Biomedical subjects
Publications and source records attributed to R D Ellefson.
A study of 103 healthy, white schoolchildren who were between 6 and 16 years of age revealed no positive correlation between the level of serum cholesterol and the mean daily amount of total calories, cholesterol, fat, saturated fat, or sugar in the diet.
The non-polar lipids from sera of 54 patients, with various types of hereditary motor and sensory neuropathies, and from 72 healthy subjects were evaluated. A small but highly significant decrease in the percentage of linoleate to total fatty acids in both cholesteryl ester and triglyceride fractions was found in the sera of the neuropathy patients, except in those who had dominantly inherited sensory neuropathy (HSN-I) and who had spinocerebellar degeneration with retinitis pigmentosa and other features (SpC+). A significant decrease of serum lecithin-cholesterol acyltransferase activity was also found in those patients with hereditary motor and sensory neuropathies, Type I and Type II (two types of peroneal muscular atrophy). The biochemical basis of these abnormalities is not apparent. The biochemical abnormalities reported here have been found in several neurologic disorders and hence are unlikely to be disease-specific.
The long-term effects of milk diets in infancy on the blood serum cholesterol concentrations were studied in 97 school children 7-12 yr of age. Detailed histories were available for these children with respect to their diets during infancy. The major criterion for inclusion in the study was milk (human milk, cow's milk, or commercial formula) as the exclusive source of diet cholesterol during the first 3 mo of life. Fasting blood cholesterol and triglycerides were measured in these 97 school children, and the current diets of 29 of the 97 were evaluated for daily cholesterol intake. A 7-day diet diary was recorded, the food intake was measured and analyzed for nutrients, and validity of the diets was verified by determination of urinary nitrogen excretion. Results of the study showed that the school children fed a low cholesterol formula during infancy had a mean serum cholesterol value 7-12 yr later that was lower than the mean values of the groups fed greater amounts of cholesterol in infancy. Analysis of current diets of 29 of the 97 school children showed that their current dietary intake of cholesterol was low. Dietary intake of cholesterol did not have a noticeable effect on the serum cholesterol levels of the 29 children.
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Liver specimens from 8 patients with Reye-Johnson syndrome, 2 patients with acute encephalopathy, and 10 patients without liver disease were analyzed for aflatoxins. An aflatoxin was obtained from the liver of one patient, a 15-year-old girl who had clinical, laboratory, and pathologic features of Reye-Johnson syndrome. Thin-layer chromatography, fluorescence emission, infrared spectroscopy, and derivatization studies characterized the aflatoxin as related structurally to but not identical with aflatoxin B1. This report is added to previous ones from Thailand, New Zealand, and Czechoslovakia in which the association of aflatoxins and fatty liver has been found in Reye-Johnson syndrome.
The relationship between Reye-Johnson syndrome and acute encephalopathy without fatty liver was investigated by comparing the lipid composition of liver samples obtained from five patients with Reye-Johnson syndrome, two patients with acute encephalopathy, and five controls. The mean total hepatic triglyceride concentration was increased nearly sevenfold in Reye-Johnson syndrome and slightly decreased in acute encephalopathy when compared with the mean control value. The mean total hepatic free fatty acid concentration was increased nearly threefold in acute encephalopathy when compared with the mean value in Reye-Johnson syndrome. Total phospholipid content was decreased in the liver in Reye-Johnson syndrome, and this difference was caused mainly by a diminution of the hepatic lecithin fraction. The ratio of palmitic acid to oleic acid and hepatic free fatty acids was 2.5 in Reye-Johnson syndrome, 0.7 in acute encephalopathy, and 0.8 in controls. These results suggest that, despite clinical similarities and laboratory evidence of hepatic dysfunction in both Reye-Johnson syndrome and acute encephalopathy, different pathogenic mechanisms may be responsible for the liver abnormalities found in the two syndromes.
Lipoproteins and lipoprotein profiles were determined in 96 adult nephrotic patients. The serum cholesterol-serum albumin, serum triglycerides-serum albumin and 24-hour urine protein loss-serum albumin values were all significantly inversely correlated. The serum triglycerides and serum cholesterol levels were not significantly lower in the group of lupus nephrotic patients compared to the nonlupus nephrotics. All lipoprotein types except type I were observed. The lipoprotein types fell into three nearly equal groups--IIa, IIb, and V. Type IV, the most common lipoprotein abnormality in uremic patients, was distinctly uncommon.
A newly developed radioimmunoassay specific for conjugates of cholic acid (CCA) was used to measure the fasting serum levels of these bile acids in 233 patients with primary hyperlipoproteinemia classified as type IIa, IIb, III, IV, or V hyperlipoproteinemia as well as in 80 healthy control subjects. Subjects with type IIa hyperlipoproteinemia had significantly lower levels of CCA (0.9 plus or minus 0.06 muM, mean plus or minus SE) than did healthy controls (0.50 plus or minus 0.08 muM). Patients with type IIb, III, IV, and V hyperlipoproteinemia had values similar to those of the control group. The abnormally low fasting value of CCA in type IIa patients is considered to reflect decreased secretion of bile acid into the intestine in such patients, who are known to have defective bile acid synthesis rates and decreased bile acid pool sizes.
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Comparison of the cellular fatty acids of 10 strains of Mycobacterium marinum and 35 strains of Mycobacterium kansasii revealed similarities within each species but differences between these two photochromogenic mycobacteria. A branched-chain fatty acid characteristic of M. kansasii was found in trace amounts in 2 of the 10 strains of M. marinum.
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