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