Two cases of Tangier disease are described in children from families unrelated to each other. Necropsy in one case, the first to be reported in this condition, showed large collections of cholesterol-laden macrophages in tonsils, thymus, lymph nodes, and colon, and moderate numbers in pyelonephritic scars and ureter. As the storage cells may be scanty in marrow, jejunum, and liver, the rectum is suggested as the site of choice for biopsy.The diagnosis was confirmed by demonstrating the absence of α-lipoproteins from the plasma of the living child, and by finding low plasma levels in both parents of both cases. The disease can be distinguished from other lipidoses by differences in the predominant sites of storage, staining reactions, and serum lipid studies.
As a guide to physicians, patients, and potential patients concerned with the prospect and practicalities of changing the diet to lower the blood cholesterol in the hope of preventing disease, estimates have been made of the importance of various items of food which figure prominently in relation to this topic. The measure of importance adopted is the predicted average servings of these foods, the prediction being based on the content of fatty acids and cholesterol. The major contributors, each adding more than 10 mg/100 ml to the plasma level, include brains, double servings of meat, one egg per day, and butter. At the other extreme, polyunsaturated oils and margarines actively lower plasma cholesterol while oysters, skim milk, and nuts have practically no effect. The relative importance of items, includeing the newer polyunsaturated ruminant meats and other products, has been charted for easy reference. The culinary costs involved in a cholesterol-lowering diet, considered not in monetary terms but as debits and credits in the food statement, must be balances against the potential benefits to be gained from disease prevention.
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