Glycogen storage disease type Ib: infectious complications and measures for prevention.
A patient with glycogen storage disease (GSD) type Ib, neutrophenia, chronic inflammatory bowel disease and recurrent abscesses was treated with recombinant human granulocyte-macrophage colony stimulating factor (GM-CSF). GM-CSF (and also granulocyte colony stimulating factor) therapy markedly increased the neutrophil counts and reduced the frequency of infections and inflammation. We conclude that myeloid growth factors are effective for the treatment and prevention of acute infections and chronic inflammatory complications in patients with GSD Ib.
Wendel
U
U
University Children's Hospital, Düsseldorf, Federal Republic of Germany.
Schroten
H
H
Burdach
S
S
Wahn
V
V
D000038:Abscess / Q000209:etiology / Q000517:prevention & control*; D000293:Adolescent; D001004:Anus Diseases / Q000209:etiology / Q000517:prevention & control*; D002908:Chronic Disease; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000150:complications; D009059:Mouth Diseases / Q000209:etiology / Q000517:prevention & control*; D009503:Neutropenia / Q000209:etiology / Q000517:prevention & control*; D011994:Recombinant Proteins / Q000627:therapeutic use
D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor; D016178:Granulocyte-Macrophage Colony-Stimulating Factor