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.

Personal Guides: Heterogeneous Robots Sharing Personal Tours in Multi-Floor Environments.

GidaBot is an application designed to setup and run a heterogeneous team of robots to act as tour guides in multi-floor buildings. Although the tours can go through several floors, the robots can only service a single floor, and thus, a guiding task may require collaboration among several robots. The designed system makes use of a robust inter-robot communication strategy to share goals and paths during the guiding tasks. Such tours work as personal services carried out by one or more robots. In this paper, a face re-identification/verification module based on state-of-the-art techniques is developed, evaluated offline, and integrated into GidaBot's real daily activities, to avoid new visitors interfering with those attended. It is a complex problem because, as users are casual visitors, no long-term information is stored, and consequently, faces are unknown in the training step. Initially, re-identification and verification are evaluated offline considering different face detectors and computing distances in a face embedding representation. To fulfil the goal online, several face detectors are fused in parallel to avoid face alignment bias produced by face detectors under certain circumstances, and the decision is made based on a minimum distance criterion. This fused approach outperforms any individual method and highly improves the real system's reliability, as the tests carried out using real robots at the Faculty of Informatics in San Sebastian show.

Recombinant Enolase of Trypanosoma cruzi as a Novel Vaccine Candidate against Chagas Disease in a Mouse Model of Acute Infection.

Trypanosoma cruzi is the protozoan parasite that causes Chagas disease, which is considered by the World Health Organization to be a neglected tropical disease. Two drugs exist for the treatment of Chagas disease, nifurtimox and benznidazole; they are only effective in the acute phase, and a vaccine is currently not available. In this study, we used the recombinant enolase from T. cruzi H8 strain (MHOM/MX/1992/H8 Yucatán) (rTcENO) and its encoding DNA (pBKTcENO) to immunize mice and evaluate their protective effects in an experimental murine model of acute phase infection. Our results showed that mice vaccinated with rTcENO or its encoding DNA were able to generate typical specific antibodies (IgG1, IgG2a, and IgG2b), suggesting that a mixed Th1/Th2 immune response was induced. The parasite burden in the blood was reduced to 69.8% and 71% in mice vaccinated with rTcENO and pBKTcENO, respectively. The group vaccinated with rTcENO achieved 75% survival, in contrast to the group vaccinated with pBKTcENO that showed no survival in comparison to the control groups. Moreover, rTcENO immunization elevated the production of IFN-γ and IL-2 after the parasite challenge, suggesting that the Th1-type immune response was polarized. These results indicated that rTcENO could be used as a vaccine against Chagas disease.

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.

Serum cytokines and activation ex vivo of CD4+ and CD8+ T cells in chagasic chronic Mexican patients

The clinical manifestations of human Chagas disease are associated with several factors, including immunological alterations, in this regard, many studies propose that tissue damage might be more severe in the absence of immune regulatory mechanisms, other factors are the genetic background of host and parasite. Trypanosoma cruzi population is genetically, biochemistry and pathogenic diverse along the Latin-America continent and phylogenetic ally are divided into six intra-species lineages TcI-VI. The TcI lineage has a wide distribution with heterogeneous virulence and pathogenesis within strains. In Mexico, the main circulating lineage is TcI in human infections. We analyzed intracytoplasmic cytokines of unstimulated peripheral T lymphocytes, and the level of cytokines (IL-2, IL-4, IL-12, IL-10, IFN-γ and sIL-2R) in the serum of Mexican chagasic subjects. The population studied consisted of 15 asymptomatic individuals, 17 patients with chronic chagasic cardiopathy (CCC), 20 patients with cardiopathy but negative serology for T. cruzi, and 10 healthy subjects. The analysis of CD4+ cells revealed that CCC and asymptomatic patients have higher CD25+ and CD69 activation markers than controls. The Th1 subset (CD4+/IFN- γ +) was higher in CCC than in asymptomatic and control subjects, whereas Th2 subset was markedly high in asymptomatic subjects. Circulating cytokines were below level detection with the exception of IL-2 and sIL-2R. Infection with Mexican Trypanosoma cruzi strains in asymptomatic chagasic subjects have a tendency for a Th2 response with higher CD8+/IFN-γ T cells. In contrast, CCC patients have low levels of intracellular IFN- γ and IL-2 cytokines. In both groups circulating serum cytokines are below the detectable level.

Estandarización de la técnica Dot-ELISA para la detección de anticuerpos anti-Trypanosoma cruzi y su comparación con ELISA y Western blot / Standardization of Dot-ELISA for detection of anti- Trypanosoma cruzi antibodies, compared to ELISA and Western blot

INTRODUCCIÓN: La enfermedad de Chagas es endémica de Latinoamérica. Debido a la migración de individuos de esta región a zonas no endémicas, como es el caso de Estados Unidos, Canadá y Europa, esta infección se ha convertido en un problema de salud mundial. Para realizar el diagnóstico existen pruebas parasitológicas y serológicas, pero solo las últimas son útiles durante la fase crónica. Muchas de estas técnicas requieren equipos costosos, lo que limita su uso. En este trabajo se estandarizó la técnica Dot-ELISA para el diagnóstico de la infección con Trypanosoma cruzi por ser una técnica sencilla, de bajo costo y accesible. Métodos Se evaluaron 360 muestras: 96 sueros de pacientes chagásicos y 153 de individuos sanos; 40 muestras de sangre recogidas en papel filtro, así como 71 sueros de pacientes con otras infecciones. Se calculó la sensibilidad, la especificidad y el índice kappa de Dot-ELISA en comparación con las pruebas ELISA y Western blot previamente estandarizadas para el diagnóstico de la infección por T. cruzi. Resultados Dot-ELISA obtuvo una sensibilidad del 97% y una especificidad del 89%, ya que presentó reacción cruzada principalmente con Leishmania spp. El índice kappa calculado fue de 0,79.ConclusionesDot-ELISA mostró buena correlación con otras pruebas que ya son utilizadas para el diagnóstico de la enfermedad de Chagas. Por tratarse de una técnica sencilla y económica, que puede realizarse sin equipo sofisticado, resulta ser más accesible y puede utilizarse como una prueba para un cribado inicial en el diagnóstico en el laboratorio o en estudios en campo

INTRODUCTION: Chagas disease is considered endemic of Latin America. Because of migration of people from this region to non-endemic areas, such as the United States, Canada and Europe, it has become a major health problem. There are parasitology and serology tests for its diagnosis, but only the latter are useful during the chronic phase. Most of these tests require expensive equipment, which make them also inaccessible for laboratories in endemic areas. In the present work we standardize Dot-ELISA as a diagnostic test for Trypanosoma cruzi infection, since it is an easy, inexpensive and an accessible test. METHODS: A total of 360 samples were tested: 96 sera from Chagas patients and 153 from healthy people;40 blood samples spots collected and eluted from filter paper were also tested, as well as 71 serum samples of patients with non-related infections. Sensitivity, specificity and kappa index of Dot-ELISA test were calculated, in order to determine a correlation value of this technique compared to ELISA and Western blot that are already being used for diagnosis. RESULTS: Dot-ELISA obtained 97% sensitivity and 89% specificity, since it showed cross-reaction mainly with Leishmania spp., and a kappa index of 0,79. CONCLUSIONS: Dot-ELISA results correlate well with other tests that are already being used for diagnosis of Chagas disease. As it is easy and inexpensive, it may be useful as an additional diagnostic test or for field Studies

In silico approach for the identification of immunological properties of enolase from Trypanosoma cruzi and its possible usefulness as vaccine in Chagas disease.

Nowadays, Chagas disease is a major health problem in Latin America that has been disseminated also into non-endemic countries. Currently, a vaccine against Chagas disease does not exist. In the present study, the gene encoding Trypanosoma cruzi enolase (TcENO) was amplified, cloned, and sequenced and the recombinant protein was purified. We used in silico and an experimental assay to investigate the immunological role of TcENO. The in silico assays showed that TcENO sequence contains characteristic motifs of enolase; additionally, a transmembranal region was identified, and this could indicate the potential membrane localization of TcENO. Moreover, both B lymphocyte and cytotoxic T lymphocytes (CTL) predicted epitopes were localized; these results suggest the possibility that TcENO can develop both humoral and cellular immune responses. Furthermore, the presence of antibodies was verified by western blot assays, showing that the purified recombinant protein was detected by sera from experimentally infected mice and sera of patients with Chagas disease. These results indicate that TcENO is immunogenic and could be used as a vaccine candidate.

[Standardization of Dot-ELISA for detection of anti-Trypanosoma cruzi antibodies, compared to ELISA and Western blot]. / Estandarización de la técnica Dot-ELISA para la detección de anticuerpos anti-Trypanosoma cruzi y su comparación con ELISA y Western blot.

INTRODUCTION: Chagas disease is considered endemic of Latin America. Because of migration of people from this region to non-endemic areas, such as the United States, Canada and Europe, it has become a major health problem. There are parasitology and serology tests for its diagnosis, but only the latter are useful during the chronic phase. Most of these tests require expensive equipment, which make them also inaccessible for laboratories in endemic areas. In the present work we standardize Dot-ELISA as a diagnostic test for Trypanosoma cruzi infection, since it is an easy, inexpensive and an accessible test. METHODS: A total of 360 samples were tested: 96 sera from Chagas patients and 153 from healthy people; 40 blood samples spots collected and eluted from filter paper were also tested, as well as 71 serum samples of patients with non-related infections. Sensitivity, specificity and kappa index of Dot-ELISA test were calculated, in order to determine a correlation value of this technique compared to ELISA and Western blot that are already being used for diagnosis. RESULTS: Dot-ELISA obtained 97% sensitivity and 89% specificity, since it showed cross-reaction mainly with Leishmania spp., and a kappa index of 0,79. CONCLUSIONS: Dot-ELISA results correlate well with other tests that are already being used for diagnosis of Chagas disease. As it is easy and inexpensive, it may be useful as an additional diagnostic test or for field studies.

The interleukin-1 gene cluster polymorphisms are associated with Takayasu's arteritis in Mexican patients.

Takayasu's arteritis (TA) is a chronic inflammatory arteritis of unknown etiology involving mainly the aorta and its major branches. The interleukin (IL) 1ß and IL-1 receptor antagonist have been playing an important role as regulators of inflammation. We investigated whether the polymorphisms at the IL-1B and IL-1RN gene cluster were associated with the genetic susceptibility to develop TA. We analyzed the IL-1B, IL-1F10.3, and IL-1RN polymorphisms in a sample of 58 TA patients, and 248 clinically healthy unrelated Mexican individuals by 5' exonuclease TaqMan polymerase chain reaction. Polymorphic haplotypes were constructed after linkage disequilibrium analysis. We found increased frequencies of different polymorphisms (C allele and TC genotype of IL-1F10.3; TT genotype of IL-1RN.4; C allele and TC genotype of IL-1RN6.1; G allele of IL-1RN6.2 and haplotypes "1T" and "1C" of IL-RN VNTR and IL-1RN6.1) in the group of TA when compared to healthy controls. On the other hand, decreased frequency of IL-1-511 TC genotype was found in the TA group compared to controls. IL-1B and IL-1RN gene polymorphisms could be involved in the risk of developing TA in the Mexican population. These associations were independent of the affected vessels.

Specific humoral and cellular immunity induced by Trypanosoma cruzi DNA immunization in a canine model.

Chagas disease has a high incidence in Mexico and other Latin American countries. Because one of the most important known methods of prevention is vector control, which has been effective only in certain areas of South America, the development of a vaccine to protect people at risk has been proposed. In this study, we assessed the cellular and humoral immune response generated following immunization with pBCSP and pBCSSP4 plasmids containing the genes encoding a trans-sialidase protein (present in all three forms of T. cruzi) and an amastigote specific glycoprotein, respectively, in a canine model. Thirty-five beagle dogs were divided randomly into 5 groups (n=7) and were immunized twice intramuscularly with 500 µg of pBCSSP4, pBCSP, pBk-CMV (empty plasmid) or saline solution. Fifteen days after the last immunization the 4 groups were infected intraperitoneally with 500,000 metacyclic trypomastigotes. The fifth group was unimmunized/infected. The parasitaemia in the immunized/infected dogs was for a shorter period (14 vs. 29 days) and the parasite load was lower. The concentration of IgG1 (0.612±0.019 O.D.) and IgG2 (1.167±0.097 O.D.) subclasses was measured (absorbance) 15 days after the last immunization with both recombinant plasmids, the majority of which were IgG2. The treatment of parasites using the serum from dogs immunized with pBCSP and pBCSSP4 plasmids produced 54% (±11.8) and 68% (±21.4) complement-mediated lysis, respectively. At 12 h post immunization, an increase in cytokines was not observed; however, vaccination with pBCSSP4 significantly increased the levels of IFN-γ and IL-10 at 9 months post-infection. The recombinant plasmid immunization stimulated the spleen cell proliferation showing a positive stimulatory index above 2.0. In conclusion, immunization using both genes effectively induces a humoral and cellular immune response.

Plasmid DNA immunization with Trypanosoma cruzi genes induces cardiac and clinical protection against Chagas disease in the canine model.

The only existing preventive measure against American trypanosomosis, or Chagas disease, is the control of the transmitting insect, which has only been effective in a few South American regions. Currently, there is no vaccine available to prevent this disease. Here, we present the clinical and cardiac levels of protection induced by expression to Trypanosoma cruzi genes encoding the TcSP and TcSSP4 proteins in the canine model. Physical examination, diagnostic chagasic serology, and serial electrocardiograms were performed before and after immunization, as well as after experimental infection. We found that immunization with recombinant plasmids prevented hyperthermia in the acute phase of experimental infection and produced lymphadenomegaly as an immunological response against the parasite and additionally prevented heart rate elevation (tachycardia) in the acute and/or chronic stages of infection. Immunization with T. cruzi genes encoding the TcSP and TcSSP4 antigens diminished the quality and quantity of the electrocardiographic abnormalities, thereby avoiding progression to more severe developments such as right bundle branch block or ventricular premature complexes in a greater number of dogs.

Detection of IS6110 and HupB gene sequences of Mycobacterium tuberculosis and bovis in the aortic tissue of patients with Takayasu's arteritis.

BACKGROUND: Takayasu's arteritis (TA) is a chronic inflammatory disease affecting the large arteries and their branches; its etiology is still unknown. In individuals suffering from TA, arterial inflammation progresses to stenosis and/or occlusion, leading to organ damage and affecting survival. Relation of TA with Mycobacterium tuberculosis has been known, but there have been only a few systematic studies focusing on this association. The IS6110 sequence identifies the Mycobacterium tuberculosis complex and the HupB establishes the differences between M. tuberculosis and M. bovis. Our objective was to search the presence of IS6110 and HupB genes in aorta of patients with TA. METHODS: We analyzed aorta tissues embedded in paraffin from 5760 autopsies obtained from our institution, we divided the selected samples as cases and controls; CASES: aortic tissues of individuals with Takayasu's arteritis. Control positive: aortic tissues (with tuberculosis disease confirmed) and control negative with other disease aortic (atherosclerosis). RESULTS: Of 181 selected aorta tissues, 119 fulfilled the corresponding criteria for TA, TB or atherosclerosis. Thus 33 corresponded to TA, 33 to tuberculosis (TB) and 53 to atherosclerosis. The mean age was 22 ± 13, 41 ± 19, and 57 ± 10, respectively. IS6110 and HupB sequences were detected in 70% of TA tissues, 82% in tuberculosis, and in 32% with atherosclerosis. Important statistical differences between groups with TA, tuberculosis versus atherosclerosis (p = 0.004 and 0.0001, respectively) were found. CONCLUSION: We identified a higher frequency of IS6110 and HupB genes in aortic tissues of TA patients. This data suggests that arterial damage could occur due to previous infection with M. tuberculosis.

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.

Trypanosoma cruzi connatal transmission in dogs with Chagas disease: experimental case report.

Trypanosoma cruzi connatal transmission was studied in male and female mongrel dogs. Both dogs were experimentally infected, after which on the 20(th) day, lymphoadenomegaly and fever were found. Four months postinfection, they mated. At this time, Chagas disease was confirmed by two different diagnostic tests. The electrocardiogram and echocardiogram taken at the eight postinoculation month showed data consistent with ischemia, local conduction abnormalities and hypertrophy, as well as a diminished ejection fraction and left ventricular dilation, respectively. Four puppies were born and after weaning had weakness, progressive weight loss, and chronic diarrhea. Necropsy of all four showed digestive alterations and cardiac dilation. Serology in the offspring was positive for Chagas disease. The histopathological study demonstrated a cardiac chronic inflammatory process, although no parasites were found. Clinical data and serological determinations are consistent with death from advanced Chagas disease.

Coronary CT angiography in Takayasu arteritis.

OBJECTIVES: The aim of this study was to use coronary computed tomographic (CT) angiography to characterize coronary artery involvement in patients with known Takayasu arteritis who present with anginal chest pain or shortness of breath. BACKGROUND: Takayasu arteritis is a primary vasculitis of the large vessels, which mainly affects the aorta and its branches but can also involve the coronary arteries. Coronary CT angiography allows visualization of the coronary vessels and can be used to detect both stenotic and nonstenotic coronary artery lesions. METHODS: Eighteen consecutive patients with Takayasu arteritis and angina (typical or atypical) and/or dyspnea underwent contrast-enhanced 64-slice coronary CT angiography. The arterial injury was classified according to the Numano classification. Three patients had prior known coronary artery disease. Coronary arteries were evaluated concerning the presence of obstructive and nonobstructive lesions, and differences between the clinical presentations of patients with and without coronary artery involvement on CT angiography were analyzed. RESULTS: Coronary artery involvement was found in 8 patients (44.4%), 3 of them with clinical activity. A total of 19 coronary lesions were present (13 in ostial locations, 5 in proximal coronary artery segments, and 1 in a mid segment). Eight lesions exceeded 50% diameter reduction (2 in ostial locations and 6 in proximal coronary artery segments). Median disease duration was significantly different between patients with coronary artery involvement (176 months; range 13 to 282 months) compared with those without (21 months; range 1 to 142 months) (p = 0.013). CONCLUSIONS: Coronary CT angiography allows the assessment of coronary artery involvement in patients with Takayasu arteritis. These data confirm prior observations that most coronary lesions are in ostial or proximal coronary artery locations. Disease duration in patients with coronary artery involvement is longer than in patients without.

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

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.

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.

Echocardiographic study of a Mestizo-Mexican population with Marfan syndrome.

BACKGROUND: The cardiovascular manifestations of Marfan syndrome (MFS) are the main causes of morbidity and mortality. This study describes the clinical and echocardiographic findings in a Mestizo-Mexican population affected by the disease. METHODS: A total of 166 patients previously diagnosed with MFS were recruited for the study, 114 of them underwent complete clinical history, with emphasis on Ghent nosology criteria, and transthoracic echocardiography, with 68 patients also undergoing transesophageal study. RESULTS: Major cardiovascular criteria from the Ghent nosology predominated in adults (P < 0.0001), minor criteria in children (P = 0.007). Among pediatric patients, 83% had a New York Heart Association (NYHA) functional class of I; however, 64% of the adult patients had an NYHA class ≥II, (P < 0.0001). Corrected aortic echocardiographic measurements of both groups demonstrated statistically significant differences. Children had a greater prevalence of mitral valve prolapse, while adults more frequently presented with aortic complications. Seven patients died during follow-up from aortic complications, one child and six adults. CONCLUSIONS: Based on the data, we can conclude that MFS in the Mestizo-Mexican population has a distinctly different clinical pattern in children and adults, and a graver prognosis in adults. Adult patients with MFS are significantly more likely, than children, to have aortic dilation, aortic aneurysm, aortic regurgitation, aneurysm rupture, aortic dissection, and fatal outcome. Children with MFS are more likely, than adults, to present with asymptomatic mitral and tricuspid prolapse and mitral valve regurgitation.