GH deficiency (GHD) in patients with myelomeningocele leads to the question
of whether these disabled patients should be treated with human GH. To dat
e, only a few short-term reports of GH therapy are available in the:literat
ure, and long-term data for final height are lacking. We report auxological
and laboratory data for seven prepubertal myelomeningocele patients with p
roven GHD (idiopathic GHD or neurosecretory dysfunction) during GH treatmen
t.
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 . week; 0.15
mg/kg . week; daily sc injections) over a median period of 38 months (rang
e, 35-49 months). GH secretion was analyzed by measurement of spontaneous o
vernight GH secretion and two standard stimulation tests. Auxological param
eters, bone age, serum levels of insulin-like growth factor I and insulin-l
ike 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 m
onths after the start of therapy (8.1 cm/yr). The growth velocity of arm sp
an was greater than these values. Supine length so score for chronological
age increased from -4.71 (at start) to -3.35 (after 36 months; P = NS), len
gth so score for hone age increased from -2.70 to -2.23 (P = NS), and arm s
pan so score increased from -2.98 to -1.75 (P < 0.05). The growth velocitie
s of length and arm span remained significantly above the pretreatment valu
es (P < 0.05). Symptomatic tethered cord associated with progression of sco
liosis developed in two of seven children.
GH treatment significantly improved the growth velocities of body length an
d arm span. However, the increase in length SD score was not significant, w
hereas arm span SD scores significantly improved over the study period.