title: Improved growth with growth hormone therapy in a child with glycogen storage disease Ib.
abstract: In type Ib glycogen storage disease (GSD) growth is typically affected and short stature is a common feature. Reported use and effect of growth hormone (GH) therapy in children with GSD is limited. We report on the use of substitutive GH treatment in a poorly growing adolescent female with GSD type Ib. The patient's growth velocity increased from a baseline level of 2.5 cm/y to an average growth velocity during GH therapy of 8.7 cm/y. During GH therapy this patient did not demonstrate metabolic decompensation but increased levels of cholesterol and triglycerides were seen (A-1).
  
  
  CONCLUSIONS
  Patients with GSD may experience an improvement in growth response with GH treatment. Prior to GH therapy, treatment of hyperlipidemia associated with GSD should allow the therapy to be safely tolerated.
authors.affiliation: Division of Pediatric Endocrinology, Metabolism and Nutrition, New York Medical College, Valhalla, New York 10595, USA.
authors.forename: R A
authors.initials: RA
authors.lastname: Noto
authors.forename: V
authors.initials: V
authors.lastname: Vijayaraghavan
authors.forename: A
authors.initials: A
authors.lastname: Timoshin
authors.forename: D
authors.initials: D
authors.lastname: Sansobrino
mesh_terms: D000293:Adolescent; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000188:drug therapy*; D006128:Growth; D006130:Growth Disorders / Q000188:drug therapy; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans
chemical_list: D019382:Human Growth Hormone