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Biomedical subjects

A Sniderman

Publications and source records attributed to A Sniderman.

At least 37 records · Page 2Linked to original sources
PubMed1987

Malabsorption, hypocholesterolemia, and fat-filled enterocytes with increased intestinal apoprotein B. Chylomicron retention disease.

Eight infants presented with a malabsorption syndrome, normal fasting triglycerides, hypocholesterolemia (64.3 +/- 10.0 mg/dl), and deficiency of vitamins A and E. Plasma low-density lipoprotein, apolipoprotein B, and apolipoprotein A-I were decreased. After a fatty meal, plasma triglycerides did not increase and chylomicrons could not be identified. Lipoprotein composition was characterized by normal apoproteins, high phospholipids, and low cholesterol. Increased triglycerides were present in low-density lipoproteins. Immunoperoxidase localization of apolipoprotein B on fasting biopsy specimens showed increased staining of the lipid-laden intestinal epithelial cells compared to normals. On electron microscopy after a fat load, the enterocytes contained large numbers of fat particles vesiculating the endoplasmic reticulum. These particles, morphologically similar to chylomicrons, were also present as aggregates of well-individualized lipid droplets within dilated vesicles in the Golgi zone, but were not seen in the intercellular spaces and lacteals. This recessively transmitted condition differs from abetalipoproteinemia and from the homozygous form of hypobetalipoproteinemia and may be caused by a defect in the final assembly of chylomicrons or in the mechanism of their exocytosis.

Apolipoproteins B↗
PubMed1987

Hyperapobetalipoproteinemia: the major dyslipoproteinemia in patients with chronic renal failure treated with chronic ambulatory peritoneal dialysis.

In the present study, plasma cholesterol, triglyceride, low density lipoprotein (LDL)-cholesterol, high density lipoprotein (HDL)-cholesterol, and the major protein in LDL, apoB, were measured in 28 patients with chronic renal failure treated with hemodialysis and in 28 patients with chronic renal failure treated with chronic ambulatory peritoneal dialysis (CAPD). Elevated plasma triglycerides and reduced HDL cholesterol were frequent in both the hemodialysis and CAPD patients. However LDL levels were significantly higher in the CAPD patients as evident both by LDL cholesterol and LDL apoB. Even so, only one of the CAPD patients was hypercholesterolemic whereas 14 (or 50%) had hyperapobetalipoproteinemia (HyperapoB). Insulin-dependent diabetes was more frequent in the CAPD group but only 2 of the 9 insulin-dependent diabetics in this group had HyperapoB, and therefore, diabetes mellitus cannot account for the difference between the 2 groups. Thus HyperapoB appears to be a prevalent dyslipoproteinemia in CAPD patients and as such might be another factor which places CAPD patients at particularly increased risk of atherosclerosis.

Adult↗
PubMed1987

Brief periods of myocardial ischemia followed by reperfusion: a model in the dog of sudden cardiac death in humans.

Most experimental interest has focused on the electrophysiologic mechanisms which may underlie sudden death. The purpose of the present study was to develop a model in the dog where ischemia would produce not just ventricular fibrillation but also histologic abnormalities similar to those previously reported in sudden death in humans. First, the collateral blood flow to a portion of the ventricle was surgically abolished and then the arterial blood supply to this region was interrupted for 15 minutes after which nutrient flow was restored for 30 minutes. If the animal survived, this cycle was repeated up to seven times. Thirty-six animals were studied: 15 controls (Group A) survived an average of 1.75 +/- 0.8 cycles (mean +/- SD); 11 others (Group B) received a loading dose of sodium verapamil and survived 3.7 +/- 1.8 cycles while 10 (Group C) received a continuous infusion as well as the loading dose of sodium verapamil and survived 4.4 +/- 2.6 cycles--the difference between each group being significant (p less than 0.05). Four animals in Group C survived all 7 cycles; out of the other 32, 27 fibrillated just after reperfusion and the rest during ischemia. Histological examination revealed small foci of contraction band necrosis widely interspersed amongst large areas of morphologically normal tissue. The beneficial effect of verapamil and the histologic findings suggest cellular calcium overload may be important in the genesis of the fatal arrhythmias produced by reperfusion.

Animals↗
PubMed1986

Interventricular delay in severe aortic stenosis recognized by gated radionuclear blood pool scanning.

Twenty-four patients referred for cardiac catheterization for suspected aortic stenosis were investigated by equilibrium gated blood pool study. From the time-activity curves, end systole for each ventricle was determined and the delay between the two was calculated. The patients were divided into three groups according to their calculated aortic valve area. The mean delay in a control group of 20 normal subjects was 6 +/- 13 msec (mean +/- standard deviation). In group I (aortic valve area greater than 0.75 cm2) the delay was 16 +/- 25 msec (p = NS compared to controls); in group II (aortic valve area 0.5 to 0.75 cm2) the delay was 28 +/- 27 msec (p less than 0.01); and in group III (aortic valve area less than 0.5 cm2) the delay was 60 +/- 28 msec (p less than 0.001). Ten patients were restudied after valve replacement; their mean delay decreased markedly from 48 +/- 19 to 5 +/- 26 msec (p less than 0.001). Thus, this method appears to identify patients with severe aortic stenosis and may therefore be a useful adjunct to the noninvasive assessment of this disorder both before and after surgery.

Adult↗
PubMed1986

Genetic analysis of plasma sitosterol, apoprotein B, and lipoproteins in a large Amish pedigree with sitosterolemia.

We previously reported the finding of phytosterolemia, xanthomatosis, and hyperapobetalipoproteinemia (hyperapoB) in five siblings in a large Amish pedigree ascertained through a 13-year-old boy who died suddenly from advanced coronary atherosclerosis. Here, we present further analyses of the plasma levels of the plant sterol, sitosterol, of low density (beta) lipoprotein (LDL) sterol, and of LDL B protein. Of 254 relatives and spouses of the proband, 90.5% were examined. A series of genetic models were explored using a pedigree analysis where parameters reflecting frequency, transmission, and penetrance of putative genotypes were examined simultaneously using a maximum likelihood approach. Segregation analysis of the sitosterol levels showed that the phenotype of sitosterolemia was controlled by a rare autosomal recessive gene. There was also significant familial correlation in plasma sitosterol levels that was attributed to a polygenic component under a mixed model but could also be due to shared environments such as diets. The recessive model was supported by our finding that the plasma sitosterol levels in the parents and in six children born to three of the five sitosterolemics were less than 1 mg/dl, well within the normal range. The phenotype of hyperapoB is based on an elevated level of LDL B protein in the presence of a normal LDL cholesterol level (low LDL sterol to LDL B ratio). For both LDL sterol and LDL B, a polygenic model showed a slightly greater improvement in ln likelihood than did the Mendelian single locus model when both were compared to a sporadic model. Similar results were obtained for sterol levels of high density (alpha) lipoprotein (HDL) sterol. When segregation analysis was performed using the ratio of LDL sterol to LDL B, the Mendelian single locus model gave a slightly better fit to the data than did the polygenic model. While the analyses presented here provided unequivocal evidence for the recessive phenotype of phytosterolemia, we also identified a possible single gene factor that could account for the major portion of the strong familial aggregation in the ratio of LDL sterol to LDL B, and to a lesser extent LDL B. However, there is clear evidence of familial aggregation for these traits in this pedigree beyond that due to Mendelian components.

Adolescent↗
PubMed1985

Modulation of apolipoprotein B antigenic determinants in human low density lipoprotein subclasses.

To investigate the effect of low density lipoprotein (LDL) heterogeneity on the conformation of LDL apolipoprotein B (apo-B), the immunoreactivities of 6 monoclonal antibodies against LDL apo-B were measured in 3 LDL subfractions isolated by equilibrium density gradient ultracentrifugation. To ensure a broad range of LDL particles, the LDL subfractions were prepared from normal subjects and patients with hyperapobetalipoproteinemia. With 3 of the antibodies, 1D1, 5E11, and 3A10, LDL fractions 1 (the most buoyant), 2 (the intermediate), and 3 (the densest) were equally immunoreactive and competed similarly with reference whole LDL. In contrast, with 3 other antibodies, 2D8, 3F5, and 4G3, fraction 1 was significantly more reactive than fraction 3; that is for each in turn, 290, 250, and 150% more of the densest LDL protein was required to achieve the same displacement as with fraction 1. Further, the immunoreactivities of the 3 LDL fractions with antibodies 2D8, 3F5, and 4G3 were negatively correlated with their LDL cholesterol to LDL protein ratio with r values of 0.727, 0.898, and 0.870, respectively, suggesting that as LDL particle size decreases, the conformation of the LDL apo-B changes progressively. It is of interest that the antigenic determinants recognized by 3F5 and 4G3 are close to the LDL receptor recognition site on LDL apo-B. Therefore, it is possible that the reduced immunoreactivity of these determinants in dense LDL may be the in vitro correlate of the reduced fractional catabolics rate of dense LDL compared to buoyant LDL previously observed in vivo.

Antibodies, Monoclonal↗
PubMed1985

Familial aggregation and early expression of hyperapobetalipoproteinemia.

Family history is an important predictor of coronary risk. However, this relation, in large part, is not explained by the known risk factors such as systemic hypertension or hyperlipidemia. In the present study, plasma lipid, lipoprotein lipid, and plasma low-density lipoprotein (LDL) apoB levels were measured in 66 offspring (myocardial infarction [MI] offspring) of 24 families in which an index parent had premature coronary artery disease and hyperapobetalipoproteinemia. These results were compared to those obtained in 207 control children and young adults. Univariate analysis revealed that plasma LDL apoB and all other lipid and lipoprotein levels except high-density lipoprotein cholesterol were significantly higher in the MI offspring. Multivariate analysis showed plasma LDL apoB and LDL cholesterol best differentiated the MI offspring from control children and young adults. Of the 66 children, 22 had hyperapobetalipoproteinemia, of whom only 7 had clearly abnormal LDL cholesterol or plasma triglyceride levels. Thus, a substantial portion of children born to a parent with premature coronary artery disease and hyperapobetalipoproteinemia have the same disorder of lipoprotein metabolism.

Adolescent↗
PubMed1985

Early diastolic posterior aortic root slope, a clinical guide to the severity of mitral stenosis.

This report tests the hypothesis that, in early diastole, motion of the anterior left atrial wall corresponds to the motion that can be observed in the contiguous posterior wall of the aortic root. To test this hypothesis, we examined the effects of mitral stenosis, exercise in normals, exercise induced left ventricular ischemia, left ventricular hypertrophy and left ventricular dysfunction on this slope. Each altered early diastolic atrioventricular interaction as predicted and therefore, the early diastolic motion of the anterior left atrial wall does appear to be mirrored by the early diastolic slope of the posterior wall of the aortic root. Consequently, if interpreted in the clinical context, measurement of early diastolic slope of the posterior wall of the aortic root may serve as a useful guide to separate patients with severe from those with mild mitral stenosis.

Aorta↗
PubMed1984

The relation of risk factors to the development of atherosclerosis in saphenous-vein bypass grafts and the progression of disease in the native circulation. A study 10 years after aortocoronary bypass surgery.

We examined 82 patients 10 years after saphenous-vein aortocoronary bypass surgery to determine their angiographic status and to relate those findings to the risk factors for coronary-artery disease. Of 132 grafts shown to be patent 1 year after surgery, only 50 were unaffected at 10 years. The remainder were narrowed (43) or occluded (39). Disease progression in coronary arteries without grafts was also frequent, both in vessels that were normal (15 of 32) and in those with minor stenosis (25 of 53). New lesions did not develop in 15 patients, whereas they did in 67--in the grafts, the native vessels, or both. There was no significant difference between the two groups in the incidence of hypertension, diabetes, or smoking, whereas plasma levels of very-low-density lipoproteins (VLDLs) and low-density lipoproteins (LDLs) were higher, and high-density lipoprotein (HDL) levels were lower in those with new disease than in those without. Univariate analysis showed that plasma cholesterol and triglyceride levels were significantly higher at the time of surgery and at the 10-year examination in those with new lesions. Multivariate analysis indicated that among the lipoprotein indexes, levels of HDL cholesterol and plasma LDL apoprotein B best distinguished the two groups. The findings indicate that atherosclerosis in these patients was a progressive disease, frequently affecting both the grafts and the native vessels, and that the course of such disease may be related to the plasma lipoprotein levels.

Cholesterol↗
PubMed1984

Correlative electrocardiographic studies in myocardial infarction.

The electrocardiogram remains our first and most widely available tool to study myocardial infarction. We have attempted to suggest that we must be both more circumspect and adventurous in its use. The traditional electrocardiographic labels imply a level of precision that is unwarranted and so obscure real, often clinically important differences that exist among patients. We believe the electrocardiogram becomes more powerful when it is used with other noninvasive tests, and all such tests are best interpreted within rather than removed from the clinical context. We suggest as well that frequently after infarction, zones of viable muscle exist that are still vulnerable to ischemia. These zones may lie within or be remote from the actual infarct region, and noninvasive tests should be milked for clues that these regions are present, for while there is little to be done for necrotic tissue after infarction, there is considerable therapy available, both medical and surgical, to rescue tissue at risk of ischemia after infarction.

Electrocardiography↗
PubMed1984

Pulmonary blood flow. A potential factor in the pathogenesis of pulmonary edema.

The purpose of this study is to examine the hemodynamics of the pulmonary circulation and the potential role of pulmonary blood flow in the pathogenesis of cardiogenic pulmonary edema. To do so, the pulmonary circulation was isolated and controlled such that, within a closed circuit, pulmonary blood flow and left atrial pressure (LAP) could be regulated independently: the first by a constant flow pump, the second by a variable height reservoir. The effect of pulmonary blood flow on pulmonary artery pressure and intravascular blood volume was then determined at different LAPs. Contrary to our expectations, the results indicate that (1) pulmonary vascular resistance does not change appreciably as flow increases, (2) the microcirculation comprises the major capacitance vessels of the lung, and (3) increased pulmonary flow in the normal lung causes little change in intravascular pulmonary blood volume, whereas, by contrast, major changes in pulmonary blood volume occur as LAP rises. Next, the effect of pulmonary blood flow on edema formation in the lungs was examined. Below a critical level of LAP (15 mm Hg in these experiments), pulmonary blood flow up to 5 L/min did not produce pulmonary edema. Above this level, however, such an effect was clear. Thus, at an LAP of 20 mm Hg, edema did not develop if pulmonary flow was low (0.7 L/min) but did if flow was increased to 2 L/min. As well, if the LAP was 17.5 mm Hg and pulmonary flow 3.5 L/min, severe pulmonary edema also resulted.

Animals↗
PubMed1983

Phase and amplitude imaging in the diagnosis of acute right ventricular damage in inferior infarction.

Right ventricular infarction is not uncommon in patients with inferior myocardial infarcts. Phase and amplitude analysis of equilibrium gated cardiac blood pool studies is useful in documenting right ventricular damage complicating left ventricular infarction. In 15 of 37 patients with acute inferior infarction, right ventricular extension of the infarct was demonstrated by this approach, complementing the findings of infarct avid imaging with Tc-99m pyrophosphate (Tc-99m PYP).

Aged↗
PubMed1982

Clinical outcomes after inferior myocardial infarction.

We studied the clinical outcomes of 46 patients followed prospectively for the initial 6 months after inferior infarction. Twenty-one patients (Group A) had no anterior ST depression (V2 to V4) present during the acute phase of the inferior infarction, whereas 25 patients (Group B) had such findings transiently. Although the clinical course during hospitalization was similar in the two groups, that after discharge differed. Only one of 21 patients in Group A had exertional angina and none had rest angina during follow-up; no infarcts or deaths occurred. In contrast, 15 patients in Group B had exertional angina; 12 also had rest pain (p less than 0.001, exact probability test). Two patients had reinfarction, one of whom died, and one sudden death also occurred. Of 15 patients in Group B who had cardiac catheterization, only eight had significant lesions in the anterior vessels, whereas seven did not; six of the seven patients became asymptomatic during follow-up without surgical therapy. Thus, electrocardiograms taken during the early phases of inferior myocardial infarction may be a valuable tool to recognize patients likely to have further ischemic symptoms during the early follow-up period.

Angina Pectoris↗
PubMed1980

Differentiation between reperfusion and occlusion myocardial necrosis with technetium-99m pyrophospate scans.

Although infarct-avid scanning is used to diagnose myocardial infarction, no distinction is made between two very different mechanisms of myocardial cell death. These are occlusion necrosis and reperfusion necrosis. The former is the major form of cell injury after a sustained occlusion of a coronary artery; the latter occurs immediately after reperfusion of ischemic myocardium. The present study examines whether the technetium pyrophosphate scan becomes positive more rapidly with one form of injury rather than the other. Myocardial damage was produced in dogs by either sustained coronary occlusion (Group I, 6 dogs) or reperfusion (Group II, 18 dogs). Infarct-avid myocardial scans obtained with technetium-99m pyrophosphate were positive in only one of six animals in Group I when isotope was injected 4.5 hours after sustained coronary occlusion. In contrast, 12 or 13 animals (Group IIa) with reperfusion necrosis had a positive scan when isotope was injected 3.5 hours after reperfusion. When isotope was injected only 30 minutes after the onset of reperfusion (Group IIb), only two of five scans were clearly positive. The results indicate that if the interval between myocardial injury and a positive scan is known, inference can be made concerning the predominant type of injury.

Animals↗