Hereditary phytosterolaemia

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Phytosterolaemia in three unrelated South African families.

Phytosterolaemia (beta-sitosterolaemia), a rare, autosomal recessive disorder, has not hitherto been reported in Southern Africa. We report four new homozygous patients, from three unrelated families with significant beta-sitosterolaemia (6.6-11.3%), campesterolaemia (2.2-4.6%) and clearly detectable, though unquantified, levels of cholestanol. Three of the four patients had characteristic cuta...

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ژورنال

عنوان ژورنال: British Journal of Haematology

سال: 2019

ISSN: 0007-1048,1365-2141

DOI: 10.1111/bjh.16076