Abstract
An unusual opportunity to study the lipid composition of various tissues in an extremely rare condition, that of generalized lipodystrophy, was offered when death occurred in a 9-year-old boy in whom an almost complete absence of adipose tissue from the body had been present for the past 6 years (Case 1). In addition to this remarkable apparent lack of body fat, the symptom complex was composed of cirrhosis of the liver, chronic fibrosis of the spleen, pancreas and certain lymph nodes, and diabetes mellitus. Necropsy was begun within one hour following demise, at which time samples of various tissues were obtained. After being weighed, the specimens (usually about 1 g of tissue) were placed in 95% alcohol and allowed to stand for 24 hours. The tissues were then ground with sea sand in a mortar, rinsed several times with alcohol and ether, returned to the original flasks, and sufficient ether added to make approximately a 3:1 alcohol-ether mixture. The flasks were immersed in a boiling water bath for about 5 minutes and allowed to cool; the contents were filtered through fat-free filter paper into volumetric flasks, brought to volume, and stored in a refrigerator until analyses were made. Aliquots were measured and the following procedures employed: The method of Wilson and Hansen 1 was used for the determination of the unsaponifiable and saponi-fiable fractions, while the technic followed by Hansen 2 was used for the determination of the fatty acids in the acetone-insoluble (phospholipid) fraction and the acetone-soluble (cholesterol ester-neutral fat) fraction. The total cholesterol and cholesterol esters were determined by the procedure described by Bloor, 3 4 the photoelectric colorimeter being used in obtaining the final readings. For the control studies, similar tissues from a 14-vear-old boy dying in uremia from subacute nephritis (Case 2), the best available material at the time, were used.
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