Volume 2015 Supplement 1

Abstracts from the 8th APPES Biennial Scientific Meeting

Open Access

Long term follow up of growth in children with Congenital Adrenal Hyperplasia 21- Hydroxylase deficiency

  • Ruchi Parikh1,
  • Sudha Rao1,
  • Meena Desai1,
  • Ruchi Shah1,
  • Poonam Singh1,
  • Neha Dighe1,
  • Rajesh Joshi1 and
  • Aparna Limaye1
International Journal of Pediatric Endocrinology20152015(Suppl 1):P47

DOI: 10.1186/1687-9856-2015-S1-P47

Published: 28 April 2015

Obesity and decreased final height are described in children with CAH 21OHD. Of 119 children (50 M, 69F;79 Salt Wasters(SW), 40 Simple Virilizers (SV)) diagnosed over 24 years, various growth parameters were studied in 43 children with regular follow up for 5 years or more.

Clinical data, anthropometry, genotype, hormonal and biochemical profile were evaluated at presentation. On follow up, growth and clinical characteristics, metabolic control (8am 17OH-Progesterone), bone age and replacement doses of gluco-corticoid (GC) and mineralo-corticoid (MC) were studied. Growth parameters were expressed as SDS. Obesity, defined as BMI SDS >/= 2 and short stature, defined as Ht SDS </= -2 were correlated with all the variables, using Unpaired T Test, Pearson Correlation and One way ANOVA Test.

43 children (16M, 27F; 32SW, 11SV; 36 mutations proven) had a mean duration of follow up of 11 +/- 4.14 years. At last follow up, Ht SDS was </= -2 in 27.9% cases (N=12/43: 4M, 8F; 9SW, 3SV). Age at onset of puberty (p=0.035), higher GC dose at presentation (>40mg/m2) (p=0.034) and at 3 years of age (p=0.047) and use of Hydrocortisone and/or Prednisolone (p=0.002) had a negative correlation with Ht SDS. 6 (13.95%) children (1M, 5F; 6SW) had achieved Adult Height (AH) SDS of -2.36 +/- 1.25, and AH SDS – TH SDS (Target Height) was -0.11 +/- 1.23. BMI SDS >/=2 found in 32.6% cases (N=14/43: 2M, 12F; 8SW, 6SV) correlated positively with 17 OHP values (p=0.013). Girls were heavier than boys (p=0.006). Adiposity rebound occurred at 4 years for both the genders. At the time of study analysis, Ht SDS showed a distinct shift to the left and BMI SDS, a distinct shift to the right of mean of the reference population as cited [1].

In the present series, there was a higher incidence of obesity (32.6%) but short stature was noted in 27.9% only. Aggressive lifestyle management, dietary control, optimizing dose of therapy (GC) and regular monitoring should be an integral part of long term management of patients with CAH 21OHD.

Authors’ Affiliations

(1)
Bai Jerbai Wadia Hospital for Children

References

  1. Agarwal KN, Agarwal DK: Indian Pediatrics. 1992, 29: 1203-1994; 31:377, 2001; 38:1217-35PubMedGoogle Scholar

Copyright

© Parikh et al; licensee BioMed Central Ltd. 2015

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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