Lipid abnormalities in a variant of the Hurler syndrome.

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Abstract
In a study of 2 brothers with clinical evidence of Hurler''s syndrome, fibroblasts obtained from 1 of the brothers were strikingly abnormal but did not show typical metachromasia. After the specimens were treated with chloroform:methanol (2:1, v/v), metachromatic granules could be demonstrated, suggesting that excess lipids were present which interfered with staining. Details regarding the occurrence of abnormal quantities of certain lipids in fibroblasts of this patient as compared with the fibroblasts of a patient with typical Hurler''s disease and those of a normal child are reported.