title: 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.affiliation: Center for Endocrinology, Diabetes, and Metabolism, Children's Hospital Los Angeles, 4650 Sunset Boulevard, Mailstop #61, Los Angeles, CA 90027, USA. avedin@chla.usc.edu.
authors.forename: Amy M
authors.initials: AM
authors.lastname: Vedin
authors.forename: Hanna
authors.initials: H
authors.lastname: Karlsson
authors.forename: Cassandra
authors.initials: C
authors.lastname: Fink
authors.forename: Mark
authors.initials: M
authors.lastname: Borchert
authors.forename: Mitchell E
authors.initials: ME
authors.lastname: Geffner
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