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Biomedical subjects

S Margolis

Publications and source records attributed to S Margolis.

At least 37 records · Page 2Linked to original sources

The golden tapetal sheen reflex in retinal disease.

A mother and son with dominant cone dystrophy manifested the retinal reflexes seen in Oguchi's disease (mother) and the carrier female of X-linked retinitis pigmentosa (son). Another patient with cone dystrophy (simplex) showed localized areas of a golden reflex in each eye. A patient with juvenile macular dystrophy exhibited a diffuse golden-orange reflex throughout the posterior pole. The latter two patients did not have the Mizuo phenomenon.

Adult↗

Nutritional management of plasma lipid disorders.

Prevention of vascular disease and acute pancreatitis is the goal of hyperlipidemia treatment. The risk of coronary heart disease (CHD) increases with increasing plasma cholesterol levels because low-density lipoprotein (LDL), the major carrier of cholesterol in the plasma, is atherogenic. High-density lipoprotein (HDL), especially the HDL2 subfraction, protects against CHD. Hypertriglyceridemia, although not an independent risk factor for CHD, is generally accompanied by low HDL cholesterol (HDLch), which may predispose to CHD. Reducing plasma LDL and raising HDL levels are thus goals in preventing CHD. Serum LDL levels may be lowered by reducing saturated fat and cholesterol intake; weight loss may decrease LDL but is more effective in lowering plasma triglycerides and raising HDLch. The percent of total calories from polyunsaturated, monounsaturated, and saturated fats should be less than 10%, up to 10-15%, and less than 10%, respectively. High cholesterol intake increases the flux of cholesterol, which may be harmful to arterial walls, but beyond a certain point does not increase plasma cholesterol levels. Some diets change the composition rather than the level of LDL and apoproteins. Weight reduction and maintenance are the most effective dietary measures to lower plasma triglycerides; omega-3 fatty acids (fish oils) have shown promise in reducing triglyceride but not cholesterol levels. Substitution of starch for sugar lowered triglyceride levels toward normal in hypertriglyceridemia patients. Fasting triglyceride levels rise in all individuals fed high-carbohydrate diets, but the high levels persist in hypertriglyceridemia patients. Weight loss, cessation of cigarette smoking, increased physical activity, good control of diabetes, and moderate alcohol use all raise HDLch levels. Vitamin E deficiency causes neurological sequelae in children with severe malabsorption problems due to abetalipoproteinemia or cholestatic liver disease.

Acute Disease↗

Improved glycemic control lowers plasma apoprotein E and triglyceride levels following ingestion of a fat load in insulin-dependent diabetic subjects.

To assess the effect of glycemic control on triglyceride (TG) and apoprotein E (apo E) metabolism, plasma levels of TG and apo E were studied in nine nonobese subjects with insulin-dependent diabetes mellitus (IDDM) following acute ingestion of polyunsaturated fat. Each subject was studied twice: before and after ten days of continuous subcutaneous insulin infusion (CSII). Each subject ingested identical meals on both study days. Plasma glucose was determined in all patients before and two hours after each meal and at 3 AM, and a mean value was calculated for each patient. CSII reduced mean plasma glucose from 205 to 113 mg/dL (P less than .005, paired t test); there was no change in the total daily insulin dose. Plasma TG and apo E levels were measured before and 3.5, 5, and 7 hours after a breakfast which contained 50 g of fish oil (five subjects) or vegetable oil (four subjects). A repeated-measures ANOVA was performed to assess the effects of the following three factors on plasma TG and apoE levels: type of oil ingested (Oil, factor A), glycemic control (Glycemic control, factor B), and the response to fat ingestion over time (Times, factor C). Plasma levels of both apo E and TG increased significantly after fat ingestion (F test, ANOVA, P less than .005 and P less than .001, respectively). Glycemic control significantly reduced the rise in both apo E and TG levels (P less than .005 and P less than .05, respectively). The effect of the type of oil and the interactions tested (AB, AC, BC, ABC) were not statistically significant.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Effect of improved glycemic control on the response of plasma triglycerides to ingestion of a saturated fat load in normotriglyceridemic and hypertriglyceridemic diabetic subjects.

This study tests the hypothesis that improved glycemic control decreases the postprandial plasma triglyceride (TG) response to ingestion of a saturated fat load. Fifteen normotriglyceridemic subjects with insulin-dependent diabetes mellitus (IDDM, group I) and six hypertriglyceridemic subjects with non-insulin-dependent diabetes mellitus (NIDDM, group II) were studied. Each subject was studied before and after 12 days of continuous subcutaneous insulin infusion (CSII). Each subject ingested identical meals on both study days. Plasma glucose was determined in all patients before and two hours after each meal and at 3 AM, and a mean value was calculated for each patient. CSII reduced mean plasma glucose from 252 to 140 mg/dL in group I, and from 209 to 120 mg/dL in group II (P less than .001 in both groups, paired t test). Plasma TG levels were measured before and 1.5, 3, 4.5, 6, and 7.5 hours after a breakfast which contained 50 g of mostly saturated fat. A repeated-measures ANOVA was performed to assess the effects of glycemic control (factor A) and TG response (factor B) to fat ingestion. In both groups plasma TG levels increased significantly after fat ingestion (P less than .001), and were significantly reduced during improved glycemic control (P less than .001). The reduction was observed in 14 of 15 patients in group I and in all patients in group II. In group I the lowering of the postprandial plasma TG levels after CSII was secondary to a decrease in the fasting plasma TG levels, as shown by the unchanged mean percent TG elevation over the baseline.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

The social image of smokeless tobacco use in three different types of teenagers.

The current study used an unobtrusive methodology to describe the social image associated with smokeless tobacco use and with cigarette smoking in three "types" of teenage models--an athlete, a cowboy, and an average teenager. As in previous research, the social image associated with smokeless tobacco use was more positive than that associated with cigarette smoking, suggesting that adopting smokeless tobacco use may have perceived social image benefits for adolescent males. The image conveyed by smokeless tobacco use was similar across the three different types of adolescent. Relations between social image factors and tobacco use behavior were examined, and implications for tobacco use prevention are discussed.

Adolescent↗

Intracardiac extension of intravenous leiomyomatosis.

A 42-year-old woman was found to have intravenous leiomyomatosis of the uterus with extension into the inferior vena cava and right atrium. Intravenous leiomyomatosis is a rare neoplastic disease characterized by invasion of venous channels by a benign smooth muscle tumor arising either from the wall of a vessel or from a uterine myoma. Intracardiac extension is often initially misdiagnosed as a right atrial myxoma and may cause death by mechanical obstruction. The diagnosis of intravenous leiomyomatosis should be considered in young women with cardiac symptoms associated with a right atrial mass who also have a pelvic mass or who have previously undergone hysterectomy because of leiomyoma uteri.

Adult↗

Variable expressivity in fundus albipunctatus.

A healthy, 14-year-old girl presented with nyctalopia, good vision, and multiple, irregular, yellowish lesions of the fundus. Dark adaptometry showed prolonged cone and rod branches, elevated thresholds, and the cone-rod transition occurring after 50 minutes in darkness. Her scotopic electroretinogram (ERG) b-wave attained normal amplitudes after 45 minutes of dark adaptation. The half-time for regeneration of rhodopsin after an extensive bleach was 16 minutes, four times longer than normal, and the maximum density difference measured by fundus reflectometry was at the lower limit of the normal range. Although photopigment kinetics were significantly faster than observed in other reported cases of fundus albipunctatus, it appears likely that there is a wide spectrum of functional and funduscopic abnormalities in this disorder. However, fundus appearance, adaptometric findings, and rhodopsin determinations serve to distinguish fundus albipunctatus from other flecked retina diseases.

Adolescent↗

Aniridia, congenital glaucoma, and hydrocephalus in a male infant with ring chromosome 6.

A premature infant with unilateral aniridia and congenital ectropion uveae, contralateral Rieger anomaly, bilateral congenital glaucoma, and hydrocephalus was found to have ring chromosome 6. The findings are consistent with multiple manifestations of a neural crest-derived maldevelopment of the anterior segment and central nervous system. Comparison with the 14 previously reported cases of ring chromosome 6 illustrates the phenotypic variability of this syndrome.

Abnormalities, Multiple↗

Evidence that cholesteryl ester hydrolase and triglyceride lipase are different enzymes in rat liver.

Studies on intracellular cholesteryl ester hydrolase (CEH) and triglyceride lipase (TGL) from rat adipose tissue and adrenal cortex have suggested that a single protein is responsible for both activities. To determine whether one hepatic protein catalyzes both reactions, we studied several properties of CEH and TGL in rat liver. During liver perfusion with heparin, perfusate peaks of TGL and CEH did not consistently coincide, and TGL activity was considerably higher and less heat-stable than that of CEH. Significant TGL, but not CEH, activity was released during incubation of isolated hepatocytes. Although microsomes isolated from hepatocytes contained both activities, the specific activities of CEH and TGL in cytosol from hepatocytes were 95% and 3%, respectively, of those found in cytosol from whole liver. Preincubation of liver cytosol with 5 mM Mg2+ decreased CEH, but not TGL, activity. Intracellular CEH and TGL activities were completely separated by prep-disc gel electrophoresis. Finally, both cytosolic and microsomal TGL, but not CEH, activities were inhibited by antiserum against rat hepatic TGL. We conclude that extracellular TGL does not have CEH activity and intracellular CEH differs from TGL.

Animals↗

Effect of large-dose progesterone on plasma levels of lipids, lipoproteins and apolipoproteins in males.

Eleven men with sexual deviation syndrome were hospitalized for treatment with medroxyprogesterone acetate (Depo-provera). Plasma total cholesterol, triglycerides, high density lipoprotein (HDL) cholesterol, low density lipoprotein (LDL) cholesterol, apo A-I and LDL apo B were measured before and during Depo-provera treatment. Ten normolipidemic and one mildly hypertriglyceridemic patient with 117 +/- 17% ideal body weight were maintained on a regular hospital diet before and during the study. They received an average total dose of 1273 +/- 467 mg Depo-provera by im injections over a mean period of 17 +/- 6 days. In the whole group, Depo-provera significantly reduced the plasma total cholesterol by 12% (p less than 0.0005), triglycerides by 24% (p less than 0.005), LDL cholesterol by 13% (p less than 0.01), LDL apo B by 15% (p less than 0.05), and apo A-I by 7% (p less than 0.05). Total HDL cholesterol, HDL2 cholesterol and HDL3 cholesterol did not change significantly. Excluding from the data analysis a normolipidemic patient who had a significant weight loss during the study and the hypertriglyceridemic patient, the fall in apo A-I during Depo-provera treatment was no longer statistically significant. We conclude that short-term, pharmacological doses of progesterone significantly reduce plasma concentrations of cholesterol, triglycerides, LDL cholesterol, and LDL apo B in men.

Adult↗

Long-term effects of guar gum on blood lipids.

While guar gum has been shown to lower total cholesterol and low density lipoprotein cholesterol (LDL-C) in diabetic patients over the short-term, the long-term effects are less well studied and may be unpredictable. Granola bars with and without 6.6 g guar gum were developed and fed to 16 adult volunteers with Type II diabetes mellitus who had been randomized in a double-blind fashion into guar and placebo groups of equal size. Four to six bars were consumed daily with an ad lib diet over a 6-month period. Total cholesterol, total high density lipoprotein cholesterol (HDL-C), subfractions HDL2-C and HDL3-C, LDL-C, and beta-apoprotein were measured at 0 and 6 months. Although LDL-C was lower and triglycerides higher at 6 months than at baseline, these changes were of equal magnitude and direction in both guar and placebo groups. Using each subject as his own control, only the change in triglycerides was statistically significant (P less than 0.025). When male subjects alone were analyzed, the guar group showed a statistically significant decrease in LDL, while the placebo group did not. Other lipid parameters were not significantly changed during the study, despite a positive effect on carbohydrate metabolism from the guar bars. The data suggest either that the hypolipemic effects of guar gum in patients with Type II diabetes mellitus are not sustained for 6 months, or the effects occur only in men.

Adult↗

Immobilized E. coli alkaline phosphatase. Its properties, stability, and utility in studying the dephosphorylation of proteins.

We have immobilized E. coli alkaline phosphatase (EC 3.1.3.1) by linking it covalently to sepharose 4B. This preparation has several advantages over the soluble enzyme. The immobilized enzyme is easily separable from other constituents in incubation mixtures. The immobilized enzyme can be reused repeatedly and is more stable than the soluble enzyme to heat treatment in the presence of 10 mM Mg2+. The insoluble and soluble phosphatases removed 75 and 77%, respectively, of the inorganic phosphorus from casein. The immobilized enzyme inactivated two enzymes believed to be active in the phosphorylated state, acyl-CoA:cholesterol acyltransferase (ACAT) by 39% and NADPH-cytochrome P-450 reductase by 89%. The utility of immobilized alkaline phosphatase for studying the phosphorylation and dephosphorylation of soluble or membrane-bound enzymes and proteins is discussed.

Alkaline Phosphatase↗

Effect of dietary treatment on the plasma levels of lipids, lipoprotein cholesterol and LDL B protein in children with type II hyperlipoproteinemia.

These data confirm the effectiveness of therapeutic diets in children with type II hyperlipoproteinemia in conventional terms. However, the decrease in LDL cholesterol was not accompanied by a significant decrease in LDL B (suggesting only a small change in the number of LDL particles in plasma), and a fall in HDL cholesterol occurred, with no significant improvement in the LDL to HDL cholesterol ratio. These findings indicate that further research is needed to develop diets with more optimal effects. Finally, our hypothesis that such diets may produce an LDL particle of altered composition, needs to be confirmed using additional physical-chemical measurements of the size, composition and molecular weight distribution of LDL in plasma before and after dietary intervention.

Adolescent↗

Retinal and optic nerve findings in Goldenhar-Gorlin syndrome.

Involvement of the posterior segment of the eye in Goldenhar-Gorlin syndrome is more common than is generally appreciated. We examined seven patients with this syndrome. Abnormalities included diminished visual acuity, tilted optic disc, optic nerve hypoplasia, tortuous retinal vessels, macular hypoplasia and heterotopia, microphthalmia and anophthalmia. In one case, pathologic study showed agenesis of the optic nerve. It is proposed that retinal, optic nerve and craniofacial abnormalities in this condition may reflect an asynchrony in the migration of the neural crest cells in the early stages of embryonal development.

Adolescent↗