title: Growth Hormone Excess in Children with Optic Pathway Tumors Is a Transient Phenomenon.
abstract: OBJECTIVE
  Growth hormone (GH) excess in children with chiasmal optic pathway tumors (OPT), often associated with neurofibromatosis type 1 (NF1), is likely underrecognized. These children have elevated insulin-like growth factor 1 (IGF-1) levels, evidence of rapid growth despite treatment of precocious puberty, and failure to suppress GH levels following oral glucose challenge. The aim of this report is to describe the treatment course and natural history of this rare clinical condition in 7 patients.
  
  
  METHODS
  This is a descriptive case series of 5 children previously described and 2 additional children more recently diagnosed at our institution. All 7 children had clinical and biochemical evidence of GH excess and received treatment with the somatostatin analog octreotide.
  
  
  RESULTS
  Length of treatment varied among the patients. Five of the 7 patients have had resolution of GH excess and currently have normal IGF-1 levels without treatment.
  
  
  CONCLUSIONS
  Unrestrained GH secretion occurs in a subset of children with OPT with potential adverse outcomes. Since GH excess appears to resolve over time, the benefit of treatment to alter outcomes or prevent tumor progression is unclear.
authors.affiliation: Division of Endocrinology, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Ill., USA.
authors.forename: Jami L
authors.initials: JL
authors.lastname: Josefson
authors.forename: Robert
authors.initials: R
authors.lastname: Listernick
authors.forename: Joel
authors.initials: J
authors.lastname: Charrow
authors.forename: Reema L
authors.initials: RL
authors.lastname: Habiby
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D019382:Human Growth Hormone / Q000097:blood*; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D008297:Male; D009456:Neurofibromatosis 1 / Q000097:blood* / Q000628:therapy; D019574:Optic Nerve Neoplasms / Q000097:blood* / Q000628:therapy
chemical_list: D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I