title: Consensus guidelines for management of glycogen storage disease type 1b - European Study on Glycogen Storage Disease Type 1.
abstract: Life expectancy in glycogen storage disease type 1 (GSD-1) has improved considerably. Its relative rarity implies that no metabolic centre has experience of large series of patients and therefore experience with long-term management and follow-up at each centre is limited. There is wide variation in methods of dietary and pharmacological treatment. Based on data from the European Study on Glycogen Storage Disease Type 1, discussions within this study group together with those at the International SHS Symposium 'Glycogen Storage Disease Type I and II: Recent Developments, Management and Outcome', Fulda, Germany (2000) and on data from the literature, a series of guidelines were drawn up.
  
  
  CONCLUSIONS
  the following guidelines for the management of patients with glycogen storage disease type 1b are in addition to those general guidelines for glycogen storage disease type 1 and address specific problems related to neutropenia and neutrophil dysfunction.
authors.affiliation: Beatrix Children's Hospital, Groningen, The Netherlands. g.visser@oprit.rug.nl
authors.forename: Gepke
authors.initials: G
authors.lastname: Visser
authors.forename: Jan Peter
authors.initials: JP
authors.lastname: Rake
authors.forename: Philippe
authors.initials: P
authors.lastname: Labrune
authors.forename: James V
authors.initials: JV
authors.lastname: Leonard
authors.forename: Shimon
authors.initials: S
authors.lastname: Moses
authors.forename: Kurt
authors.initials: K
authors.lastname: Ullrich
authors.forename: Udo
authors.initials: U
authors.lastname: Wendel
authors.forename: G Peter A
authors.initials: GP
authors.lastname: Smit
mesh_terms: D005953:Glycogen Storage Disease Type I / Q000150:complications / Q000628:therapy*; D016179:Granulocyte Colony-Stimulating Factor / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans
chemical_list: D016179:Granulocyte Colony-Stimulating Factor