Presenting features and long-term effects of growth hormone treatment of children with optic nerve hypoplasia/septo-optic dysplasia.

Abstract

BACKGROUND:

Optic nerve hypoplasia (ONH) with/or without septo-optic dysplasia (SOD) is a known concomitant of congenital growth hormone deficiency (CGHD).

METHODS:

Demographic 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.

RESULTS:

ONH/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.

CONCLUSIONS:

Compared 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.

• Authors: Amy M Vedin, Hanna Karlsson, Cassandra Fink, Mark Borchert, Mitchell E Geffner
• Journal: International journal of pediatric endocrinology (Int J Pediatr Endocrinol) Vol. 2011 Issue 1 Pg. 17 (Nov 15 2011) ISSN: 1687-9856 [Electronic] England
• PMID: 22085631 (Publication Type: Journal Article)