An autopsy case of I-cell disease. Ultrastructural and biochemical analyses.

Kitagawa, H; Toki, J; Morimoto, T; Okumura, T; Saitoh, K; Higashino, H; Horii, T; Kobayashi, Y et al. · Am J Clin Pathol · 1991

case_report · Level V

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Abstract

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.

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