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ACANTHOCYTOSIS WITH BETA–LIPOPROTEIN DEFICIENCY IN AN INDIAN GIRL

An Indian infant was found to have acanthocytes in her blood with low serum concentrations of cholesterol and beta–lipoproteins, and other clinical and biochemical features of the Bassen–Kornzweig syndrome. Some members of her immediate family were also investigated. The findings are discussed in the light of the concept that this genetic disorder is one of variable expression and may be more common than previously thought. Several dietary oils were analysed by gas chromatography to assess their fatty acid structure. The customary use by the patient of coconut oil, which contains a fair amount of medium‐chain triglycerides, may explain the mildness of the disease.