Cardiothoracic surgery and peripheral endovascular intervention in cardiovascular damage from a cohort of orphan rheumatological diseases-epidemiological and survival analysis.

Background: Aortic diseases in some orphan rheumatological diseases require medical, surgical or peripheral endovascular intervention because they can be catastrophic. Objectives: to analyze the main clinical and epidemiological characteristics of patients with Takayasu arteritis (TA), Marfan syndrome (MS) and similar conditions that were treated with cardiothoracic surgery and peripheral endovascular intervention. Methods: Retrospective and descriptive cohort study that included patients of any age and gender with TA (as per the criteria of the American College of Rheumatology and EULAR/PRINTO), MS (according to Ghent criteria), and similar conditions who underwent cardiothoracic surgery or peripheral endovascular intervention. Data were collected from electronic charts. Results: A total of 77 patients with TA and 135 patients with MS and similar conditions were included. The frequency of surgical or interventional requirements in patients with TA and MS/similar conditions was 77/364 (21.2%) and 135/300 (45%), respectively; such patients were followed for a median of 6 [2-12] and 3.29 (0.42-6.62) years, with (maximum follow-up range of 47 and 21.37 years, respectively). Aneurysms were present in 11 (14.3%) and 66 (48.9%) in patients with TA and MS/similar conditions, respectively. Aortic, mitral and tricuspid valve damage occurred in 8 (10.4%) patients, 4 (5.2%) patients and 1 (1.3%) patient with TA, respectively; corresponding frequencies in patients with MS/similar conditions were 98 (72.6%), 50 (37.0%) and 20 (14.8%). We identified that 20% of patients with TA died after 5.08 years (95% CI: 0.23-25.42 years) and 20 % of the patients with MS and other similar conditions died after 7.52 years (95% CI: 1.10-9.02 years). Conclusions: The frequency of surgical intervention was low in this study. Long-term prognosis is good if surgery is performed in a timely manner. Epidemiological studies provide relevant information for public health decisions related to the management of orphan rheumatological diseases.

Obesity, Thrombotic Risk, and Inflammation in Cancer.

Neoplasms exhibits a high incidence and mortality rates due to their complex and commonly overlapping clinical, biochemical, and morphologic profiles influenced by acquired or inherited molecular abnormalities, cell of origin, and level of differentiation. Obesity appears related to ~20% of cancers including endometrial, esophageal, colorectal, postmenopausal breast, prostate, and renal. Several factors other than obesity, i.e., insulin, insulin-like growth factor, sexual hormones, and adipokines may play a potential role in neoplasia. Cancer-associated hypercoagulable and thrombotic states are influenced by abnormalities in the vascular wall and susceptibility to invasion, interference in blood flow and increase in circulating tissue factor and thrombin, activation of cell growth factors, the presence of a central catheter, chemotherapies, neoplasm type, and surgery. In cancer, thromboembolic complications are the second most frequent cause of death with pulmonary thromboembolism in ~50% of cases postmortem. Thrombosis worsens prognosis as demonstrated with a survival rate as low as 12% per year vs 36% in nonthrombic patients. Deep vein thrombosis is the most frequent thromboembolic complication in cancer. It is usually detected at diagnosis and within the first 3 months of chemotherapy. The underlining mechanisms of this association should be further studied to identify patients at higher risk and develop adequate prevention, diagnostic, and treatment measures. The D-dimer test can be successfully used to assess the fibrinolytic phase of coagulation and as such is routinely used in suspected cases of deep vein thrombosis and pulmonary thromboembolism. In addition, significant advances have been made in understanding the composition and functional capabilities of the gut microbiota in the inflammatory process, obesity, and its roles in cancer; however, the intricate balance that exists within the microbiota may not only affect the host directly, it can also disrupt the entire microbial community. CONCLUSIONS: Cancer is a prothrombotic and inflammatory state in which the activation of coagulation is related to tumor growth, angiogenesis, and metastasis. It is important to identify the relationship between body mass index with these processes and clarify their importance in cancer prognosis. Future research should answer the question if manipulation of resident microbial communities could potentially improve prognosis and treatment outcome.

The clinical significance of coagulation and the inflammatory response in autoimmunity.

Maintenance of normal blood flow requires equilibrium between procoagulant and anticoagulant factors; occasionally, procoagulant activity predominates, leading to clot formation; frequently, tissue damage is the triggering factor. Hereditary factors, primary or acquired, play a role in the development of thrombosis. Primary thrombophilia is associated with hereditary factors, which promote hypercoagulability because natural anticoagulants are not exerting their activity. On the other hand, acquired thrombophilia may occur associated to autoimmune diseases, cancer, surgical procedures, pregnancy, postpartum period, and obesity. Activation of the coagulation system is characterized by the co-participation of inflammatory response components, factors related to the subjacent disease, and other procoagulant factors. The study of patients with thrombosis should include both inflammatory and autoimmune response markers.

Algunos dilemas éticos en la práctica médica / Some ethical dilemmas in the medical praxis

Con la intención de prevenir conflictos en el acto médico y desde el punto de vista bioético, éste, el manejo paliativo y de la muerte digna; situaciones que pueden ser fuente de demandas entre el paciente y el médico. Sostenemos que la actitud y aptitud del médico, del paciente, su familia, amigos y representantes legales, con apertura y honestidad, pueden prevenir la gran mayoría de las causas de conflicto y evitar las consecuencias del mismo entre los profesionales de la salud y los enfermos. La prevención es posible si hay buena voluntad y conocimiento de normas, leyes, usos y sentido común.

We reviewed from a bioethical perspective and attempting prevention of potential conflicts derived communication failure during medical practice, palliative treatments and dignified death in the institutional practice as well as general practice; most of conflicts related to patient-doctor relationship could de prevented. We propose an attitude and aptitude plus in deep knowledge of patient, family, friends and legal representatives in terms fully honest communication to prevent most of conflicts and avoid its consequences against doctors and other health workers. Prevention is better and it depends of knowledge of norms, laws, general beliefs and common sense in this country and maybe others.

Performance levels of four Latin American laboratories for the serodiagnosis of Chagas disease in Mexican sera samples

In nearly all of the previous multicentre studies evaluating serological tests for Trypanosoma cruzi infection, sera samples from Central or South American countries have been used preferentially. In this work we compared the reliability of the serological tests using Mexican sera samples that were evaluated in four independent laboratories. This included a reference laboratory in Brazil and three participant laboratories, including one in Central America and two in Mexico. The kappa index between Brazilian and Honduran laboratories reached 1.0 and the index for the Mexican laboratories reached 0.94. Another finding of this study was that the source of antigen did not affect the performance of the serological tests.

Comparison distribution of HLA-B alleles in mexican patients with takayasu arteritis and tuberculosis.

A possible relationship between Takayasu arteritis (TA) and Tuberculosis (Tb) has been suggested. Both diseases present similar chronic inflammatory lesions and occasionally granulomas on the arterial walls. The genetic relationship between these two diseases has not been explored before, however, both diseases have been associated with human leukocyte antigen (HLA) alleles. Therefore, the aim of the present study was to analyze the distribution of HLA-B alleles in TA (n = 40) and Tb (n = 34) patients and healthy controls (72 exposed and 99 nonexposed). HLA-B alleles were determined by reverse dot blot. The statistical methods used included the Chi(2), and odds ratio (OR) with 95% confidence intervals. In spite of the loose clinical relationship between TA and Tb, we did not detected any genetic relationship between them when the HLA-B alleles were analyzed in these groups of patients. On the contrary, we detected distinct specific HLA-B alleles for each disease. TA was characterized by HLA-B39, -B44, and -B52, pulmonary Tb by HL-B35 and extrapulmonary Tb by HLA-B39 and -B40. This preliminary study suggests a difference in the distribution of HLA-B alleles in patients with TA and Tb.

Pre-pulseless Takayasu's arteritis evaluated with 18F-FDG positron emission tomography and gadolinium-enhanced magnetic resonance angiography.

Takayasu's arteritis is a primary vasculitis that affects large vessels and is characterized by chronic granulomatous inflammation. Diagnosis has been primarily clinical, with verification by angiography as the gold standard. More recently, however, it has become apparent that positron emission tomography enables better evaluation of vascular inflammation. This study presents 2 cases of Takayasu's arteritis. Magnetic resonance angiography was used to evaluate aortic anatomy by analyzing vascular wall thickness and also to quantify disease activity by measuring gadolinium enhancement. Positron emission tomography was used to evaluate active vascular inflammation by quantifying fluorodeoxyglucose F18 uptake. We conclude that both techniques support clinical diagnosis and aid in the evaluation of disease activity during and after treatment.

Cardiopatía chagásica crónica en el Hospital General de Zona No 24 IMSS. Poza Rica, Veracruz / Chronic Chagasic Cardiomyopathy at the Hospital General de Zona no. 24 IMSS. Poza Rica, Veracruz

Northern Veracruz has conditions, biotic and abiotic, to support Triatomine bugs and vectorial transmission of Trypanosoma cruzi to human beings. Therefore we explore seroprevalence of antibodies to this parasite and the presence of Chronic Chagasic Cardiopathy (CCC) at Cardiology ward in a General Hospital serving North of Veracruz State, and neighbord states Hidalgo, Puebla San Luis Potosi and Tamaulipas. MATERIAL AND METHODS: We search for consecutive adult patients attending outpatient and beds assigned to Cardiology between March through September, 2003. An epidemiology questionnaire, clinical work up, chest roentgenogram, 12 lead peripheral EKG and transthoracic echocardiogram were performed in 240 female/males patients. All of them were bled to blindly search for T. cruzi antibodies. RESULTS: Seroprevalence was 8%, 49 cases of dilated cardiomyopathy were diagnosed 23 attributed to chronic diseases such as systemic hypertension diabetes mellitus or ischemic heart disease 12 with idiopathic disease and 14 (29%) had CCC. The latter accumulated epidemiologic features suggestive of vectorial infection. Four additional individuals without CCC but having specific antibodies were considered indeterminate Chagasic cases. DISCUSSION AND CONCLUSIONS: This case series identify American Trypanosomiasis among 19 people attending a Cardiology Service, and 14 of them had a severe heart disease linked to progressive and fatal course. This observation points out that Chagas disease could be a regional public health problem in Northern Veracruz.

Panorama epidemiológico y clínico de la cardiopatía chagásica crónica en México / Epidemiological and clinical outlook of chronic Chagas' heart disease in Mexico

OBJECTIVO: Comparar las características epidemiológicas y clínicas de la cardiopatía chagásica crónica con otras miocardiopatías dilatadas. MÉTODOS: Se incluyeron a 128 pacientes consecutivos en un hospital de espcialidad, de 1993 a 2003 con miocardiopatías dilatadas, donde 51 (40 por ciento) con anti Tripanosoma cruzi. Se recopiló información epidemiológica por entrevista directa, y datos clínicos en los servicios asistenciales. Se utilizaron la prueba de la Chi-cuadrado o prueba exacta de Fischer, prueba t de Student ó la prueba de U de Mann Whitney y análisis multivariado. RESULTADOS: Los pacientes con cardiopatía chagásica crónica, eran más viejos (55±10 años) que los pacientes con miocardiopatías (42±17 años), nacieron en zonas rurales (90 por ciento vs 68 por ciento), en viviendas precarias (75 por ciento vs 16 por ciento), con hacinamiento (45 por ciento vs 20 por ciento), convivencia con animales domésticos (71 por ciento vs 61 por ciento) y conocían al vector (73 por ciento vs 25 por ciento). Los trastornos del ritmo y de la conducción, así como la colocación de marcapaso definitivo fueron frecuentes en los pacientes con cardiopatía chagásica crónica (84 por ciento vs 55 por ciento, 78 por ciento vs 64 por ciento Y 24 por ciento vs 10 por ciento respectivamente). La insuficiencia cardiaca congestiva venosa fue más frecuente en los pacientes con miocardiopatía seronegativa (88 por ciento vs 71 por ciento) y la perfusión miocárdic anormal con arterias epicárdicas normales fue igual en ambos grupos. Con respecto a co-morbilidad, los pacientes con cardiopatía chagásica crónica tenían sólo dos padecimientos, mientras que en el otro grupo era más amplia. CONCLUSIONES: La enfermedad de Chagas causa la miocardiopatía dilatada específica más común. Debido a su distribución regional en la República Mexicana, merece atención y se recomienda a nivel público adoptar medidas de prevención que ya probaron eficacia en otros países.

Miocarditis aguda primaria. Experiencia de diez años en el Instituto Nacional de Cardiología "Ignacio Chávez" / Primary Acute Myocarditis. A 10- years institutional experience at the National Institute of Cardiology "I. Chavez"

La miocarditis aguda (MA) se asocia a infecciones virales: Coxsackie B, ECHOvirus y otros. Mecanismos autoinmunes se suponen patogénicos. La clínica es variable. El beneficio de inmunosupresión clásica (prednisona-azatioprina) o inmunomodulación (IgG-monomérica) no ha sido confirmado. Objetivo: Revisar la incidencia y enfoque de estudio de la miocarditis. Material y métodos: De 1992-2003, aplicamos un cuestionario estándar a 49 pacientes consecutivos con diagnóstico establecido en forma independiente. Se excluyeron MA con causa identificable. Resultados: La MA ocurrió en 17 mujeres y 32 hombres, edad (mediana) 24 y 28 años. Predominaron disnea/ortopnea (70-47%), edema/plétora yugular (41-37%), dolor precordial y taquicardia (50%). En 22 (45%) la clase funcional era III-IV. El electrocardiograma mostró taquicardia sinusal (35%) y bloqueos de rama (24%). En 47 pacientes el ecocardiograma mostró FEVI 41% (promedio), DDVI 53 mm (promedio) y discinesia 89%. En 11 (22%) casos investigamos anticuerpos contra Coxsackie/ECHOvirus, 6 (54%) tenían anticuerpo reconocido. Veintinueve casos (61%) progresaron a miocardiopatía dilatada (MD), hubo tres defunciones (6%). No fue posible valorar tratamiento inmunomodulador, se estudió sólo a 12 casos. Conclusiones: La incidencia de MAes 1/1,000 ingresos/año. Es necesario estandarizar manejo diagnóstico y tratamiento, pues la progresión a MD y/o muerte en etapa aguda ocurre en 2/3 de los pacientes.

Acute myocarditis (AM) is associated with viral infections: Coxsackie and ECHOviruses among others. Autoimmunity has been proposed as a pathogenic mechanism. Benefit of classic immunosuppression (prednisone-azathioprine) or immunomodulation (monomeric-human IgG) is still uncertain. Objective: To review incidence and clinical approach to AM at a Cardiology referral center. Material and methods: A 10-yeard period (1992-2003) is reviewed. A standard ques-tionary was applied to 49 consecutive patients referred by clinicians with a diagnosis of AM. Results: AM was found in 17 women and 32 men, median age 24 and 28 years, respectively. They presented heart failure with dyspnea/ortop-nea (70-47%), peripheral edema/jugular vein plethora (41-37%), chest pain, and tachycardia (50%), NYHA functional class was III-IV in 22. The EKG showed sinus tachycardia or conduction defects. Transthoracic echocardiograms in 47 cases showed EF (mean) of 41% with enlarged left ventricle diameter. Antivirus antibodies were present in 54% of those cases studied, Coxsackie or ECHOvirus were identified through a serologic assay. Twenty-nine (61%) of our cases developed dilated cardiomyopathy, three patients died. It is not possible to reach a conclusion regard to immunomodulation therapy, because it was applied to only 12 patients. Conclusions: At the Instituto Nacional de Cardiología "I. Chávez", AM depicts an incidence of 1/1,000 patients a year. It is necessary to standardize the clinical approach for diagnosis and treatment, progression to dilated cardiomyopathy and deadth during acute stage occurs in two-thirds of our patients.

Anticuerpos contra Trypanosoma cruzi en pacientes con miocardiopatía dilatada en Tuxtla Gutiérrez, Chiapas / Antibodies against Trypanosoma cruzi in patients with dilated cardiomyopathy in Tuxtla Gutierrez, Chiapas

La enfermedad de Chagas es causada por el protozoario flagelado Trypanosoma cruzi. Encuestas seroepidemiológicas realizadas en Chiapas informan individuos seropositivos en comunidades rurales, y cabe la posibilidad de que la forma crónica de la enfermedad ocurra en el estado. La expresión clínica dominante en la enfermedad de Chagas crónica es cardíaca. Material y métodos: Con el objeto de conocer la frecuencia de cardiopatía chagásica crónica (CCC) se hizo una encuesta serológica a pacientes con miocardiopatía dilatada (MD) internados entre octubre de 2002 a octubre de 2003, en el Servicio de Cardiología del Hospital General Regional "Dr. Rafael Pascacio Gamboa" de Tuxtla Gutiérrez, Chiapas. El diagnóstico fue establecido en forma independiente y el estudio seroinmunológico fue ciego. Resultados: Quince (54%) de 28 sujetos con MD tenían anticuerpos a T. cruzi. Todos provenían de medio rural pobre, su cuadro clínico incluyó insuficiencia cardíaca y/o trastornos del ritmo o conducción. Conclusiones: Esta evidencia sugiere que en Chiapas, una zona endémica de enfermedad de Chagas, hay CCC. Dentro del estudio de la MD, la etiología chagásica, debe ser considerada, ya que las condiciones socioeconómicas, culturales y regionales favorecen su presencia.

Chagas disease is caused by the flagellate protozoan T. cruzi. Seroepidemiological surveys in Chiapas, Mexico have shown seropositive individuals, therefore, we searched for people affected by the chronic form of Chagas disease which involves the heart, causing a chronic, progressive and fatal disease called Chronic Chagasic Cardiopathy (CCC). Material and methods: To establish the frequency of CCC we studied 28 patients seen at the Hospital General Regional "Dr. Rafael Pascacio Gamboa" during October 2002 through October 2003 in Tuxtla Gutierrez, Chiapas, the State capital city, with diagnosis of dilated cardiomyopathy (DC), a serological survey for antibodies against T. cruzi was done. This hospital cares for people from all parts of Chiapas, Mexico. Clinical diagnosis of DC was established there and blind serological studies were performed in Mexico City. Results: Fifteen out of 28 DC patients (54%) had anti T. cruzi antibodies. All of them came from poor rural villages and they had heart failure and/or arrhythmia or heart blockade on EKG. Conclusions: This observation suggest that in Chiapas were Chagas disease is endemic, there are CCC patients. Any case with a clinical diagnosis of DC should be tested for antibodies against T. cruzi. The low socioeconomic status, culture and environment in this Mexican State favour the presence and transmission of this parasitic disease.

Trypanosoma cruzi isolates from Mexican and Guatemalan acute and chronic chagasic cardiopathy patients belong to Trypanosoma cruzi I

Trypanosoma cruzi is classified into two major groups named T. cruzi I and T. cruzi II. In the present work we analyzed 16 stocks isolated from human cases and four isolated from triatomines from diverse geographical origins (Mexico and Guatemala). From human cases four were acute cases, six indeterminates, and six from chronic chagasic cardiophatic patients with diagnosis of dilated cardiomyopathy established based on the left-ventricular end systolic dimension and cardiothoracic ratio on chest X-radiography and impaired contracting ventricle and different degree conduction/rhythm aberrations. DNA samples were analyzed based on mini-exon (ME) polymorphism, using a pool of three oligonucleotide for the amplification of specific intergenic region of T. cruzi ME gene. All the Mexican and Guatemalan isolates regardless their host or vector origin generated a 350 bp amplification product. In conclusion T. cruzi I is dominant in Mexico and Guatemala even in acute and chronic chagasic cardiopathy patients. To our knowledge, this is the first study describing predominance of T. cruzi I in human infection for North and Central America.

Factores bióticos y abióticos que determinan la seroprevalencia de anticuerpos contra Trypanosoma Cruzi en el municipio de Palmar de Bravo, Puebla, México / Biotic and abiotic determinants of seroprevalence of antibodies against Trypanosoma cruzi in Palmar de Bravo, Puebla, Mexico

OBJETIVO: Determinar la prevalencia de anticuerpos contra Trypanosoma cruzi y su relación con los factores bióticos y abióticos en Palmar de Bravo, Puebla, México. MATERIAL Y MÉTODOS: Estudio transversal efectuado en agosto de 2000 a septiembre de 2001, con una muestra aleatoria simple de 390 voluntarios residentes en Palmar de Bravo, Puebla, México. Se hizo determinación de anticuerpos contra T cruzi con técnicas serológicas validadas, búsqueda del vector y de reservorios domésticos, así como determinación de asociación entre caso positivo y factores de riesgo bióticos y abióticos. El análisis estadístico consistió en índice Kappa para las pruebas diagnósticas, empleando tabla de contingencia de 2 x 2; ji cuadrada corregida de Yates, exacta de Fisher y la razón de posibilidad para estimar la significancia de la asociación de factores bióticos y abióticos. RESULTADOS: La seroprevalencia fue de 4 por ciento en la población humana estudiada y de los reservorios (equinos, porcinos y caninos), sólo 10 por ciento de los caninos resultaron reactivos. Los vectores identificados fueron T barberi y T pallidipennis, con índice de dispersión e índice de colonización de 55 y 40 por ciento, respectivamente. Los factores de riesgo más importantes fueron la altitud (>2 150 y <2 180 metros sobre el nivel del mar), los años de residencia, el pertenecer a un programa de asistencia social, la presencia de triatóminos y la edad. CONCLUSIONES: En localidades ubicadas a una altitud mayor a los 2 000 metros sobre el nivel del mar se reconocieron vectores infectados con T cruzi, casos humanos y probablemente reservorios domésticos.

Tumor necrosis factor-alpha promoter polymorphisms in Mexican patients with rheumatic heart disease.

The major histocompatibility genes (MHC) have been associated with the genetic susceptibility to rheumatic heart disease (RHD). Results have been inconsistent and new genes located on the MHC region such as tumor necrosis factor (TNF-alpha) need to be analyzed. TNF-alpha polymorphisms (positions -238 and -308) were determined in 87 RHD Mexican Mestizo patients and 101 healthy controls. Patients were classified into mitral valve damage (MVD) and multivalvular lesion (MVL) categories. TNF-238 G allele and GG genotype were increased in patients when compared to healthy controls (pC=0.001, OR=14.1 and pC=0.003, OR=14.1, respectively). Also, decreased frequencies of TNF-238 A allele (pC=0.001) and AG genotype (pC=0.003) were found. TNF-308 polymorphism analysis showed increased frequencies of T2 (A) allele (pC<10(-3), OR=10.8) and T1T2 (AG) genotype (pC<10(-3), OR=9.85) and decreased frequencies of T1 (G) allele and T1T1 (GG) genotype (pC<10(-3)). When comparing valvular damage to healthy controls, patients with MVD showed increased frequencies of -238 GG (pC=0.03, OR=ND), -308 T1T2 (AG) (pC<10(-3), OR=14) and -308 T2 (A) (pC<10(-3), OR=11.7). Also, this group showed decreased frequencies of T1 (G) allele and T1T1 (GG) genotype (pC<10(-3)). Patients with MVL presented increased frequency of -308 T2 (A) allele (pC=0.0003, OR=8.65) and decreased frequencies of -308 T1 (G) allele and -308 T1T1 (GG) genotype (pC=0.0003 and pC=0.006, respectively). Distribution of -238 and -308 polymorphisms were similar between MVD and MVL. The data demonstrate that RHD is associated with TNF-alpha polymorphisms in the Mexican population; however, these polymorphisms do not have relation with the valve damage.

Electrocardiographic findings in Mexican chagasic subjects living in high and low endemic regions of Trypanosoma cruzi infection

In México the first human chronic chagasic case was recognized in 1940. In spite of an increasing number of cases detected since that time, Chagas disease in México has been poorly documented. In the present work we studied 617 volunteers subjects living in high and low endemic regions of Trypanosoma cruzi infection with seroprevalence of 22 percent and 4 percent respectively. Hemoculture performed in those seropositive subjects failed to demonstrate circulating parasites, however polymerase chain reaction identified up to 60 percent of them as positives. A higher level of anti-T. cruzi antibodies was observed in seropositive residents in high endemic region, in spite of similar parasite persistence (p < 0.05). On standard 12 leads electrocardiogram (ECG) 20 percent to 22 percent seropositive individuals from either region showed right bundle branch block or ventricular extrasystoles which were more prevalent in seropositive than in seronegative individuals (p < 0.05). In conclusion, the frequency or type of ECG abnormality was influenced by serologic status but not by endemicity or parasite persistence. Furthermore, Mexican indeterminate patients have a similar ECG pattern to those reported in South America