THE GROWTH OF GROWTH HORMONE TREATED AND NOM TREATED CHILDREN WITH MYELOMENINGOCELE BEFORE AND AFTER TETHERED CORD RELEASE

Abstract

Children with nryelomeningocele (MM) are very short and respond to treatment (Rx) with recombinant human growth hormone (RHGH) with acceleration of growth rate (GR). This complex patient group frequently has tethering of the spinal cord (TSC) which untreated, may cause scoliosis and loss of neurofunction. The effect of tethered cord release (TCR) on the GR of children with HH alone or with RHGH Rx has not been assessed. We compared short term CR and standard deviation score for stature (SDS) of 13 pre-pubertal children (7 males, 6 females of mean age 6.1 ± 2.5 yrs) with MM, pre end post symptomatic TCR to a MM control group (MC) matched for age, tex and level of lesion who were followed over the seme time period, and did not have TCR. We also compared these two groups to 7 pre-pubertal children (4 males, 3 females of mean age 6.5 ± 2.0 yrs) with MM and GH inadequacy who had TCR and were then treated with RHGH. We evaluated GR from recumbent lengths measured on a horizontal stadiometer. GR was based on annualization of data points approximately 6 months pre and post TCR. For MC, we annualized lengths pre and post 6 months of the age of TCR to match the TCR group. For the RHGH Rx group, annualization of lengths, 10 months pre and post TCR, were used. TSC influences the GR of children with MM. Within the TCR group, GR increased significantly (p<.01). For TCR and TCR & RHGH groups, RHGH provides an increased gain of GR over TCR alone (p<.01). Among the three groups, TCR & RHGH in combination provides the largest gain in GR and SDS. Further study of TCR and RHGH will be necessary to assess their influence on adult stature of this extremely short population.