1 Department of Pediatrics
2 Department of Biochemistry, Asaf Harofe Government Hospital, Tel-Aviv University Medical School Zerifin
3 Department of Chemical Pathology, Tel-Aviv University Medical School, Tel-Hashomer, Israel
Acanthocytosis or a-beta-lipoproteinemia is a rare inborn error of metabolism known also as the "Bassen Kornzweig syndrome."1 We had
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the opportunity to examine a Jewish girl, H.F., 11 years old, with a-beta-lipoproteinemia. The girl has healthy parents of Turkish origin who are first cousins, and a healthy brother and sister. At the age of 33
months she was admitted to this hospital with a picture of malabsorption and from then on she was under our permanent observation.