One-Year Treatment with Recombinant Human Growth Hormone of Children with Meningomyelocele and Growth Hormone Deficiency: A Comparison of Supine Length and Arm Span

Abstract

Growth retardation and precocious puberty are frequently found in children with meningomyelocele (MMC). Lower limb contractions, spasticity and kyphoscoliosis may lead to disproportionate short stature. Most of these patients have structural brain defects or hydrocephalus which can cause growth hormone deficiency. In this study, 19 children aged between 3.5 and 12.8 years with MMC and growth hormone (GH) deficiency were treated with recombinant human GH for a period of 12 months. Supine length, arm span and growth velocity were compared before, and after 6 and 12 months of treatment with rhGH (daily dose 2.0 IU/m2 BSA s.c.). Mean supine length standard deviation score (SDS) increased by +0.8 SDS after 6 months and +1.2 SDS after 12 months of therapy. Mean arm span standard deviation score increased by +0.9 SDS and +1.3 SDS. Growth velocity increased in supine length from 3.3 cm/yr (-2.1 SDS) to 8.4 cm/yr (+2.4 SDS) and in arm span from 4.8 cm/yr (-1.3 SDS) to 8.6 cm/yr (+3.1 SDS) in the first 6 months and was 8.1 cm/yr (+2.4 SDS) and 8.3 cm/yr (+2.6 SDS) after 12 months of therapy. Linear correlation between SDS growth velocity supine length and SDS growth velocity arm span during one year of treatment was excellent (r = 0.65, p < 0.0025). We surmise that body proportions do not deteriorate when growth velocity is stimulated in MMC patients. Both supine length and arm span measurements are necessary to document growth in children with spinal dysraphism.

Obesity and Myelomeningocele: Anthropometric Measures

“…Our data would support previously published reports (13)(14)(15)(16)(17)(18)(19) that arm span is a better parameter than height to calculate body functions for patients with myelomeningocele and scoliosis and/or hypoplastic lower extremities. For patients with myelomeningocele and with intermediate or minimal paralysis (LL2 and LL3) without scoliosis or lower extremity hypoplasia, either height or arm span can be reliably used to estimate BMI.…”

“…The arm span as a substitute for height method we propose is applicable to most clinics caring for patients in wheelchairs or for those where it is difficult to obtain height or linear length measures because of the patients' inability to stand, their scoliosis, or their lower extremity deformities (13)(14)(15)(16)(17)(18)(19). We have not used more stringent investigative measures (4-9,11) in our study because they are expensive or radioactive and not available in most clinic situations.…”

“…These children have a high incidence of decreased height or linear length secondary to kyphosis, scoliosis, abnormal vertebrae, and lower extremity hypoplasia associated with their myelomeningocele (3,4,6,(10)(11)(12). A number of studies have reported the value of using arm span as a substitute for estimating body functions (eg, pulmonary function, growth) and for calculating medication dosages for people with scoliosis or when height or length are difficult to obtain (13)(14)(15)(16)(17)(18)(19). Others have stated arm span is not a substitute for height in the evaluation of growth or BMI and is not optimal for evaluating lean body mass in patients with myelomeningocele or cerebral palsy (20)(21)(22).…”

Obesity across the lifespan among persons with spina bifida

“…Obesity rates among people with spina bifida have been assessed by a variety of methods [9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] and range from 28 to 50% among children to 34-64% among adolescents and adults [9,16,17,23,24]. The causes of obesity in this population are multifactorial.…”

Growth in pre-pubertal children with myelomeningocele (MMC) on growth hormone (GH): The KIGS experience

“…In 1989, Rotenstein et al [10] first reported on a positive short-term GH response in seven pre-pubertal MMC patients. However, there are still only few reports available documenting short-term treatment effects of hGH [11][12][13][14]. Since these first published reports, there is an ongoing discussion concerning long-term efficacy and safety.…”