TREATMENT WITH LENOGRASTIM (GLYCOSYLATED RECOMBINANT HUMAN GRANULOCYTE-COLONY-STIMULATING FACTOR) AND ORTHOTOPIC LIVER-TRANSPLANTATION FOR GLYCOGEN-STORAGE-DISEASE TYPE-IB

Citation
A. Lachaux et al., TREATMENT WITH LENOGRASTIM (GLYCOSYLATED RECOMBINANT HUMAN GRANULOCYTE-COLONY-STIMULATING FACTOR) AND ORTHOTOPIC LIVER-TRANSPLANTATION FOR GLYCOGEN-STORAGE-DISEASE TYPE-IB, The Journal of pediatrics, 123(6), 1993, pp. 1005-1008
Citations number
10
Categorie Soggetti
Pediatrics
Journal title
ISSN journal
00223476
Volume
123
Issue
6
Year of publication
1993
Pages
1005 - 1008
Database
ISI
SICI code
0022-3476(1993)123:6<1005:TWL(RH>2.0.ZU;2-2
Abstract
A 7 1/2-year-old patient with glycogenosis type lb had chronic neutrop enia, 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 p latelet counts rapidly reached normal values, and infectious episodes were reduced. She resumed oral feeding after transplantation and had n o subsequent hypoglycemic episodes.