LIGHT-MICROSCOPIC AND ELECTRON-MICROSCOPIC HISTOCHEMISTRY OF FABRYS-DISEASE

  • 1 January 1981
    • journal article
    • research article
    • Vol. 103  (2) , 247-262
Abstract
A histochemical study was performed on EM and light microscopic level in a case of Fabry''s disease. The patient underwent kidney transplantation for renal failure and died of heart failure 6 mo. later. Patient''s tissues were studied at the EM and light microscopic levels with various embedding and staining techniques for lipids and carbohydrates. Two peroxidase-labeled lectins (from Ricinus communis and Bandeiraea simplicifolia) with affinity for .alpha.- and .beta.-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 1st reported case of recurrence of the storage disease in the allograft. Of interest also was the fact that the patient''s blood inhibited normal .alpha.-galactosidase activity, suggesting a possible inhibitor-related mechanism in the pathogenesis of the recurrence.