Consensus guidelines for management of glycogen storage disease type 1b - European Study on Glycogen Storage Disease Type 1.
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.
Visser
Gepke
G
Beatrix Children's Hospital, Groningen, The Netherlands. g.visser@oprit.rug.nl
Rake
Jan Peter
JP
Labrune
Philippe
P
Leonard
James V
JV
Moses
Shimon
S
Ullrich
Kurt
K
Wendel
Udo
U
Smit
G Peter A
GP
D005953:Glycogen Storage Disease Type I / Q000150:complications / Q000628:therapy*; D016179:Granulocyte Colony-Stimulating Factor / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans
D016179:Granulocyte Colony-Stimulating Factor