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Int J Pediatr Endocrinol. 2011 Nov 15;2011(1):17. doi: 10.1186/1687-9856-2011-17.

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

International journal of pediatric endocrinology

Amy M Vedin, Hanna Karlsson, Cassandra Fink, Mark Borchert, Mitchell E Geffner

Affiliations

  1. Center for Endocrinology, Diabetes, and Metabolism, Children's Hospital Los Angeles, 4650 Sunset Boulevard, Mailstop #61, Los Angeles, CA 90027, USA. [email protected].

PMID: 22085631 PMCID: PMC3256097 DOI: 10.1186/1687-9856-2011-17

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.

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