Abstract
BackgroundOptic nerve hypoplasia (ONH) with/or without septo-optic dysplasia (SOD) is a known concomitant of congenital growth hormone deficiency (CGHD).MethodsDemographic and longitudinal data from KIGS, the Pfizer International Growth Database, were compared between 395 subjects with ONH/SOD and CGHD and 158 controls with CGHD without midline pathology.ResultsONH/SOD subjects had higher birth length/weight, and mid-parental height SDS. At GH start, height, weight, and BMI SDS were higher in the ONH/SOD group. After 1 year of GH, both groups showed similar changes in height SDS, while weight and BMI SDS remained higher in the ONH/SOD group. The initial height responses of the two groups were similar to those predicted using the KIGS-derived prediction model for children with idiopathic GHD. At near-adult height, ONH/SOD and controls had similar height, weight, and BMI SDS.ConclusionsCompared to children with CGHD without midline defects, those with ONH/SOD presented with greater height, weight, and BMI SDS. These differences persisted at 1 year of GH therapy, but appeared to be overcome by long-term GH treatment.
Highlights
Optic nerve hypoplasia (ONH) with/or without septo-optic dysplasia (SOD) is a known concomitant of congenital growth hormone deficiency (CGHD)
In a study of 47 subjects [age 15.2 ± 10.6 months] with ONH followed until 59.0 ± 6.2 months of age, Ahmad, et al reported a prevalence of endocrinopathies of 71.7%; these were not associated with ONH laterality, absence of septum pellucidum, or pituitary abnormalities on neuro-imaging [2]
The birth length and weight standard deviation scores (SDS) were significantly greater in the ONH/SOD group compared to the non-ONH/SOD CGHD group [birth length SDS median (0.27); 10th and 90th percentiles (-1.3, 2.0) vs. median (-0.62); 10th and 90th percentiles (-2.5, 1.2); p < 0.001) and birth weight SDS (-0.31; -1.7, 1.1) vs. (-0.58; -2.0, 1.0); p = 0.021)]
Summary
Optic nerve hypoplasia (ONH) with/or without septo-optic dysplasia (SOD) is a known concomitant of congenital growth hormone deficiency (CGHD). The only prior large, More recently, it has been suggested that obesity is a frequent occurrence in children with ONH perhaps on a hypothalamic basis [5,6] In his cohort of 47 subjects with ONH, Ahmad found that 44% had a body mass index (BMI) > 85th percentile at 5 years of age [2]. The ONH/ SOD group continued to have a significantly higher height SDS, weight SDS, and BMI SDS after one year of GH therapy (all p < 0.001). Use of the prediction model for first-year growth response in children with idiopathic GHD showed that, there was a slightly greater than predicted response in the ONH/SOD group versus the non-ONH/SOD CGHD group, there was no difference in actual height response between both groups, with Studentized residuals equivalent in both groups (Figure 2). At NAH, the two groups had the same prevalence of hypogonadism
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