Abstract
GH deficiency (GHD) in patients with myelomeningocele leads to the question of whether these disabled patients should be treated with human GH. To date, only a few short-term reports of GH
therapy are available in the literature, and long-term data for final
height are lacking. We report auxological and laboratory data for seven
prepubertal myelomeningocele patients with proven GHD (idiopathic GHD or neurosecretory dysfunction) during GH
treatment. All patients (five males and two females; median
chronological age, 6.6 yr) had shunted hydrocephalus and were treated
with GH (0.5 IU/kg x week; 0.15 mg/kg x week; daily sc injections) over a median period of 38 months (range, 35-49 months). GH secretion was analyzed by measurement of spontaneous overnight GH secretion and two standard stimulation tests. Auxological parameters, bone age, serum levels of insulin-like growth factor I and insulin-like growth factor-binding protein-3, and neurological and orthopedic status were documented regularly. Median growth
velocity of supine length improved during treatment (at start, 3.7
cm/yr; after 36 months, 5.7 cm/yr; P < 0.05), with highest levels 6
months after the start of therapy (8.1 cm/yr). The growth
velocity of arm span was greater than these values. Supine length SD
score for chronological age increased from -4.71 (at start) to -3.35
(after 36 months; P = NS), length SD score for bone age increased from
-2.70 to -2.23 (P = NS), and arm span SD score increased from -2.98 to
-1.75 (P < 0.05). The growth
velocities of length and arm span remained significantly above the
pretreatment values (P < 0.05). Symptomatic tethered cord associated
with progression of scoliosis developed in two of seven children. GH treatment significantly improved the growth
velocities of body length and arm span. However, the increase in length
SD score was not significant, whereas arm span SD scores significantly
improved over the study period.
PMID: 10946874
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