Abstract

Summary Sialidosis is characterized by the Hurler-like clinical pictures, progressive neurodegenerative course and an increase in sialic acid-rich saccharides in urine. However, it looks a heterogenous category consisted of several clinical disorders. Recently it has been established that acid neuraminidase is deficient primarily in this disease. We reviewed sialidosis and discussed biochemical characteristics including coexisting β-galactosidase abnormality.

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