An autopsy case of I-cell disease. Ultrastructural and biochemical analyses.
An autopsy case of I-cell disease in a 4-year-old Japanese girl is presented. In this report, the authors analyze the relationship between morphologic (including electron microscopic) and biochemical findings. Lymph node, spleen, and kidney, which were stained with Hale's colloidal iron method, contained large amounts of hexosamine. These substances had accumulated in lymphocytes of B-cell lineage.
Kitagawa
H
H
First Department of Pathology, Kansai Medical University, Osaka, Japan.
Toki
J
J
Morimoto
T
T
Okumura
T
T
Saitoh
K
K
Higashino
H
H
Horii
T
T
Kobayashi
Y
Y
Ikehara
S
S
D002102:Cadaver; D002675:Child, Preschool; D005260:Female; D006595:Hexosamines / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D007668:Kidney / Q000378:metabolism; D008198:Lymph Nodes / Q000378:metabolism; D008214:Lymphocytes / Q000378:metabolism; D008854:Microscopy, Electron; D009081:Mucolipidoses / Q000378:metabolism / Q000473:pathology*; D013154:Spleen / Q000378:metabolism
D006595:Hexosamines