Constitutional Delay Influences the Auxological Response to Growth Hormone Treatment in Children with Short Stature and Growth Hormone Sufficiency

Abstract

In a retrospective, population based cohort study, we examined whether constitutional delay was associated with the growth response to growth hormone (GH) in children with short stature and normal GH responses. 70 patients were treated with 21 GH iu/m2/week from 1975 to 2013 throughout New Zealand. Demographic and auxological data were prospectively collected and standard deviation scores (SDS) were calculated for height (HtSDS), yearly growth velocity (GV-SDS), body mass index (BMI-SDS) and predicted adult height (PAH-SDS) at time of the last available bone age. In the first year, GH was associated with marked increase in HtSDS (+0.46 (0.19, 0.76), p < 0.001) and GV-SDS (from −1.9 (−3.6, −0.7) to +2.7 (0.45, 4.2), p < 0.001). The increase in HtSDS but not in GV-SDS was greatest with younger patients and greater bone age delay, with no effect of sex, BMI-SDS or baseline HtSDS. PAH-SDS increased with treatment (+0.94 (0.18, 1.5)); increased PAH-SDS was associated with less bone age delay and greater initial increase in HtSDS. This study shows that greater bone age delay was associated with greater initial improvement in height but less improvement in predicted adult heights, suggesting that children with very delayed bone ages may show accelerated maturation during GH treatment.

Severe short stature and Wolf-Hirschhorn syndrome: response to growth hormone in two cases without growth hormone deficiency

“…Children with idiopathic short stature and poor growth in New Zealand are eligible for publically funded treatment with GH in the same dose as used in these cases. In a systematic audit of non-syndromic, GH-sufficient children, the median increase for height was +0.46 SDS (interquartile range 0.19, 0.76) in the first year of treatment [ 7 ]. The present two cases show that GH treatment of WHS in childhood can achieve a similar or potentially even greater acceleration of growth.…”

“…Classically, children with WHS show developmental delay with hypotonia and seizures, with marked pre- and postnatal growth retardation [ 1 ]. Given the often extreme short stature, children with WHS would be potentially eligible for treatment with exogenous growth hormone (GH) in many countries [ 7 ]. We report the response to GH in childhood of two unrelated cases without GH deficiency.…”

A surprising treatment response in a patient with rare isolated growth hormone deficiency, type IB

“…However, these trends are by no means definitive. Moreover, both IGHD and Constitutional Growth Delay may show poor response to provocative testing ( 9 ), calling into question the inter-trial reliability of testing ( 10 ). Given the clinical equipoise, we elected for genetic testing.…”