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Published on: January 7, 2016
Does growth hormone (GH) enhance growth in GH-deficient children with myelomeningocele?
R Trollmann1, E Strehl, D Wenzel
1Hospital for Children and Adolescents, University Erlangen, Germany.
Insights
Growth hormone (GH) treatment improved growth velocity in children with myelomeningocele and GH deficiency. While body length showed increased growth rates, final height SD scores were not significantly improved, though arm span improved.
Area of Science:
- Pediatric Endocrinology
- Developmental Biology
- Clinical Genetics
Background:
- Growth hormone deficiency (GHD) in patients with myelomeningocele raises questions about the efficacy and safety of human GH treatment.
- Limited short-term data exist, with a lack of long-term final height outcomes in this specific patient population.
Purpose of the Study:
- To evaluate the auxological and laboratory effects of long-term human GH treatment in prepubertal children with myelomeningocele and proven GHD.
- To assess changes in growth velocity, standard deviation scores for length and arm span, and relevant biochemical markers.
Main Methods:
- Seven prepubertal patients with myelomeningocele and GHD received daily subcutaneous GH injections (0.15 mg/kg/week) for a median of 38 months.
- Evaluated auxological parameters, bone age, GH secretion, insulin-like growth factor I (IGF-I), IGF-binding protein-3 (IGFBP-3), and neurological/orthopedic status.
- Compared growth parameters before and during GH therapy, including growth velocity and SD scores.
Main Results:
- Median growth velocity of supine length significantly improved from 3.7 cm/yr to 5.7 cm/yr after 36 months (P < 0.05).
- Arm span SD scores significantly increased from -2.98 to -1.75 (P < 0.05), while length SD scores showed non-significant improvements.
- Two patients developed symptomatic tethered cord with progressive scoliosis.
Conclusions:
- Human GH treatment significantly enhances growth velocity in children with myelomeningocele and GHD.
- While arm span shows significant improvement, the impact on final length SD score requires further investigation.
- Potential risks such as tethered cord progression necessitate careful monitoring during GH therapy.
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.
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