Biochemical and clinical profiles of 52 Tunisian patients affected by Zellweger syndrome

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MR of Zellweger syndrome.

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Zellweger syndrome (cerebro-hepato-renal syndrome).

A syndrome with distinctive clinical features affecting brain, liver and kidneys was described by Bowen et al in 1964 1 and Smith et al. in 1965 2 . In 1973 Goldfisher et al 3 has reported that peroxisomes were absent in the liver and kidneys of affected children. More recently lack of dihydroxyacetone phosphate acyletransferase (DHAP-AT) a peroxisomal enzyme with a major role in glycerol ether...

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ژورنال

عنوان ژورنال: Pediatrics & Neonatology

سال: 2017

ISSN: 1875-9572

DOI: 10.1016/j.pedneo.2016.08.011