Light- and electron-microscopic histochemistry of Fabry's disease

T. Faraggiana, J. Churg, E. Grishman, L. Strauss, A. Prado, D. F. Bishop, E. Schuchman, R. J. Desnick

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92 Scopus citations


A histochemical study was performed on light- and electron-microscopic level in a case of Fabry's disease. The patient underwent kidney transplantation for renal failure and died of heart failure 6 months later. Patient's tissues were studied at the light- and electron-microscopic levels with various embedding and staining techniques for lipids and carbohydrates. Two peroxidase-labeled lectins (from Ricinus communis and from Bandeiraea simplicifolia) known to have affinity for α- and β-D-galactose, were strongly reactive with the storage material on frozen sections. The ultrahistochemical and extraction tests showed that the typical granules had a variable reactivity and morphologic characteristics in different cells, probably reflecting different composition. A small number of typical deposits were also observed in the transplanted kidney. This is the first reported case of recurrence of the storage disease in the allograft. Of interest was also the fact that the patient's blood inhibited normal α-galactosidase activity, suggesting a possible inhibitor-related mechanism in the pathogenesis of the recurrence.

Original languageEnglish
Pages (from-to)247-262
Number of pages16
JournalAmerican Journal of Pathology
Issue number2
StatePublished - 1981


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