Treatment with lenograstim (glycosylated recombinant human granulocyte colony-stimulating factor) and orthotopic liver transplantation for glycogen storage disease type Ib.
A 7 1/2-year-old patient with glycogenosis type lb had chronic neutropenia, recurrent bacterial infections, and severe hypoglycemia in spite of continuous nasogastric feeding. She was treated with lenograstim (glycosylated recombinant human granulocyte colony-stimulating factor) and orthotopic liver transplantation. Absolute neutrophil counts and platelet counts rapidly reached normal values, and infectious episodes were reduced. She resumed oral feeding after transplantation and had no subsequent hypoglycemic episodes.
Lachaux
A
A
Department of Pediatrics, Liver Transplantation Unit, Hôpital E. Herriott, Lyon, France.
Boillot
O
O
Stamm
D
D
Canterino
I
I
Dumontet
C
C
Regnier
F
F
Floret
D
D
Hermier
M
M
D000276:Adjuvants, Immunologic / Q000627:therapeutic use*; D002648:Child; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000601:surgery / Q000628:therapy*; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D000078224:Lenograstim; D016031:Liver Transplantation; D011994:Recombinant Proteins / Q000627:therapeutic use
D000276:Adjuvants, Immunologic; D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor; D000078224:Lenograstim