Familial Lecithin: Cholesterol Acyltransferase Deficiency: Studies on Lipid Composition and Morphology of Tissues

K. T. Stokke Institute of Clinical Biochemistry, Medical Dept. A, Institute of Pathology, Section B, and Surgical Dept. B, Rikshospitalet, Oslo; Institute for Nutrition Research, School of Medicine, University of Oslo, Oslo, Norway

K. S. Bjerve Institute of Clinical Biochemistry, Medical Dept. A, Institute of Pathology, Section B, and Surgical Dept. B, Rikshospitalet, Oslo; Institute for Nutrition Research, School of Medicine, University of Oslo, Oslo, Norway

J. P. Blomhoff Institute of Clinical Biochemistry, Medical Dept. A, Institute of Pathology, Section B, and Surgical Dept. B, Rikshospitalet, Oslo; Institute for Nutrition Research, School of Medicine, University of Oslo, Oslo, Norway

B. Öystese Institute of Clinical Biochemistry, Medical Dept. A, Institute of Pathology, Section B, and Surgical Dept. B, Rikshospitalet, Oslo; Institute for Nutrition Research, School of Medicine, University of Oslo, Oslo, Norway

A. Flatmark Institute of Clinical Biochemistry, Medical Dept. A, Institute of Pathology, Section B, and Surgical Dept. B, Rikshospitalet, Oslo; Institute for Nutrition Research, School of Medicine, University of Oslo, Oslo, Norway

K. R. Norum Institute of Clinical Biochemistry, Medical Dept. A, Institute of Pathology, Section B, and Surgical Dept. B, Rikshospitalet, Oslo; Institute for Nutrition Research, School of Medicine, University of Oslo, Oslo, Norway

E. Gjone Institute of Clinical Biochemistry, Medical Dept. A, Institute of Pathology, Section B, and Surgical Dept. B, Rikshospitalet, Oslo; Institute for Nutrition Research, School of Medicine, University of Oslo, Oslo, Norway

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Abstract

Kidney transplantation was performed on a patient with familial LCAT deficiency. She died 5 months later from various infections. Lipid deposition was demonstrated in spleen, liver, and kidney, accumulation of unesterified cholesterol being most marked. The lipid composition of an atheroma indicated that lipoproteins relatively rich in free cholesterol and in saturated and monounsaturated fatty acids had been filtered from the plasma to the atheromatous lesion. The half-life of the plasma esterifying activity in this patient was 4.6 days.

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Familial Lecithin: Cholesterol Acyltransferase Deficiency: Studies on Lipid Composition and Morphology of Tissues

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Familial Lecithin: Cholesterol Acyltransferase Deficiency: Studies on Lipid Composition and Morphology of Tissues

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