Skip to main content
Log in

Long-term Growth in Congenital Adrenal Hyperplasia

  • Original Article
  • Published:
The Indian Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Objectives

To retrospectively assess growth of children with congenital adrenal hyperplasia (CAH) with special reference to puberty and to assess longitudinal growth and final height of subset of children with CAH.

Methods

A retrospective analysis of 30 children (14 boys) with classic CAH (11 salt wasters, 19 simple virilisers) followed up for a mean duration of 9.9 ± 2.4 y (Study period December 2002 through December 2016) was performed. Height Z scores, target height Z scores, height velocities and laboratory parameters were analysed.

Results

Children were treated with hydrocortisone in a mean dose of 15.7 ± 3.3 mg/m2/d. Mean 17-hydroxy progesterone in boys and girls were 10.8 ± 6.7 ng/ml and 11.3 ± 9.3 ng/ml respectively. Fifteen children (7 boys) developed central precocious puberty at mean age of 7.6 ± 1.8 y and 13 were treated with GnRH analogues for 3.5 y. Of all patients, 18 (10 girls, 8 boys) reached final height at a mean age of 14.2 ± 1.6 y. Mean final height achieved was 158.0 ± 8.5 cm in boys [target height (TH) -165.5 ± 3.8 cm] and in girls it was 149.9 ± 6.7 cm [target height (TH) 154.7 ± 6.4 cm]. Final height standard deviation scores (SDS) for boys and girls were − 2.06 ± 1.1 (TH-SDS -1.06 ± 0.5) and − 1.47 ± 1.1 (TH-SDS -0.56 ± 1.2) respectively and were not significantly different from target height Z scores (p > 0.05). Growth velocity was attenuated during pubertal years.

Conclusions

Monitoring growth and puberty in children with CAH is critical for optimizing final height.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Wilkins L, Lewis RA, Klien R, Rosemberg E. The suppression of androgen secretion by cortisone in case of congenital adrenal hyperplasia: preliminary report. Bull Johns Hopkins Hosp. 1950;86:249–52.

    CAS  PubMed  Google Scholar 

  2. Speiser PW, Azziz R, Baskin LS, et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2010;95:4133–60.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Mullis PE, Hindmarsh PC, Brook CG. Sodium chloride supplement at diagnosis and during infancy in children with salt-losing 21-hydroxylase deficiency. Eur J Pediatr. 1990;150:22–5.

    Article  CAS  PubMed  Google Scholar 

  4. Marshall WA, Tanner JM. Variations in pattern of pubertal changes in girls. Arch Dis Child. 1969;44:291–303.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Khadilkar VV, Khadilkar AV, Cole TJ, Sayyad MG. Cross-sectional growth curves for height, weight, body mass index for affluent Indian children. Indian Pediatr. 2009;46:477–89.

    CAS  PubMed  Google Scholar 

  6. Tanner JM, Goldstein H, Whitehouse RH. Standards for children’s height at ages 2-9 years allowing for heights of parents. Arch Dis Child. 1970;45:755–62.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Nwosu B, Lee MM. Evaluation of short and tall stature in children. Am Fam Physician. 2008;78:597–604.

    PubMed  Google Scholar 

  8. Dattani MT, Ziaferi VT, Hindmarsh PC. Evaluation of disordered puberty. In: Brook CGD, Clayton P, Brown RS, editors. Brook’s clinical pediatric endocrinology. 6th ed. United Kingdom: Wiley Blackwell; 2009. p. 213–38.

    Google Scholar 

  9. Bayley N, Pinneau SR. Tables for predicting final height for skeletal age: revised for use with Greulich-Pyle hand standards. J Pediatr. 1952;40:423–41.

    Article  CAS  PubMed  Google Scholar 

  10. Gilsasz V, Ratib O. Hand bone age: a digital atlas of skeletal maturity. United Kingdom: Springer; 2011.

    Google Scholar 

  11. Tanner JM, Healy M, Goldstein H, Cameron N. Assessment of skeletal maturity and prediction of adult height (TW3 method). 3rd ed. London: WB Saunders, Harcourt Publishers Ltd; 2001.

    Google Scholar 

  12. Miller WL. The adrenal cortex and its disorders. In: Brook CGD, Clayton P, Brown RS, editors. Brook’s clinical pediatric endocrinology. 6th ed. United Kingdom: Wiley Blackwell; 2009. p. 283–325.

    Google Scholar 

  13. Soliman A, Allamki M, Alsalmi I, Maurice A. Congenital adrenal hyperplasia complicated by central precocious puberty: linear growth during infancy and treatment with gonadotropic –releasing analog. Metabolism. 1997;46:513–7.

    Article  CAS  PubMed  Google Scholar 

  14. Gueven A, Nurcan Cebeci A, Hancili S. Gonadotropin releasing hormone analog treatment in children with congenital adrenal hyperplasia complicated by central precocious puberty. Hormones (Athens). 2015;14:265–71.

  15. Aycan Z, Ocal G, Berberoglu M, Cetinkaya E, Adiyaman P, Evliyaoglu O. Experience with long term glucocorticoid treatment in congenital adrenal hyperplasia: growth pattern compared with genetic height potential. J Pediatr Endocrinol Metab. 2006;19:245–51.

    Article  CAS  PubMed  Google Scholar 

  16. Bonfig W, Pozza SB, Schimdt H, Pagel P, Scharz HP. Hydrocortisone dosing during puberty in patients with classical congenital adrenal hyperplasia: an evidence based recommendation. J Clin Endocrinol Metab. 2009;94:3882–8.

    Article  CAS  PubMed  Google Scholar 

  17. Finkielstein GP, Kim MS, Sinaii N, et al. Clinical characteristics of a cohort of 244 patients with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 2012;97:4429–38.

    Article  CAS  Google Scholar 

  18. Manoli I, Kanaka-Gantenbein C, Voutetakis A. Maniati- Christidi M, Dacou-Voutetakis C. Early growth, pubertal development, body mass index and final height of patients with congenital adrenal hyperplasia: factors influencing the outcome. Clin Endocrinol. 2002;57:669–76.

    Article  CAS  Google Scholar 

  19. Bretones P, Riche B, Pichot E, et al. Growth curves for congenital adrenal hyperplasia from a national retrospective cohort. J Pediatr Endocrinol Metab. 2016;29:1379–88.

    Article  CAS  PubMed  Google Scholar 

  20. Grigorescu-Sido A, Bettendorf M, Schulze E, Duncea I, Heinrich U. Growth analysis in patients with 21-hydroxylase deficiency, influence of glucocorticoid dosage, age at diagnosis, phenotype and genotype on growth and height outcome. Horm Res. 2003;60:84–90.

    CAS  PubMed  Google Scholar 

  21. Bonfig W, Bechtold S, Schmidt H, Knorr D, Schwarz HP. Reduced final height outcome in congenital adrenal hyperplasia under prednisolone treatment: deceleration of growth velocity during puberty. J Clin Endocrinol Metab. 2007;92:1635–9.

    Article  CAS  PubMed  Google Scholar 

  22. Eugster EA, DiMeglio LA, Wright JC, Freidenberg GR, Seshadri R, Pescovitz OH. Height outcome in congenital adrenal hyperplasia caused by 21-hydroxylase deficiency: a meta analysis. J Pediatr. 2000;138:26–32.

    Article  Google Scholar 

  23. Muthuswamy K, Elamin MB, Smushkin G, et al. Clinical review: adult height in patients with congenital adrenal hyperplasia: a systemic review and metaanalysis. J Clin Endocrinol Metab. 2010;95:4161–72.

    Article  CAS  Google Scholar 

  24. Jaaskelainen J, Voutilainen R. Growth of patients with 21-hydroxylase deficiency: an analysis of the factors influencing adult height. Pediatr Res. 1997;41:30–3.

    Article  CAS  PubMed  Google Scholar 

  25. Frisch H, Waldhauser F, Lebl J, et al. MEWPE- CAH study group. Congenital adrenal hyperplasia: lessons from a multinational study. Horm Res. 2002;57:95–101.

    CAS  PubMed  Google Scholar 

  26. Muirhead S, Sellers EAC, Guyda H. Indicators of adult height outcome in classical 21- hydroxylase deficiency congenital adrenal hyperplasia. J Pediatr. 2002;141:247–52.

    Article  CAS  PubMed  Google Scholar 

  27. Balsamo A, Cicognani A, Baldazzi L, et al. CYP21 genotype, adult height and pubertal development in 55 patients treated for 21- hydroxylase deficiency. J Clin Endocrinol Metab. 2003;88:5680–8.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Contributions

VK: Design and concept of the study, design, revision and final submission of manuscript; AM: Design and concept of the study, data collection, initial analysis, design and final submission of the manuscript; PG: Data collection, design and final submission of the manuscript; AK: Initial analysis, revision and final submission of the manuscript; VK will act as guarantor for this paper.

Corresponding author

Correspondence to Anuradha Khadilkar.

Ethics declarations

Conflict of Interest

None.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Maheshwari, A., Khadilkar, V., Gangodkar, P. et al. Long-term Growth in Congenital Adrenal Hyperplasia. Indian J Pediatr 86, 154–158 (2019). https://doi.org/10.1007/s12098-018-2753-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12098-018-2753-6

Keywords

Navigation