and some original observations.

Authors:  S Oparil
Journal:  Clin Chem       Date:  1976-05       Impact factor: 8.327

4.  The effect of treatment of final height in classical congenital adrenal hyperplasia (CAH).

Authors:  J DiMartino-Nardi; E Stoner; A O'Connell; M I New
Journal:  Acta Endocrinol Suppl (Copenh)       Date:  1986

5.  Experience with long-term therapy in congenital adrenal hyperplasia.

Authors:  C G Brook; M Zachmann; A Prader; G Mürset
Journal:  J Pediatr       Date:  1974-07       Impact factor: 4.406

6.  Circadian variation in plasma 17-hydroxyprogesterone in patients with congenital adrenal hyperplasia.

Authors:  S M Atherden; N D Barnes; D B Grant
Journal:  Arch Dis Child       Date:  1972-08       Impact factor: 3.791

7.  Modern medical therapy of congenital adrenal hyperplasia. A decade of experience.

Authors:  J S Winter; R M Couch
Journal:  Ann N Y Acad Sci       Date:  1985       Impact factor: 5.691

8.  Monitoring treatment in congenital adrenal hyperplasia. Use of serial measurements of 17-OH-progesterone in plasma, capillary blood, and saliva.

Authors:  I A Hughes; J Dyas; J Robinson; R F Walker; D R Fahmy
Journal:  Ann N Y Acad Sci       Date:  1985       Impact factor: 5.691

9.  Standards from birth to maturity for height, weight, height velocity, and weight velocity: British children, 1965. II.

Authors:  J M Tanner; R H Whitehouse; M Takaishi
Journal:  Arch Dis Child       Date:  1966-12       Impact factor: 3.791

10.  Circadian patterns of plasma cortisol, 17-hydroxyprogesterone, and testosterone in congenital adrenal hyperplasia.

Authors:  H Frisch; K Parth; E Schober; W Swoboda
Journal:  Arch Dis Child       Date:  1981-03       Impact factor: 3.791

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  12 in total

1.  Sodium chloride supplement at diagnosis and during infancy in children with salt-losing 21-hydroxylase deficiency.

Authors:  P E Mullis; P C Hindmarsh; C G Brook
Journal:  Eur J Pediatr       Date:  1990-11       Impact factor: 3.183

2.  Randomised controlled trial of growth effect of hydrocortisone in congenital adrenal hyperplasia.

Authors:  I N Silva; C E Kater; C F Cunha; M B Viana
Journal:  Arch Dis Child       Date:  1997-09       Impact factor: 3.791

3.  Monitoring treatment in congenital adrenal hyperplasia.

Authors:  I A Hughes
Journal:  Arch Dis Child       Date:  1990-03       Impact factor: 3.791

4.  Response to treatment of congenital adrenal hyperplasia in infancy.

Authors:  M C Young; I A Hughes
Journal:  Arch Dis Child       Date:  1990-04       Impact factor: 3.791

5.  Effect of treatment on growth in congenital adrenal hyperplasia.

Authors:  O Ercan; S Hatemi; E Kutlu; N Turan
Journal:  Indian J Pediatr       Date:  2000-11       Impact factor: 1.967

6.  An Evidence-Based Model of Multidisciplinary Care for Patients and Families Affected by Classical Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency.

Authors:  Traci L Schaeffer; Jeanie B Tryggestad; Ashwini Mallappa; Adam E Hanna; Sowmya Krishnan; Steven D Chernausek; Laura J Chalmers; William G Reiner; Brad P Kropp; Amy B Wisniewski
Journal:  Int J Pediatr Endocrinol       Date:  2010-03-18

7.  Obesity in 21-hydroxylase deficient patients.

Authors:  R E Cornean; P C Hindmarsh; C G Brook
Journal:  Arch Dis Child       Date:  1998-03       Impact factor: 3.791

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

Authors:  Robert Stein; Diane Wherrett; Denis Daneman
Journal:  Paediatr Child Health       Date:  2005-07       Impact factor: 2.253

Review 9.  Diagnosis of diseases of steroid hormone production, metabolism and action.

Authors:  John W Honour
Journal:  J Clin Res Pediatr Endocrinol       Date:  2009-08-02

10.  The Range of 2.2-3.3 mg/gCr of Pregnanetriol in the First Morning Urine Sample as an Index of Optimal Control in CYP21 Deficiency.

Authors:  Masako Izawa; Keiko Aso; Asako Higuchi; Daisuke Ariyasu; Yukihiro Hasegawa
Journal:  Clin Pediatr Endocrinol       Date:  2008-08-08
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