Final adult height in long-term growth hormone-treated achondroplasia patients

Eur J Pediatr. 2017 Jul;176(7):873-879. doi: 10.1007/s00431-017-2923-y. Epub 2017 May 13.

Abstract

The objective of this study was to evaluate the gain in final height of achondroplasia (ACH) patients with long-term growth hormone (GH) treatment. We analyzed medical data of 22 adult patients (8 males and 14 females) treated with GH at a dose of 0.05 mg/kg/day. Optionally, tibial lengthening (TL) was performed with the Ilizalov method in 15 patients and TL as well as femoral lengthening (FL) in 6 patients. Concomitant gonadal suppression therapy with buserelin acetate was applied in 13 patients. The mean treatment periods with GH were 10.7 ± 4.0 and 9.3 ± 2.5 years for males and females, respectively. GH treatment augmented the final height +0.60 ± 0.52 SD (+3.5 cm) and +0.51 ± 1.29 SD (+2.8 cm) in males and females compared to non-treated ACH patients, respectively. Final height of ACH patients that underwent GH and TL increased +1.72 ± 0.72 SD (+10.0 cm) and +1.95 ± 1.34 SD (+9.8 cm) in males and females, respectively. GH, TL, and FL increased their final height +2.97 SD (+17.2 cm) and +3.41 ± 1.63 SD (+17.3 cm) in males and females, respectively. Gonadal suppression therapy had no impact on final height.

Conclusions: Long-term GH treatment contributes to 2.6 and 2.1% of final adult height in male and female ACH patients, respectively.

Keywords: Achondroplasia; Final height; Gonadal suppression; Growth hormone; Limb lengthening.

MeSH terms

  • Achondroplasia / drug therapy*
  • Achondroplasia / physiopathology
  • Adolescent
  • Adult
  • Body Height*
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Growth Hormone / therapeutic use*
  • Humans
  • Injections, Subcutaneous
  • Male
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Growth Hormone