Septo-optic dysplasia/optic nerve hypoplasia: data from the National Cooperative Growth Study (NCGS).
We analyzed data from 65 children with septo-optic dysplasia (SOD) referred for evaluation and followed in the National Cooperative Growth Study (NCGS) Substudy 8 and from 758 children treated with growth hormone (GH) and followed in the NCGS core study. Compared to other children referred for evaluation of short stature, children with SOD were younger (mean age 3.7 +/- 3.6 vs 8.6 +/- 4.9 years), had less severe short stature (mean +/- SD height SDS -1.80 +/- 1.64 vs -2.17 +/- 0.95), and were more likely to be female (46% F vs 31% M). Children with SOD who received GH were older and shorter than those referred and untreated, but the gender distribution was similar. Other pituitary hormone deficits were reported in untreated patients, including thyroid hormone deficiencies (8%) and adrenocorticotropic hormone (ACTH) deficiency (3%), as compared to 27% and 24%, respectively, in GH-treated children. Data on adult height were available for 71 patients, who showed an average gain in height SDS of 1.17 +/- 1.49. GH therapy was well tolerated in children with SOD.
Parker
Katrina L
KL
Department of Pediatrics, University of Alabama at Birmingham, USA. kparker@peds.uab.edu
Hunold
Joanne Julius
JJ
Blethen
Sandra L
SL
D001827:Body Height / Q000187:drug effects; D001921:Brain / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006130:Growth Disorders / Q000188:drug therapy* / Q000473:pathology; D013006:Growth Hormone / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D009901:Optic Nerve Diseases / Q000188:drug therapy / Q000473:pathology*; D010907:Pituitary Hormones / Q000172:deficiency*; D011358:Product Surveillance, Postmarketing; D012727:Sex Characteristics; D013577:Syndrome
D010907:Pituitary Hormones; D013006:Growth Hormone