Atherogenic dyslipidemia in patients with established coronary artery disease.

BACKGROUND AND AIMS: Patients with stable coronary heart disease (CHD) and atherogenic dyslipidemia (AD) have a high-risk of recurrence and are those who derive most benefit from treatment with lipid-lowering agents. The aim of this study was to examine the prevalence of AD in patients with stable coronary heart disease and to investigate associated factors. METHODS: Cross-sectional study involving 7823 subjects admitted for a coronary event between 6 months and 10 years previously. AD was considered to be the concurrent presence of low HDL-cholesterol (<1.03 mmol/L [40 mg/dL] in males, <1.29 mmol/L [50 mg/dL] in females) and elevated triglycerides (≥1.7 mmol/L [150 mg/dL]). RESULTS: Mean age was 65.3 (10.1) years, 73.6% were males and 80.3% were receiving treatment with statins. Low HDL-cholesterol was observed in 26.3% of the participants, 39.7% had elevated triglyceride concentration and 13.0% had AD. The percentage of AD in patients with criteria for metabolic syndrome was 30.9%. Factors associated directly and independently with the presence of AD in the multivariate analysis were female sex, history of coronary syndrome without ST elevation or coronary revascularization, presence of atrial fibrillation, body mass index, LDL-cholesterol, systolic blood pressure and blood glucose levels, while age and glomerular filtration rate were significantly and inversely associated with AD. CONCLUSION: A significant proportion of patients with coronary disease could benefit from interventions aimed at increasing HDL-cholesterol and reducing triglycerides.

Analysis of relapses in anti-NMDAR encephalitis.

OBJECTIVE: The clinical characteristics of patients with relapsing anti-NMDA receptor (NMDAR) encephalitis are not well-defined. In this study, we report the clinical profile and outcome of relapses in a series of anti-NMDAR encephalitis. METHODS: We did a retrospective review of relapses that occurred in 25 patients with anti-NMDAR encephalitis. Relapses were defined as any new psychiatric or neurologic syndrome, not explained by other causes, which improved after immunotherapy or, less frequently, spontaneously. RESULTS: A total of 13 relapses were identified in 6 patients. Four of them had several, 2 to 4, relapses. There was a median delay of 2 years (range 0.5 to 13 years) for the first relapse. Median relapse rate was 0.52 relapses/patient-year. Relapse risk was higher in patients who did not receive immunotherapy in the first episode (p = 0.009). Most cases (53%) presented partial syndromes of the typical anti-NMDAR encephalitis. Main symptoms of relapses were speech dysfunction (61%), psychiatric (54%), consciousness-attention disturbance (38%), and seizures (31%). Three relapses (23%) presented with isolated atypical symptoms suggestive of brainstem-cerebellar involvement. An ovarian teratoma was detected at relapse in only 1 patient (17%). Relapses did not add residual deficit to that caused by the first episode. CONCLUSIONS: Relapses in anti-NMDAR encephalitis are common (24%). They may occur many years after the initial episode. Relapses may present with partial aspects or with isolated symptoms of the full-blown syndrome. Immunotherapy at first episode reduces the risk of relapses.

[Prevalence of metabolic syndrome in patients with stable coronary disease: therapeutic objectives and utilization of cardiovascular drugs]. / Prevalencia de síndrome metabólico en pacientes con enfermedad coronaria estable: objetivos terapéuticos y utilización de fármacos cardiovasculares.

OBJECTIVE: The achievement of the therapeutic objectives in patients with ischemic heart disease and metabolic syndrome is unknown. This study has aimed to evaluate whether the prevalence of risk factors, the prescription rate of evidence-based cardiovascular therapies and the attainment of therapeutic goals differ in coronary patients with and without the metabolic syndrome (MS). METHODS: A multicenter, cross-sectional study carried out with the participation of 7,600 patients with stable coronary heart disease (mean age 65.3 years, 82% males, 37.7% with MS) attended in primary care. Data on drug prescription and goal attainment were extracted from clinical records. MS was defined according to the National Cholesterol Education Program (NCEP) criteria. RESULTS: Patients with MS had a higher prevalence of cardiovascular risk factors and cardiovascular disease. They also had a higher prescription rate of blood-pressure lowering drugs, statins and antidiabetic agents, without differences in the rate of use of antithrombotics and beta-blockers. After adjusting for cardiovascular risk factors and co-morbidity, only fibrates and angiotensin II receptor blockers were used more frequently in MS patients. A lower percentage of subjects with MS achieved therapeutic goals of LDL cholesterol (23.4% vs 27.7%, P<.001), blood pressure (29.1% vs 52.2%, P<.001) and, in diabetics, of glycated hemoglobin (54.7% vs 75.9%, P<.001). CONCLUSION: Patients with stable coronary disease and MS do not reach therapeutic objectives as frequently as those without MS, in spite of receiving a higher amount of cardiovascular drugs.

Prevalencia de síndrome metabólico en pacientes con enfermedad coronaria estable: objetivos terapéuticos y utilización de fármacos cardiovasculares / Prevalence of metabolic syndrome in patients with stable coronary disease: therapeutic objectives and utilization of cardiovascular drugs

Objetivo. La consecución de objetivos terapéuticos en pacientes con cardiopatía isquémica crónica y síndrome metabólico (SM) es desconocida. El objetivo del estudio fue analizar en pacientes con enfermedad coronaria estable si la prevalencia de los factores de riesgo, la utilización de fármacos cardiovasculares y la consecución de objetivos terapéuticos eran diferentes en función de la presencia o no del SM. Métodos. Estudio transversal multicéntrico en el que participaron 7.600 sujetos con enfermedad coronaria estable atendidos en Atención Primaria. Para el diagnóstico del SM se utilizaron los criterios del National Colesterol Educational Programm – Adult Treatment Programm III (NCEP-ATP III). Resultados. La edad media fue 65,3 años (hombres, 82%). El 37,7% cumplía criterios de SM. Los pacientes con SM tenían una significativa mayor prevalencia e intensidad de los factores de riesgo, así como una mayor comorbilidad cardiovascular. Además, utilizaban con mayor frecuencia antihipertensivos, hipolipemiantes e hipoglucemiantes, no existiendo diferencias en antitrombóticos y betabloqueantes. Tras ajustar por los factores de riesgo y la comorbilidad solo los fibratos y los antagonistas del receptor de la angiotensina II eran utilizados más frecuentemente por los pacientes con SM. Los objetivos terapéuticos de colesterol-LDL (23,4% versus 27,7%, p<0,001), de presión arterial (29,1% versus 52,2%, p<0,001) y de hemoglobina glucada en diabéticos (54,7% versus 75,9%, p<0,001), se alcanzaron menos frecuentemente en los pacientes con SM. Conclusión. Los pacientes con enfermedad coronaria estable y SM alcanzan unos objetivos terapéuticos con menor frecuencia que los enfermos sin SM, a pesar de recibir una mayor cantidad de fármacos(AU)

Objective. The achievement of the therapeutic objectives in patients with ischemic heart disease and metabolic syndrome is unknown. This study has aimed to evaluate whether the prevalence of risk factors, the prescription rate of evidence-based cardiovascular therapies and the attainment of therapeutic goals differ in coronary patients with and without the metabolic syndrome (MS). Methods. A multicenter, cross-sectional study carried out with the participation of 7,600 patients with stable coronary heart disease (mean age 65.3 years, 82% males, 37.7% with MS) attended in primary care. Data on drug prescription and goal attainment were extracted from clinical records. MS was defined according to the National Cholesterol Education Program (NCEP) criteria. Results. Patients with MS had a higher prevalence of cardiovascular risk factors and cardiovascular disease. They also had a higher prescription rate of blood-pressure lowering drugs, statins and antidiabetic agents, without differences in the rate of use of antithrombotics and beta-blockers. After adjusting for cardiovascular risk factors and co-morbidity, only fibrates and angiotensin II receptor blockers were used more frequently in MS patients. A lower percentage of subjects with MS achieved therapeutic goals of LDL cholesterol (23.4% vs 27.7%, P<.001), blood pressure (29.1% vs 52.2%, P<.001) and, in diabetics, of glycated hemoglobin (54.7% vs 75.9%, P<.001). Conclusion. Patients with stable coronary disease and MS do not reach therapeutic objectives as frequently as those without MS, in spite of receiving a higher amount of cardiovascular drugs(AU)

Beneficios de la reducción del colesterol unido a lipoproteínas de baja densidad por debajo de 100 mg/dL en los pacientes con enfermedad coronaria / Benefits of LDL-cholesterol reduction below 100 mg/dl in patients with coronary disease

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[Joint presentation of facial hemangioma, posterior fossa malformation, and carotid-vertebral hypoplasia (Pascual-Castroviejo syndrome II): report of 2 new cases]. / Presentación conjunta de hemangioma facial, malformación de fosa posterior e hipoplasia carótido-vertebral (síndrome de Pascual-Castroviejo II): aportación de dos nuevos casos.

INTRODUCTION: The association of external capillary hemangiomas with intracranial malformations (vascular or nonvascular) was first described by Pascual-Castroviejo in 1978. The commonest anomalies found included: Dandy-Walker syndrome, cerebellar hypoplasia, arterial angiomas and alterations in the origin or distribution of the main cerebral arteries. In 1996 the same author named it the 'hemangiovascular complex syndrome'. The syndrome association is very similar, perhaps even identical, to that recently described in the English literature under the heading of PHACE. CLINICAL CASES: We describe two new cases of facial hemangioma, Dandy-Walker type posterior fossa malformation and hypoplasia of the carotid-vertebral trunk ipsilateral to the facial hemangioma. The first patient, a three year old girl had needed a ventriculo-peritoneal shunt for hydrocephalus secondary to a Dandy-Walker malformation. During the third month she had severe symptoms of laryngeal obstruction due to the angiomatous lesion and was satisfactorily treated with corticosteroids. At the present time her psychomotor development seems normal on neurological examination and evaluation. The second patient, a thirteen year old boy, besides showing the characteristic features of this syndrome also had attention-deficit hyperactivity and clumsy movements. In both cases the facial hemangioma was present at birth and gradually became smaller although it did not disappear completely. CONCLUSION: It is important to know about this neurocutaneous syndrome to avoid confusion with similar conditions such as the Sturge-Weber syndrome, so as to carry out suitable clinical investigations: cerebral magnetic resonance, angio-resonance of the intracranial vessels and supra-aortic trunks, arteriography, echocardiography and ophthalmological assessment and to prevent signs of upper respiratory tract obstruction which may be very serious.

Presentación conjunta de hemangioma facial, malformación de fosa posterior e hipoplasia carótido-vertebral (síndrome de Pascual-Castroviejo II): aportación de dos nuevos casos / Association of facial hemiplegia, posterior fossa malformation and carotid-vertebral hypoplasia (Pascual- Castroviejo II syndrome): a report of two new cases

Introducción. La asociación de hemangiomas capilares externos con malformaciones intracraneales (vasculares y no vasculares) fue descrita por primera vez por Pascual-Castroviejo en 1978, siendo las anomalías más frecuentemente encontradas: síndrome de Dandy-Walker, hipoplasia cerebelosa, angiomas arteriales y alteraciones en el origen o la distribución de las arterias cerebrales principales. En 1996 este mismo autor lo denomina `síndrome complejo hemangiovascular'. Esta asociación sindrómica es muy parecida, o quizás idéntica, a la descrita recientemente con el acrónimo PHACE en la literatura anglosajona. Casos clínicos. Aportamos dos nuevos casos que presentan hemangioma facial, malformación de fosa posterior de tipo Dandy-Walker e hipoplasia del eje carotidovertebral ipsilateral al hemangioma facial. El primero de ellos, una niña de 3 años, precisó en los primeros meses de vida una derivación ventriculoperitoneal por la hidrocefalia secundaria a su malformación de Dandy-Walker. En el tercer mes presentó síntomas graves de obstrucción laríngea por componente angiomatoso a ese nivel, evolucionando favorablemente con corticoterapia. Actualmente la exploración neurológica y su desarrollo psicomotor es normal. El segundo, un varón de 13 años, presenta, además de los hallazgos característicos Pascual-Castroviejo I es la displasia cerebro-facio-torácica descrita por este autor en 1975 [13].Nuestros dos pacientes son dos nuevos ejemplos de este síndrome, con unas características muy similares entre sí, pues ambos presentan hemangioma capilar facial, malformación de fosa posterior e hipoplasia de arterias carótida y vertebral ipsilateral al hemangioma. Podríamos considerarlos como pacientes tipo, ya que las otras malformaciones suelen ser menos frecuentes (cardiopatía congénita, anomalías oculares y defectos de cierre abdominal).Por último, recomendamos en todo niño con un hemangioma facial (capilar o cavernoso) la realización de RM cerebral, angio-RM de vasos intracraneales y troncos supraórticos o arteriografía, ecocardiografía y valoración oftalmológica. Debe vigilarse también en estos niños la posible aparición de síntomas de obstrucción de las vías respiratorias altas (estridor, cianosis, dificultad respiratoria), dada la frecuente extensión de estos hemangiomas a la laringe y la parte superior de la traquea, que pueden llegar a comprometer su vida, como ocurrió en uno de nuestros pacientes y en otros casos publicados [5,10]. de este síndrome, un cuadro de déficit de atención con hiperactividad y torpeza motora. En ambos el hemangioma facial estaba presente en el nacimiento y fue desapareciendo lentamente, pero sin llegar a suprimirse de forma completa. Conclusión. Es importante el conocimiento de este síndrome neurocutáneo para evitar la confusión con cuadros similares como el síndrome de Sturge-Weber, para poder realizar las pruebas complementarias adecuadas: resonancia magnética cerebral, angiorresonancia de vasos intracraneales y troncos supraórticos o arteriografía, ecocardiografía y valoración oftalmológica, y para prevenir la aparición de síntomas de obstrucción de vías altas que pueden ser muy graves (AU)

Malformación arteriovenosa en la neurofibromatosis tipo I.Caso clínico y revisión de la literatura / Arteriovenous malformation in neurofibromatosis type I. A case report and review of the literature

Introducción. La neurofibromatosis tipo I puede acompañarse de complicaciones cerebrovasculares, principalmente estenosis y aneurismas, y más raramente fístulas arteriovenosas vertebrales y malformaciones. Presentamos el primer caso infantil de conocemos con neurofibromatosis tipo I y hemorragia subaracnoidea originada por la ruptura de una malformación arteriovenosa. Caso clínico. Se trata de una niña de 9 años de edad, que sufre un cuadro de inicio agudo precedido de vómitos, de deterioro del nivel de conciencia y rigidez de nuca, motivada por una hemorragia subaracnoidea e intraventricular. En la angiografía se demostró la existencia de una malformación arteriovenosa interpeduncular derecha dependiente de la arteria coroidea anterior de dicho lado. La paciente requirió estabilización en UVI e intervención quirúrgica con buena evolución. Conclusiones. Revisamos las complicaciones cerebrovasculares asociadas a la neurofibromatosis tipo I descritas en la literatura. La neurofibromatosis tipo I puede asociarse a complicaciones cerebrovasculares, siendo las más frecuentes las oclusivas o estenóticas, aisladas o con un patrón vascular de enfermedad oclusiva arterial cerebral progresiva tipo moyamoya, y aneurismas, aunque también estás descritos en al literatura otras más raras como fístulas y malformaciones arteriovenosas. La paciente que hemos presentado es el primer caso de malformación vascular en niños asociada a neurofibromatosis tipo I del que tengamos conocimiento (AU)

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Cholesterol reduction improves myocardial perfusion abnormalities in patients with coronary artery disease and average cholesterol levels.

OBJECTIVES: We sought to evaluate whether pravastatin treatment increases myocardial perfusion, as assessed by thallium-201 single-photon emission computed tomographic (SPECT) dipyridamole testing, in patients with coronary artery disease (CAD) and average cholesterol levels. BACKGROUND: Previous studies in hypercholesterolemic patients have demonstrated that cholesterol reduction restores peripheral and coronary endothelium-dependent vasodilation and increases myocardial perfusion. METHODS: This was a randomized, placebo-controlled study with a cross-over design. Twenty patients with CAD were randomly assigned to receive 20 mg of pravastatin or placebo for 16 weeks and then were crossed over to the opposite medication for a further 16 weeks. Lipid and lipoprotein analysis and dipyridamole thallium-201 SPECT were performed at the end of each period. The SPECT images were visually analyzed in eight myocardial segments using a 4-point scoring system by two independent observers. A summed stress score and a summed rest score were obtained for each patient. Quantitative evaluation was performed by the Cedars-Sinai method. The magnitude of the defect was expressed as a percentage of global myocardial perfusion. RESULTS: Total and low density lipoprotein cholesterol levels during placebo were 214 +/- 29 mg/dl and 148 +/- 25 mg/dl, respectively. These levels with pravastatin were 170 +/- 23 mg/dl and 103 +/- 23 mg/dl, respectively. The summed stress score and summed rest score were lower with pravastatin than with placebo (7.2 +/- 2.3 vs. 5.9 +/- 2.3, p = 0.012 and 3.2 +/- 1.6 vs. 2.4 +/- 2.2, p = 0.043, respectively). Quantitative analysis showed a smaller perfusion defect with pravastatin (29.2%) as compared with placebo (33.8%) (p = 0.021) during dipyridamole stress. No differences were found at rest. CONCLUSIONS: Reducing cholesterol levels with pravastatin in patients with CAD improves myocardial perfusion during dipyridamole stress thallium-201 SPECT.

[Arteriovenous malformation in neurofibromatosis type 1. A case report and review of the literature]. / Malformación arteriovenosa en la neurofibromatosis tipo I. Caso clínico y revisión de la literatura.

INTRODUCTION: Neurofibromatosis type I may be accompanied by cerebrovascular complications, mainly stenosis or aneurysms and more rarely vertebral arteriovenous fistulas and malformations. We report the first case of a child, as far as we know, with neurofibromatosis type I and subarachnoid hemorrhage caused by rupture of an arteriovenous malformation. CLINICAL CASE: A 9 year old girl presented with the acute onset of a condition preceded by vomiting, deterioration in her level of consciousness and neck rigidity caused by subarachnoid and intraventricular hemorrhage. On angiography a right interpeduncular arteriovenous malformation was seen which arose from the right anterior choroid artery. The patient was admitted to the intensive care unit for stabilization of her condition, followed by surgical operation and good subsequent progress. CONCLUSIONS: We review the cerebrovascular complications associated with neurofibromatosis type I that have been described in the literature. Neurofibromatosis type I may be associated with cerebrovascular complications. The most frequent of these are occlusive or stenotic, isolated or with a vascular pattern of progressive cerebral artery occlusive disease of moyamoya type and aneurysms. Other rarer cerebrovascular complications include fistulas and arteriovenous malformations. The patient we report is the first case of vascular malformation in children associated with neurofibromatosis type I as far as we are aware.

Effectiveness of low-dose crystalline nicotinic acid in men with low high-density lipoprotein cholesterol levels.

BACKGROUND: Hypoalphalipoproteinemia (low serum concentration of high-density lipoprotein cholesterol [HDL-C]) is a common pattern of dyslipidemia associated with coronary heart disease. High doses of nicotinic acid effectively raise HDL-C levels in this condition, but they are commonly accompanied by side effects. The efficacy of low doses of nicotinic acid that may produce fewer side effects has not been adequately studied. OBJECTIVE: To determine the effects of low-dose nicotinic acid on HDL-C levels in patients with hypoalphalipoproteinemia. METHODS: Forty-four men with low HDL-C levels (< 1.03 mmol/L [< 40 mg/dL]) entered the study. Twenty-four patients otherwise had normal lipid levels, and 20 were moderately hypertriglyceridemic (range of plasma triglyceride levels, 2.82 to 5.64 mmol/L 250 to 500 mg/dL). The trial consisted of 3 phases; each phase lasted 8 weeks. The first phase was diet only (30% fat diet); in the second phase, crystalline nicotinic acid was added at 1.5 g/d; and in the third phase, the dose was increased to 3 g/d. RESULTS: Of the 44 patients who entered the study, 37 completed the low-dose phase (1.5 g/d); the remaining patients were withdrawn because of side effects to nicotinic acid. Four other patients who completed the low-dose phase were excluded from the higher dose phase because of side effects that developed when they were receiving the low dose. Ten other patients withdrew during the high-dose phase because of side effects. In both groups, responses to nicotinic acid therapy tended to be dose-dependent. For both groups, the higher dose generally produced a greater reduction in apolipoprotein B-containing lipoproteins and a greater rise in HDL-C levels. However, for both groups, the low dose of nicotinic acid gave an average 20% increase in HDL-C levels. CONCLUSIONS: A low dose (1.5 g/d) of crystalline nicotinic acid causes an average 20% increase in HDL-C levels and significantly lowers triglyceride levels in both normolipidemic and hyperlipidemic patients with low HDL-C levels. Although the changes induced by this dose are less than those that can be achieved by a higher dose, the lower dose is better tolerated. Nicotinic acid may be useful in combined drug therapy for secondary prevention of coronary heart disease, and if higher doses cannot be tolerated, use of a lower dose should still be useful for producing a moderate rise in HDL-C levels in patients with hypoalphalipoproteinemia.

[Urate production in a porcine model of myocardial ischemia-reperfusion]. / Producción de urato en un modelo porcino de isquemia-reperfusion miocárdica.

OBJECTIVE: This study was designed to investigate urate production by swine hearts using an in vivo regionally ischemic-reperfused model. ANIMALS AND METHODS: Ten female pigs underwent 60 minutes of myocardial ischemia by clamping of the left anterior descending artery and afterwards 120 minutes of reperfusion. Epicardial biopsies and blood samples from coronary sinus were taken before ligation, at the end of ischemic period and 5, 30, 60 and 120 minutes upon reperfusion. RESULTS: During ischemia, tissue levels of ATP and ADP greatly declined with a subsequent increase in the concentration of AMP, inosine and hypoxanthine (33 +/- 12 vs 93 +/- 17, 26 +/- 8 vs 768 +/- 86 and 32 +/- 10 vs 219 +/- 26 nmol/g dry weight, p < 0.01 for each). Despite the great increase in the hypoxanthine levels, uric acid concentration remained constant (69 +/- 9 vs 32 +/- 12 nmol/g dry weight, NS). Hypoxanthine, xanthine and uric acid concentrations increased in blood samples obtained from the coronary sinus at the end of ischemic period (17.99 vs 31.03 nmol/ml, p < 0.01, 0.29 vs 1.45 nmol/ml, p < 0.05 and 1.20 vs 2.31 nmol/ml, p < 0.01 respectively) and were enhanced upon reperfusion (35.8 and 3.89 nmol/ml for hypoxanthine and uric acid respectively, p < 0.05) without any significant modifications in their concentrations at the arterial level. CONCLUSION: These results demonstrate that the ischemic-reperfused swine heart produces urate probably outside the myocardium.