MR imaging of bone marrow in glycogen storage disease type IB in children and young adults.
OBJECTIVE
Patients with glycogen storage disease type IB have neutropenia and neutrophil dysfunction that predispose them to frequent infections, for which they are given granulocyte colony--stimulating factor. Because neutropenia is a consequence of defects in myeloid maturation, the bone marrow aspirations show hypercellularity due to myeloid hyperplasia. This study evaluated MR imaging of bone marrow in glycogen storage disease type IB with and without granulocyte colony-stimulating factor.


CONCLUSIONS
As confirmed by the histologic results in bone marrow aspirations, abnormal findings on MR images of bone marrow in patients with glycogen storage disease type IB indicate an increased myelopoietic activity, which is augmented by treatment with granulocyte colony-stimulating factor.
Scherer
A
A
Institute of Diagnostic Radiology, Heinrich-Heine University, Moorenstr. 5, D-40225 Düsseldorf, Germany.
Engelbrecht
V
V
Neises
G
G
May
P
P
Balsam
A
A
Spiekerkötter
U
U
Wendel
U
U
Mödder
U
U
D000293:Adolescent; D000328:Adult; D001853:Bone Marrow / Q000473:pathology*; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000188:drug therapy / Q000473:pathology*; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D019891:Leukopoiesis; D008279:Magnetic Resonance Imaging; D008297:Male; D009504:Neutrophils; D013997:Time Factors
D016179:Granulocyte Colony-Stimulating Factor