A- -lipoproteinaemia (Bassen-Kornzweig Syndrome): Report of a Case

Abstract
A 17-month-old Maori child with Bassen-Kornzweig syndrome is described. As well as showing acanthocytosis, a-[beta]-lipoproteinemia, hypocholesterolemia, and steatorrhea, he had incipient neuropathy. The child also had impairment of renal function, amino-aciduria, and suffered from recurrent pulmonary infections. None of these features appear to have been described in other patients with this disease and therefore they may be unrelated to the primary disorder.