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We studied a case of Wolman's disease that is apparently the first to be reported in a black infant. Culture of the skin fibroblasts showed massive accumulation of neutral lipids, accompanied by decreased esterolytic activity as measured by fluorometric assay. Electrophoresis showed decreased activity of an esterase allotype, with low electrophoretic mobility.

The nephrotic syndrome developed in a patient receiving therapy with gold for rheumatoid arthritis. The results of a histopathological examination of the renal biopsy specimen were unremarkable. Immunofluorescent studies showed deposits of immunoglobulins and C3 in a granular pattern in the glomerular basement membranes. Ultrastructurally, the discrete osmiophilic immune complexes were epimembranous. By x-ray microanalysis, gold that was complexed with sulfur was present in proximal tubular cytoplasmic vacuoles and nuclei. Gold and sulfur could not be demonstrated in glomerular epimembranous deposits. The results of these studies suggest that immune complex deposition does not involve gold and sulfur acting as haptens. Gold-salt therapy may result in damage to proximal tubules that leak renal tubular antigens, which in turn complex with autoantibody and produce an autoimmune membranous nephropathy. The evidence for this mechanism is not convincing. Although the data indicate an immune-complex cause for gold-salt nephropathy, the incident antigen (or antigens) and mechanism of action remain unidentified.

Study of 115 kidneys from 60 patients with chronic renal failure maintained by dialysis for two months to five years revealed an unexpected number and variety of epithelial proliferative processes, several types of which are hitherto unreported. Proliferative activity was defined either by the presence of epithelial structures in ectopic situations, continuity with existing structures being demonstrable by serial sections, or by mitotic figures, or by both. The tendency for renal carcinoma development may relate to these dialysis-related epithelial proliferations originating in both glomerular and tubular epithelia. Enhanced renal epithelial proliferative capacity in dialysis may be employable in the experimental study of renal regeneration and in the therapy of patients with preterminal renal disease.

A 30-month-old girl exhibited the 19th known case of type IV glycogenosis. Extensive involvement of the nervous system was found at autopsy. This represents only the second patient in whom the fine structure of the CNS and skeletal muscle has been described. We have also identified the abnormal polysaccharide in peripheral nerve, a finding that, to our knowledge, has not been reported previously. Our review of the literature indicates that approximately 50% of these patients exhibit signs or symptoms referable to the neuromuscular system. Most clinical and pathologic studies have focused on the severe liver involvement; insufficient attention has been directed toward the nervous system. This emphasizes the need for more detailed neurologic and neuropathologic examinations of children with type IV glycogenosis.