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The urine of patients with proteinuria of various etiologies was examined to determine if proteinuria alone was associated with significant glycosphingolipiduria. In all cases of proteinuria examined, the level of glycosphingolipids in the urine was found to be markedly elevated. There was no evidence of a glycosphingolipid storage disorder in any case. It was concluded that significant glycosphingolipiduria may occur in proteinuria as well as in the glycosphingolipid storage disorders.
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