Cardiovascular risk assessment by coronary artery calcium score in subjects with maturity-onset diabetes of the young caused by glucokinase mutations.

AIMS: Maturity-Onset Diabetes of the Young (MODY) caused by glucokinase (GCK) mutations is characterized by lifelong mild non-progressive hyperglycemia, with low frequency of coronary artery disease (CAD) compared to other types of diabetes. The aim of this study is to estimate cardiovascular risk by coronary artery calcification (CAC) score in this group. MATERIALS AND METHODS: Twenty-nine GCK-MODY cases, 26 normoglycemic controls (recruited among non-affected relatives/spouses of GCK mutation carriers), and 24 unrelated individuals with type 2 diabetes were studied. Patients underwent CAC score evaluation by computed tomography and were classified by Agatston score ≥ or < 10. Framingham Risk scores of CAD in 10 years were calculated. RESULTS: Median [interquartile range] CAC score in GCK-MODY was 0 [0,0], similar to controls (0 [0,0], P = 0.49), but lower than type 2 diabetes (39 [0, 126], P = 2.6 × 10-5). A CAC score ≥ 10 was seen in 6.9% of the GCK group, 7.7% of Controls (P = 1.0), and 54.2% of individuals with type 2 diabetes (P = 0.0006). Median Framingham risk score was lower in GCK than type 2 diabetes (3% vs. 13%, P = 4 × 10-6), but similar to controls (3% vs. 4%, P = 0.66). CONCLUSIONS: CAC score in GCK-MODY is similar to control individuals from the same family and/or household and is significantly lower than type 2 diabetes. Besides demonstrating low risk of CAD in GCK-MODY, these findings may contribute to understanding the specific effect of hyperglycemia in CAD.

The coronary artery calcium score is linked to plasma cholesterol synthesis and absorption markers: Brazilian Longitudinal Study of Adult Health.

It is controversial whether atherosclerosis is linked to increased intestinal cholesterol absorption or synthesis in humans. The aim of the present study was to relate atherosclerosis to the measurements of plasma markers of cholesterol synthesis (desmosterol, lathosterol) and absorption (campesterol, sitosterol). In healthy male (n=344), non-obese, non-diabetics, belonging to the city of São Paulo branch of the Brazilian Longitudinal Study of Adult Health (ELSA-Brasil), we measured in plasma these non-cholesterol sterol markers, together with their anthropometric, dietary parameters, traditional atherosclerotic risk factors, and blood chemistry, coronary arterial calcium score (CAC), and ultrasonographically measured common carotid artery intima-media thickness (CCA-IMT). Cases with CAC>zero had the following parameters higher than cases with CAC = zero: age, waist circumference (WC), plasma total cholesterol (TC), low-density lipoprotein-cholesterol (LDL-C), and non-high density lipoprotein-cholesterol (non HDL-C). Plasma desmosterol and campesterol, duly corrected for TC, age, body mass index (BMI), waist circumference (WC), hypertension, smoking, and the homeostasis model assessment-insulin resistance (HOMA-IR) correlated with CAC, but not with CCA-IMT. The latter related to increased age, BMI, waist circumference (WC), and systolic blood pressure (SBP). Plasma HDL-C concentrations did not define CAC or CCA-IMT degrees, although in relation to the lower tertile of HDL-C in plasma the higher tertile of HDL-C had lower HOMA-IR and concentration of a cholesterol synthesis marker (desmosterol). Present work indicated that increased cholesterol synthesis and absorption represent primary causes of CAD, but not of the common carotid artery atherosclerosis.

Selenium-containing indolyl compounds: Kinetics of reaction with inflammation-associated oxidants and protective effect against oxidation of extracellular matrix proteins.

Activated white blood cells generate multiple oxidants in response to invading pathogens. Thus, hypochlorous acid (HOCl) is generated via the reaction of myeloperoxidase (from neutrophils and monocytes) with hydrogen peroxide, and peroxynitrous acid (ONOOH), a potent oxidizing and nitrating agent is formed from superoxide radicals and nitric oxide, generated by stimulated macrophages. Excessive or misplaced production of these oxidants has been linked to multiple human pathologies, including cardiovascular disease. Atherosclerosis is characterized by chronic inflammation and the presence of oxidized materials, including extracellular matrix (ECM) proteins, within the artery wall. Here we investigated the potential of selenium-containing indoles to afford protection against these oxidants, by determining rate constants (k) for their reaction, and quantifying the extent of damage on isolated ECM proteins and ECM generated by human coronary artery endothelial cells (HCAECs). The novel selenocompounds examined react with HOCl with k 0.2-1.0 × 108M-1s-1, and ONOOH with k 4.5-8.6 - × 105M-1s-1. Reaction with H2O2 is considerably slower (k < 0.25M-1s-1). The selenocompound 2-phenyl-3-(phenylselanyl)imidazo[1,2-a]pyridine provided protection to human serum albumin (HSA) against HOCl-mediated damage (as assessed by SDS-PAGE) and damage to isolated matrix proteins induced by ONOOH, with a concomitant decrease in the levels of the biomarker 3-nitrotyrosine. Structural damage and generation of 3-nitroTyr on HCAEC-ECM were also reduced. These data demonstrate that the novel selenium-containing compounds show high reactivity with oxidants and may modulate oxidative and nitrosative damage at sites of inflammation, contributing to a reduction in tissue dysfunction and atherogenesis.

Differences determined by optical coherence tomography volumetric analysis in non-culprit lesion morphology and inflammation in ST-segment elevation myocardial infarction and stable angina pectoris patients.

BACKGROUND: While the current methodology for determining fibrous cap (FC) thickness of lipid plaques is based on manual measurements of arbitrary points, which could lead to high variability and decreased accuracy, it ignores the three-dimensional (3-D) morphology of coronary artery disease. OBJECTIVE: To compare, utilizing optical coherence tomography (OCT) assessments, volumetric quantification of FC, and macrophage detection using both visual assessment and automated image processing algorithms in non-culprit lesions of STEMI and stable angina pectoris (SAP) patients. METHODS: Lipid plaques were selected from 67 consecutive patients (1 artery/patient). FC was manually delineated by a computer-aided method and automatically classified into three thickness categories: FC < 65 µm (i.e., thin-cap fibroatheroma [TCFA]), 65-150 µm, and >150 µm. Minimum thickness, absolute categorical surface area, and fractional luminal area of FC were analyzed. Automated detection and quantification of macrophage was performed within the segmented FC. RESULTS: A total of 5,503 cross-sections were analyzed. STEMI patients when compared with SAP patients had more absolute categorical surface area for TCFA (0.43 ± 0.45 mm(2) vs. 0.15 ± 0.25 mm(2) ; P = 0.011), thinner minimum FC thickness (31.63 ± 17.09 µm vs. 47.27 ± 26.56 µm, P = 0.012), greater fractional luminal area for TCFA (1.65 ± 1.56% vs. 0.74 ± 1.2%, P = 0.046), and greater macrophage index (0.0217 ± 0.0081% vs. 0.0153 ± 0.0045%, respectively, P < 0.01). CONCLUSION: The novel OCT-based 3-D quantification of the FC and macrophage demonstrated thinner FC thickness and larger areas of TCFA coupled with more inflammation in non-culprit sites of STEMI compared with SAP.

Coronary artery plaque burden and calcium scores in healthy men adhering to long-term wine drinking or alcohol abstinence

Observational studies suggest there are clinical benefits to moderate red wine (RW) consumption. However, the effects on coronary vasculature and overall lifestyle are unclear. We investigated whether a lifestyle of regular long-term RW consumption is associated with changes in coronary plaque burden, calcium score, carotid intima/media thickness, endothelial function, and metabolic variables, compared with alcohol abstinence. Healthy volunteers were evaluated by coronary computed tomography angiography (CTA) as well as carotid and brachial artery ultrasound. Nutritional status, psychological status, and metabolic variables were assessed. The study included 101 drinkers [aged 58.9±7.3 years (means±SD)], from wine brotherhoods, and 104 abstainers, from Anglican, Evangelical and Catholic churches both in the city of São Paulo, Brazil. No significant differences in demographics were noted. Lesion prevalence per patient assessed by coronary CTA and classified as absent (0), 1-25, 26-49, and ≥50% stenosis was similar between groups. When analyzed by individual arteries, i.e., left anterior descending, circumflex, and right coronary, prevalence was also not different. On the other hand, calcium scores were higher among drinkers than abstainers (144.4±362.2 vs 122.0±370.3; P<0.01). However, drinkers reported less history of diabetes and exercised more. RW drinkers consumed 2127.9±387.7 kcal/day while abstainers consumed 1836.0±305.0 (P<0.0001). HDL cholesterol was significantly higher among drinkers compared to abstainers (46.9±10.9 vs 39.5±9.0 mg/dL; P<0.001), while fasting plasma glucose was lower (97.6±18.2 vs 118.4±29.6 mg/dL; P<0.02). Liver enzymes were normal in both groups. In conclusion, long-term wine drinkers displayed a similar plaque burden but greater calcium score than abstainers, despite a more atherogenic diet, and the mechanisms for the increased calcium scores in the former remain speculative.

Coronary artery plaque burden and calcium scores in healthy men adhering to long-term wine drinking or alcohol abstinence.

Observational studies suggest there are clinical benefits to moderate red wine (RW) consumption. However, the effects on coronary vasculature and overall lifestyle are unclear. We investigated whether a lifestyle of regular long-term RW consumption is associated with changes in coronary plaque burden, calcium score, carotid intima/media thickness, endothelial function, and metabolic variables, compared with alcohol abstinence. Healthy volunteers were evaluated by coronary computed tomography angiography (CTA) as well as carotid and brachial artery ultrasound. Nutritional status, psychological status, and metabolic variables were assessed. The study included 101 drinkers [aged 58.9 ± 7.3 years (means ± SD)], from wine brotherhoods, and 104 abstainers, from Anglican, Evangelical and Catholic churches both in the city of São Paulo, Brazil. No significant differences in demographics were noted. Lesion prevalence per patient assessed by coronary CTA and classified as absent (0), 1-25, 26-49, and ≥ 50% stenosis was similar between groups. When analyzed by individual arteries, i.e., left anterior descending, circumflex, and right coronary, prevalence was also not different. On the other hand, calcium scores were higher among drinkers than abstainers (144.4 ± 362.2 vs 122.0 ± 370.3; P<0.01). However, drinkers reported less history of diabetes and exercised more. RW drinkers consumed 2127.9 ± 387.7 kcal/day while abstainers consumed 1836.0 ± 305.0 (P<0.0001). HDL cholesterol was significantly higher among drinkers compared to abstainers (46.9 ± 10.9 vs 39.5 ± 9.0 mg/dL; P<0.001), while fasting plasma glucose was lower (97.6 ± 18.2 vs 118.4 ± 29.6 mg/dL; P<0.02). Liver enzymes were normal in both groups. In conclusion, long-term wine drinkers displayed a similar plaque burden but greater calcium score than abstainers, despite a more atherogenic diet, and the mechanisms for the increased calcium scores in the former remain speculative.

Hybrid myocardial imaging for risk stratification prior to kidney transplantation: added value of coronary calcium and epicardial adipose tissue.

BACKGROUND: Patient selection and optimal approach to risk stratification prior to kidney transplantation remain uncertain. We sought new predictors of an abnormal myocardial perfusion (MYP) stress test result. METHODS: Retrospective study of 411 consecutive chronic kidney disease stages 4-5D patients awaiting kidney transplantation referred for risk stratification. PET-CT or SPECT-CT was used to assess MYP and quantify coronary artery calcium (CAC) and epicardial adipose tissue (EAT). Abnormal MYP was defined as a perfusion defect involving ≥5% of the left ventricular myocardium. RESULTS: Fixed or reversible MYP defects were present in 41 patients (10%). Male sex, smoking, and history of cardiovascular disease were more prevalent; age was higher and CAC and EAT were greater in patients with MYP defects than in those with normal MYP. On multivariate logistic regression, EAT and CAC were independent predictors of abnormal MYP while diabetes mellitus showed a borderline association (P = .08). EAT added incremental diagnostic value to a model including age, CAC and diabetes mellitus [AUC 0.73 (95% CI 0.64-0.81) to 0.76 (95% CI 0.68-0.84; P = .02)]. Furthermore, the model containing EAT showed improved diagnostic discrimination. CONCLUSIONS: Abnormal MYP on screening stress testing appears to be rare in patients awaiting kidney transplantation suggesting an excess of testing. EAT and CAC may help predict what patients are at higher risk of developing abnormalities of MYP under stress.

Puntuación de calcio en arterias coronarias y presencia de estenosis angiográficamente significativas / Calcium scoring in coronary arteries and presence of angiographically significant stenosis

La presencia de calcio en las arterias coronarias es patognomónica de aterosclerosis. Agatston diseñó un método para cuantificar el puntaje de calcio coronario mediante la tomografía para determinar la asociación entre el grado de calcificación en las arterias coronarias determinado por tomografía de 64 cortes y la presencia de estenosis coronarias significativas (ECS) diagnosticadas por coronariografía invasiva (CI). Se estudiaron 153 pacientes, los cuales estaban programados para CI. Previo a la CI se les cuantificó el puntaje de calcio por arteria y por paciente. El 91,7 por ciento de los pacientes con puntaje ≤ 10 UA (unidades Agatston) no presentó ECS contra 8,3 por ciento con puntaje ≥ 401 UA (p < ,0001). El 2,4 por ciento con puntaje ≤ 10 UA en el tronco de la coronaria izquierda presentó ECS contra el 75 por ciento con puntaje ≥ 401 UA (p< 0,0001). El 5,5 por ciento con puntaje ≤ 10 UA en la arteria descendente anterior tenía ECS contra el 87,5 por ciento con puntaje ≥ 401 UA (p< 0,0001). Similar comportamiento se observó en las demás arterias. Se concluyó que existía asociación entre el grado de puntaje de calcio en las arterias coronarias diagnosticado por tomografía y la presencia de ECS diagnosticadas por CI(AU)

The presence of calcium in the coronary arteries is pathognomonic of atherosclerosis. Agatston designed a method to quantify the coronary calcium scoring by tomography to determine the association between the degree of calcification in the coronary arteries by 64-slice computed tomography and the presence of significant coronary stenosis (SCS) diagnosed by invasive coronariography (IC). 153 patients that were scheduled for IC were studied. The calcium scoring was quantified by patient and by artery before performing the IC. 91.7 percent of the patients with scoring £10 UA (Agatston units) did not present SCS versus 8.3 percent with scoring ³ 401 UA (p< .0001). 2.4 percent with scoring 10 UA in the trunk of the left coronary presented SCS versus 75 percent with scoring 401 UA (p < 0.0001). 5.5 percent with scoring 10 UA in the anterior descending artery has SCS versus 87.5 percent with scoring 401 UA (p < 0.0001). Similar behavior was observed in the rest of the arteries. It was concluded that there was association between the calcium degree scoring in the coronary arteries diagnosed by computed tomography and the presence of SCS diagnosed by IC(AU)

Identification of calcifications in cardiac valves by near infrared Raman spectroscopy.

OBJECTIVE: The objective of this work was to detect calcification in cardiac valves using near infrared Raman spectroscopy (NIRS). A Ti:sapphire laser pumped by an argon-ion laser operating at a wavelength of 830 nm was used for excitation of the valve samples, and Raman emission was detected by an optical spectrometer with a liquid nitrogen-cooled CCD detector. BACKGROUND: Cardiac valves are subjected to highly repetitive mechanical stresses, due to their over 40 million cardiac cycles per year. These structures may suffer cumulative lesions, complicated by the deposition of calcium phosphate, which can lead to clinically significant diseases. NIRS can provide important information about biological tissue composition and has been used for diagnosis of some types of human pathology. METHODS: Samples of normal and pathologic tissues 5 mm in size were analyzed. RESULTS: It was observed that the Raman spectrum of calcified cardiac valves presented different behavior when compared with normal valves. Differences were observed at the intensity of 960, 1,260, 1,452, and 1,660 cm(1) peaks. CONCLUSIONS: These results suggest that this technique could be used to detect calcium phosphate mineral deposition in cardiac valves.

Puntuación de calcio en arterias coronarias y presencia de estenosis angiográficamente significativas / Calcium scoring in coronary arteries and presence of angiographically significant stenosis

La presencia de calcio en las arterias coronarias es patognomónica de aterosclerosis. Agatston diseñó un método para cuantificar el puntaje de calcio coronario mediante la tomografía para determinar la asociación entre el grado de calcificación en las arterias coronarias determinado por tomografía de 64 cortes y la presencia de estenosis coronarias significativas (ECS) diagnosticadas por coronariografía invasiva (CI). Se estudiaron 153 pacientes, los cuales estaban programados para CI. Previo a la CI se les cuantificó el puntaje de calcio por arteria y por paciente. El 91,7 por ciento de los pacientes con puntaje ≤ 10 UA (unidades Agatston) no presentó ECS contra 8,3 por ciento con puntaje ≥ 401 UA (p < ,0001). El 2,4 por ciento con puntaje ≤ 10 UA en el tronco de la coronaria izquierda presentó ECS contra el 75 por ciento con puntaje ≥ 401 UA (p< 0,0001). El 5,5 por ciento con puntaje ≤ 10 UA en la arteria descendente anterior tenía ECS contra el 87,5 por ciento con puntaje ≥ 401 UA (p< 0,0001). Similar comportamiento se observó en las demás arterias. Se concluyó que existía asociación entre el grado de puntaje de calcio en las arterias coronarias diagnosticado por tomografía y la presencia de ECS diagnosticadas por CI.

The presence of calcium in the coronary arteries is pathognomonic of atherosclerosis. Agatston designed a method to quantify the coronary calcium scoring by tomography to determine the association between the degree of calcification in the coronary arteries by 64-slice computed tomography and the presence of significant coronary stenosis (SCS) diagnosed by invasive coronariography (IC). 153 patients that were scheduled for IC were studied. The calcium scoring was quantified by patient and by artery before performing the IC. 91.7 percent of the patients with scoring £10 UA (Agatston units) did not present SCS versus 8.3 percent with scoring ³ 401 UA (p< .0001). 2.4 percent with scoring 10 UA in the trunk of the left coronary presented SCS versus 75 percent with scoring 401 UA (p < 0.0001). 5.5 percent with scoring 10 UA in the anterior descending artery has SCS versus 87.5 percent with scoring 401 UA (p < 0.0001). Similar behavior was observed in the rest of the arteries. It was concluded that there was association between the calcium degree scoring in the coronary arteries diagnosed by computed tomography and the presence of SCS diagnosed by IC.

[Calcium score in the cardiovascular evaluation of the diabetic patient]. / Escore de cálcio na avaliação cardiovascular do paciente com diabetes.

Coronary artery calcium (CAC) is a specific marker of atherosclerosis, independent of its etiology. Quantification of CAC by computed tomography (CT) is a non-invasive test recommended mainly for risk stratification for coronary heart disease, in addition to the conventional stratification, especially in intermediate risk patients. Currently, a diabetic patient is classified as a coronary heart disease equivalent, therefore a high-risk patient according to most societies. For that matter calcium score is not currently recommended for diabetic patients. Although quantification of CAC by computed tomography (CT) is not yet supported by strong evidence in diabetes, small studies showed evidence that this test could have a discriminatory power in terms of prognosis within this group of patients. That could be a future clinical application, depending on the validation of these data and the results of future studies. There are some other potential applications for this method that could be useful for diabetic patients as well, but haven't been extensively validated, like the assessment of progression of coronary artery calcium as a form of evaluating effectiveness of medical therapy. In this article we review the method and current indications for the quantification of CAC by computed tomography.

Coronary fat content evaluated by morphometry in patients with severe atherosclerosis has no relation with serum lipid levels.

The relationship between lipid serum levels and coronary atherosclerotic plaque fat content was studied in 51 necropsy patients. Serum lipids were measured by standard techniques, during life, in the absence of lipid-lowering drugs. Intima, intimal fat and media areas were measured using a computerized system in cryosections of the odd segments of the right, anterior descending and circumflex coronary arteries stained with Sudan-IV. Mean intimal and lipid areas were 5.74 +/- 1.98 and 1.22 +/- 0.55 mm2 (22.12 +/- 8.48%) in 26 cases with high cholesterol (>or=200 mg/dL) and 4.98 +/- 1.94 and 1.16 +/- 0.66 mm2 (22.75 +/- 9.06%) in 25 cases with normal cholesterol (<200 mg/dL; P > 0.05). Patients with high levels of low-density lipoprotein (>or=130 mg/dL, N = 15) had a higher intima/media area ratio than those with normal levels of low-density lipoprotein (<130 mg/dL, N = 13, P < 0.01). No significant difference in the morphometrical variables was found in groups with high or low serum levels of triglycerides (>or=200 mg/dL, N = 13 vs <200 mg/dL, N = 36) or high-density lipoprotein (>or=35 mg/dL, N = 11 vs <35 mg/dL, N = 17). The association between the morphological measurements and serum levels of cholesterol, its fractions, and triglycerides was also tested and the correlation coefficients were low. Although high cholesterol is a risk factor, we show here that in patients with severe atherosclerosis blood cholesterol and triglyceride levels seem to have little influence on coronary lipid content, indicating that other factors may contribute to arterial lipid deposition and plaque formation.

Coronary fat content evaluated by morphometry in patients with severe atherosclerosis has no relation with serum lipid levels

The relationship between lipid serum levels and coronary atherosclerotic plaque fat content was studied in 51 necropsy patients. Serum lipids were measured by standard techniques, during life, in the absence of lipid-lowering drugs. Intima, intimal fat and media areas were measured using a computerized system in cryosections of the odd segments of the right, anterior descending and circumflex coronary arteries stained with Sudan-IV. Mean intimal and lipid areas were 5.74 ± 1.98 and 1.22 ± 0.55 mm² (22.12 ± 8.48 percent) in 26 cases with high cholesterol (³200 mg/dL) and 4.98 ± 1.94 and 1.16 ± 0.66 mm² (22.75 ± 9.06 percent) in 25 cases with normal cholesterol (<200 mg/dL; P > 0.05). Patients with high levels of low-density lipoprotein (³130 mg/dL, N = 15) had a higher intima/media area ratio than those with normal levels of low-density lipoprotein (<130 mg/dL, N = 13, P < 0.01). No significant difference in the morphometrical variables was found in groups with high or low serum levels of triglycerides (³200 mg/dL, N = 13 vs <200 mg/dL, N = 36) or high-density lipoprotein (³35 mg/dL, N = 11 vs <35 mg/dL, N = 17). The association between the morphological measurements and serum levels of cholesterol, its fractions, and triglycerides was also tested and the correlation coefficients were low. Although high cholesterol is a risk factor, we show here that in patients with severe atherosclerosis blood cholesterol and triglyceride levels seem to have little influence on coronary lipid content, indicating that other factors may contribute to arterial lipid deposition and plaque formation.

Escore de cálcio na avaliação cardiovascular do paciente com diabetes / Calcium score in the cardiovascular evaluation of the diabetic patient

A presença de cálcio nas artérias coronárias é um marcador específico de aterosclerose, independentemente de sua etiologia. A quantificação do cálcio coronariano através da tomografia computadorizada é um método não-invasivo cuja principal indicação clínica atual é a estratificação de risco para doença coronariana, complementando a estratificação convencional principalmente naqueles pacientes de risco intermediário. Pela classificação atual de fatores de risco da maioria das sociedades, o paciente diabético é categorizado como equivalente de doença coronariana, sendo portanto um paciente de alto risco. Assim sendo, a quantificação do cálcio coronariano não teria indicação para esses pacientes com esta finalidade. No entanto, estudos com pequenos grupos mostram evidências de que, mesmo nesses pacientes, este teste teria um poder discriminatório em termos de prognóstico. Esta pode ser uma aplicação clínica potencial desse método, dependendo da validação desses dados e de estudos futuros. Existem outras aplicações potenciais para esse método, que necessitam de validação e que podem ser úteis para os diabéticos, como o estudo da progressão do cálcio avaliando a eficácia da terapêutica. Neste artigo revisamos o método e as principais indicações da quantificação do escore de cálcio coronariano.

Coronary artery calcium (CAC) is a specific marker of atherosclerosis, independent of its etiology. Quantification of CAC by computed tomography (CT) is a non-invasive test recommended mainly for risk stratification for coronary heart disease, in addition to the conventional stratification, especially in intermediate risk patients. Currently, a diabetic patient is classified as a coronary heart disease equivalent, therefore a high-risk patient according to most societies. For that matter calcium score is not currently recommended for diabetic patients. Although quantification of CAC by computed tomography (CT) is not yet supported by strong evidence in diabetes, small studies showed evidence that this test could have a discriminatory power in terms of prognosis within this group of patients. That could be a future clinical application, depending on the validation of these data and the results of future studies. There are some other potential applications for this method that could be useful for diabetic patients as well, but haven't been extensively validated, like the assessment of progression of coronary artery calcium as a form of evaluating effectiveness of medical therapy. In this article we review the method and current indications for the quantification of CAC by computed tomography.

Lipid abnormalities in children with types A and B Niemann Pick disease.

OBJECTIVE: To characterize the lipid profiles in patients with types A and B Niemann Pick disease (NPD) and determine if lipid abnormalities are associated with evidence of early cardiovascular disease or correlate with genotype. STUDY DESIGN: The study was a cross-sectional analysis of 10 patients with NPD type A and 30 patients with NPD type B that was carried out in the General Clinical Research Center. For each patient, fasting lipid profile and glucose, T4, height or length, weight, resting blood pressure, and acid sphingomyelinase deficiency genotype were measured. In type B patients, electrocardiograhic-gated helical computed tomography of the heart also was obtained. RESULTS: Lipid abnormalities included low (<35 mg/dL) high-density lipoprotein cholesterol in 100% of patients and hypertriglyceridemia and increased low-density lipoprotein cholesterol in 62% (25/40) and 67% (27/40) of patients, respectively. Coronary artery calcium scores were positive (>1.0) in 10 of 18 type B patients studied. There was no correlation of the Delta R608 genotype with a milder phenotype for the lipid abnormalities, as has been observed for a number of other NPD manifestations. CONCLUSIONS: Lipid abnormalities are part of the phenotype in types A and B NPD and may be associated with early atherosclerotic heart disease.

Correlation between near-infrared Raman spectroscopy and the histopathological analysis of atherosclerosis in human coronary arteries.

BACKGROUND AND OBJECTIVES: Modern diagnostic methods such as near-infrared Raman spectroscopy (NIRS) allow quantification and evaluation of human atherosclerotic lesions, which can be useful in diagnosing coronary artery disease. The objective of the present study is to obtain feasible diagnostic information to detect atheromatous plaque using NIRS combined with discriminant analysis. STUDY DESIGN/MATERIAL AND METHODS: An 830 nm Ti: sapphire laser pumped by an argon laser provides near-infrared excitation. A spectrograph disperses light scattered from arterial tissue and a liquid-nitrogen cooled CCD detects the Raman spectra. A total of 111 arterial fragments were scanned and Raman results were compared with histopathology. Principal components analysis (PCA) and Mahalanobis distance (m-distance) were used to model an algorithm for tissue classification into three categories: non-atherosclerotic (NA), non-calcified (NC), and calcified (C) using Raman spectra. Spectra were randomly separated into training and prospective groups. RESULTS: It has been found that, for the NA tissue, the algorithm has sensitivity of 84 and 78% and specificity of 91 and 93% for training and prospective groups, respectively. For the NC tissue the algorithm has sensitivity of 88 and 90% and specificity of 88 and 83%. For the C tissue both sensitivity and specificity were maximum, 100%. CONCLUSIONS: An algorithm using PCA and discriminant analysis based on m-distance has been developed and successfully applied to diagnose coronary artery disease by NIRS obtaining good sensitivity and specificity for each tissue category.