Long-term Growth in Congenital Adrenal Hyperplasia

Indian J Pediatr. 2019 Feb;86(2):154-158. doi: 10.1007/s12098-018-2753-6. Epub 2018 Aug 10.

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.

Keywords: Adult height; CAH; Growth.

MeSH terms

  • 17-alpha-Hydroxyprogesterone / blood
  • Administration, Oral
  • Adolescent
  • Adrenal Hyperplasia, Congenital / complications*
  • Adrenocorticotropic Hormone / blood
  • Body Height / drug effects
  • Body Height / physiology
  • Body Mass Index
  • Child
  • Electrolytes / blood
  • Female
  • Fludrocortisone / therapeutic use*
  • Growth / drug effects*
  • Growth / physiology*
  • Humans
  • Hydrocortisone / therapeutic use*
  • India
  • Male
  • Progesterone / blood
  • Retrospective Studies
  • Sex Factors
  • Sexual Maturation / drug effects
  • Testosterone / blood
  • Treatment Outcome

Substances

  • Electrolytes
  • Testosterone
  • Progesterone
  • 17-alpha-Hydroxyprogesterone
  • Adrenocorticotropic Hormone
  • Fludrocortisone
  • Hydrocortisone