Influence of visceral adiposity on cardiovascular risk in patients with systemic sclerosis.

Systemic sclerosis (SSc) is an autoimmune disease characterized by systemic inflammation, endothelial dysfunction, generalized fibrosis and high cardiovascular mortality. The evaluation of cardiovascular risk through the visceral adiposity index (VAI) has been helpful due to its direct relationship to the body and visceral fat percentage. We evaluated the influence of body composition and anthropometrics on cardiovascular risk as measured by VAI in healthy controls (HC) and SSc. An analytical cross-sectional study of 66 participants (33 SSc and 33 HC), mean age 52.7 ± 10, 95% women, was conducted from August 2020 to January 2021. Inclusion criteria in cases were consecutive patients with SSc (ACR/EULAR 2013), 63.6% were diffuse cutaneous (dcSS) subtype, and 36.4 were limited cutaneous (lcSS) subtype. HC was matched by age and gender. Serum lipid profiles and InBody anthropometrics were analyzed and compared. We performed descriptive statistics, bivariate analysis with Student's t, or Mann-Whitney U, correlation and chi-square according to the variable type and distribution. Total cholesterol was significantly higher in SSc than HC (345 vs 194, p = < 0.001). The BMI was higher in HC (26.2 vs 28.9, p < 0.001). Kilograms of muscle (19.8 vs 28.9, p < 0.001) and total fat (23.4 vs 28.9, p < 0.001) were lower in SSc patients compared to HC. VAI was similar when BMI < 25, but significantly higher when BMI > 25 in SSc than in HC (3 vs 1.9, p = 0.030). The increase in BMI at overweight or obese in SSc is associated with a significant increase in cardiovascular risk.

Disability and cognitive impairment are interdependent in primary antiphospholipid syndrome.

BACKGROUND: Cognitive impairment (CI) occurs at a high frequency in primary antiphospholipid syndrome (PAPS). Its psychosocial-related factors are of interest. OBJECTIVE: We aimed to determine disability and perceived stress and their correlation with CI in PAPS. METHODS: First study phase: a longitudinal study including patients with PAPS and paired controls for cardiovascular risk factors, age, and sex, determining CI with Montreal Cognitive Assessment (MoCA) and then repeating the measurement 1 year later. Second study phase: a cross-sectional analytical study by quantification of disability with the World Health Organization Disability Assessment Schedule (WHODAS 2.0) and perceived stress with the Perceived Stress Scale (PSS-14). Descriptive statistics and Spearman correlation coefficient were used. RESULTS: Sixty-three patients with PAPS and 60 controls were studied. In PAPS, age (range, 48.0 ± 13.5 years), thrombotic artery events (TAE) (44.4%), and stroke/TIA (42.8%) were found. Disability was documented in the majority of WHODAS 2.0 domains and the total score for this was higher in participation and mobility, the stress level was normal, and 65.1% had CI. PAPS exhibited greater deterioration in the WHODAS 2.0 total score (p .017) and the MoCA test (p < .0001). Personal domains and the total WHODAS 2.0 score correlated inversely with MoCA. Life activities (rho = -0.419) and self-care (rho = -0.407) were those that correlated to the greatest degree. Stroke conferred risk for CI. CONCLUSIONS: Disability in PAPS and CI are interdependent. New treatment options and neurocognitive stimulation strategies are necessary to maintain functionality and prevent further cognitive dysfunction in PAPS patients.

Risk Factors and Outcomes for COVID-19 in Autoimmune Inflammatory Diseases during the SARS-CoV-2 Pandemic: A Comparative Study.

BACKGROUND: Patients with autoimmune disease (AID) and coronavirus disease 2019 (COVID-19) could have higher mortality due to the co-morbidity and the use of immunosuppressive therapy. OBJECTIVES: To analyze the risk factors and outcomes of patients with AID and COVID-19 versus a control group. METHODS: A prospective cohort study included patients with and without AID and COVID-19. Patients were paired by age and sex. Clinical, biochemical, immunological treatments, and outcomes (days of hospital stay, invasive mechanical ventilation [IMV], oxygen at discharge, and death) were collected. RESULTS: We included 226 COVID-19 patients: 113 with AID (51.15 ± 14.3 years) and 113 controls (53.45 ± 13.3 years). The most frequent AIDs were Rheumatoid arthritis (26.5%), systemic lupus erythematosus (21%), and systemic sclerosis (14%). AID patients had lower lactate dehydrogenas, C-reactive protein, fibrinogen, IMV (P = 0.027), and oxygen levels at discharge (P ≤ 0.0001) and lower death rates (P ≤ 0.0001). Oxygen saturation (SaO2) ≤ 88% at hospitalization provided risk for IMV (RR [relative risk] 3.83, 95% confidence interval [95%CI] 1.1-13.6, P = 0.038). Higher creatinine and LDH levels were associated with death in the AID group. SaO2 ≤ 88% and CO-RADS ≥ 4 were risk factors for in-hospital mortality (RR 4.90, 95%CI 1.8-13.0, P = 0.001 and RR 7.60, 95%CI 1.4-39.7, P = 0.016, respectively). Anticoagulant therapy was protective (RR 0.36, 95%CI 0.1-0.9, P = 0.041). CONCLUSIONS: Patients with AID had better outcomes with COVID-19 than controls. Anticoagulation was associated with a lower death in patients with AID.

Effectiveness of Saccharomyces boulardii and Metronidazole for Small Intestinal Bacterial Overgrowth in Systemic Sclerosis.

BACKGROUND: Small intestinal bacterial overgrowth (SIBO) affects up to 60% of patients with systemic sclerosis (SSc), and it improves with antibiotics. The addition of probiotics could lead to better results. AIMS: To evaluate the efficacy and safety of Saccharomyces boulardii (SB) versus metronidazole (M) versus M + SB for 2 months, to reduce gastrointestinal symptoms and SIBO assessed with hydrogen breath test in SSc. METHODS: An open pilot clinical trial performed in forty patients with SIBO and SSc (ACR-EULAR 2013) who signed informed consent. Three groups were assigned: M, SB, and M + SB, for 2 months. Hydrogen was measured in parts per million with a hydrogen breath test to evaluate SIBO. The National Institutes of Health Patient-Reported Outcomes Measurement Information System (NIH-PROMIS) questionnaire was applied to quantify gastrointestinal symptoms with a raw score of eight symptoms. This study is registered in ClinicalTrials.gov with the following ID: NCT03692299. RESULTS: Baseline characteristics were similar between groups. The average age was 53.2 ± 9.3 years, and the evolution of SSc was 13.5 (1-34) years. After 2 months of treatment, SIBO was eradicated in 55% of the M + SB group: 33% of SB, and 25% of M. The SB and M + SB groups had decreased diarrhea, abdominal pain, and gas/bloating/flatulence, but M remained unchanged. Reductions in expired hydrogen at 45 to 60 min were as follows: M + SB 48% and 44%, M 18% and 20%, and SB 53% and 60% at the first and second months, respectively (p < 0.01). Adverse effects were epigastric burning and constipation in M (53%) and M + SB (36%), and flatulence/diarrhea in SB (22%). CONCLUSIONS: Metronidazole treatment is partially effective in SIBO, but S. boulardii in monotherapy or in combination improves the gastrointestinal outcomes in SSc.

Metabolic syndrome, autoimmunity and rheumatic diseases.

Metabolic syndrome (MetS) is a cluster of metabolic and cardiovascular (CV) risk factors including obesity and visceral adiposity, insulin resistance, dyslipidemia and hypertension contributing to CV mortality. The interface between the metabolic and immune systems has been of great interest recently. These interactions are regulated through genetics, nutritional status, and the intestinal microbiome. Alterations in the immune-metabolic cross-talk contribute to the development of autoimmune diseases. Adipokines exert a variety of metabolic activities contributing to the ethiopathogenesis of MetS and are involved in the regulation of both inflammatory processes and autoimmunity occurring in rheumatic diseases. Patients with autoinflammatory disease such as gout and those with autoimmune rheumatic diseases (ARD), such as systemic lupus erythematosus, rheumatoid arthritis, antiphospholipid syndrome, ankylosing spondylitis and vasculitis among others, have increased prevalence of MetS. Despite recent advances in treatment of ARD, incidence of CVD remains high. MetS and altered secretion patterns of proinflammatory adipokines could be the link between CVDs and ARD. In addition, in ARD the activation of proinflammatory signalling pathways results in the induction of several biological markers of chronic inflammation contributing to CVD. In the present paper, we review recent evidences of the interactions between MetS and ARD, as well as novel therapeutic targets.

Impact of intestinal mannitol on hyperammonemia, oxidative stress and severity of hepatic encephalopathy in the ED.

Hyperammonemia results from hepatic inability to remove nitrogenous products generated by protein metabolism of intestinal microbiota, which leads to hepatic encephalopathy (HE) in chronic liver disease (CLD). In ammonium neurotoxicity, oxidative stress (OxS) plays a pathogenic role. Our objective was to evaluate if intestinal mannitol is as effective and safe as conventional treatment for diminishing hyperammonemia, OxS, and HE in patients with CLD. MATERIAL AND METHODS: We included 30 patients with HE classified by "Haven Criteria for Hepatic Encephalopathy". They were randomized into two groups: 1) Mannitol Group (MG) with mannitol 20% administered into the intestine by an enema, 2) conventional group (CG) with lactulose 40 g enema both substances were diluted in 800 mL of double distilled solution every 6 h; all patients received neomycin. We evaluated ammonia concentration, plasma oxidative stress, HE severity, intestinal discomfort and adverse effects. RESULTS: Hyperammonemia (171 ±â€¯104 vs 79 ±â€¯49 µmol ammonia/L, p < 0.01), and oxidative stress (MDA 29 vs 27%, formazan 15 vs 11%, carbonyls 16 vs 9% and dityrosines 10 vs 5%) were reduced in MG and CG respectively. The HE severity decreased by two degrees compared to baseline values in both groups. Intestinal discomfort and electrolyte plasma alterations were less frequent (p < 0.05) in MG than CG. CONCLUSIONS: Intestinal mannitol is as effective and safe as conventional treatment for reducing hyperammonemia, oxidative stress, and hepatic encephalopathy of CLD patients in the emergency room. Likewise, mannitol is better tolerated than conventional treatment.

Malnutrition is an independent risk factor for mortality in Mexican patients with systemic sclerosis: a cohort study.

Factors for mortality in systemic sclerosis (SSc) vary in different cohorts around the world. Case-control study nested in a cohort. We included patients ≥16 years of age with SSc (ACR/EULAR 2013), from 2005 to 2015. Demographic and clinical variables and causes of mortality were recorded. We calculated Crude Mortality Rate (CMR), Standardized Mortality Ratio (SMR), and Kaplan-Meier survival analysis was performed. A Cox proportional hazard (HR) regression analysis of the potential risk factors associated with mortality was also performed. A total of 220 patients with SSc were included. During follow-up, 28 deaths occurred. The sum of total time contributed by all subjects was 1074 years-person, the CMR was 12.72%, the overall SMR was 4.5, in women 3.7, and in men 4.7. The survival rate at 5 and 10 years was 83 and 70%, respectively. The causes of death were definitively attributed to SSc in 21.4% of the cases, probably in 28.7%, unrelated in 35.6%, and unknown in 14.3%. The direct cause of death of the patients was infection in 25% of cases, cardiovascular disease in 14%, lung involvement in 14%, pulmonary embolism in 11%, and neoplasia in 11%. The Cox regression analysis showed that the factors associated with mortality were: male gender (HR 5.84, CI 95% 1.31-26, p = 0.013), severe Medsger's score for general symptoms (HR 5.12, CI 95% 1.74-14.97, p = 0.021) and severe malnutrition (HR 3.77, CI 95% 1.23-11.06, p = 0.008). Infections, cardiovascular disease, and lung involvement were the leading cause of death. Male gender and severe general affection and malnutrition were associated with a poorer prognosis of SSc.

Human monkeypox coinfection with syphilis in an immunocompromised patient.

Monkeypox is a viral zoonosis from the Poxviridae family that spreads at an unprecedented rate. It is transmitted through contact with skin lesions, respiratory droplets, body fluids, and sexual contact. The diverse presentation of the disease leads to misdiagnosis. Thus, clinicians should have a high index of suspicion, mainly with diseases with skin lesions. The most vulnerable group to developing this disease are individuals with risky sexual relationships, sexually transmitted infections, or human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS). To date, only one case of coinfection with the monkeypox virus, syphilis, and HIV has been reported; however, no cases have been revealed in the Mexican territory. Herein we describe an unusual case of syphilis-monkeypox coinfection in an immunocompromised patient; despite his coinfection, he had a favorable prognosis. Furthermore, we attach allusive pictures of the natural evolution of dermatological lesions.

New Onset Autoimmune Diseases after the Sputnik Vaccine.

The vertiginous advance for identifying the genomic sequence of SARS-CoV-2 allowed the development of a vaccine including mRNA-based vaccines, inactivated viruses, protein subunits, and adenoviral vaccines such as Sputnik. This study aims to report on autoimmune disease manifestations that occurred following COVID-19 Sputnik vaccination. Patients and Methods: A retrospective study was conducted on patients with new-onset autoimmune diseases induced by a post-COVID-19 vaccine between March 2021 and December 2022, in two referral hospitals in Mexico City and Argentina. The study evaluated patients who received the Sputnik vaccine and developed recent-onset autoimmune diseases. Results: Twenty-eight patients developed recent-onset autoimmune diseases after Sputnik vaccine. The median age was 56.9 ± 21.7 years, with 14 females and 14 males. The autoimmune diseases observed were neurological in 13 patients (46%), hematological autoimmune manifestations occurred in 12 patients (42%), with thrombotic disease observed in 10 patients (28%), and autoimmune hemolytic anemia in two patients (7.1%). Rheumatological disorders were present in two patients (7.1%), and endocrine disorders in one patient (3.5%). Principio del formulario Conclusion: Although the COVID-19 Sputnik vaccine is generally safe, it can lead to adverse effects. Thrombosis and Guillain-Barre were the most frequent manifestations observed in our group of patients.

Disease course of Chronic Relapsing Inflammatory Optic Neuropathy (CRION) in a single care center.

BACKGROUND: Chronic relapsing inflammatory optic neuropathy (CRION) is a recurrent, idiopathic optic neuritis and is considered as a rare disease. OBJECTIVE: To describe the clinical course during long-term follow-up of patients with a diagnosis of CRION. METHODS: From a cohort of 1,735 patients with demyelinating disorders, we selected patients aged over 16 years with CRION according to current criteria. Demographic and clinical data, including initial presentation, symptoms, number of relapses, time delay in diagnosis, diagnostic methods, and treatment were obtained from clinical files. Infections, autoimmune diseases, and multiple sclerosis, among other conditions, were ruled out in all patients. RESULTS: We analyzed 30 patients with CRION: 24 women and six men, with mean age of 42.8±10.2 years, median disease course of 7.9 years (5.29-13.1), and median number of attacks of 2 (IQR 2-4). The initial manifestation was ocular pain in 97% and bilateral and sequential affection in 87%. Visual acuity was recovered in 50%, did not improve in 33%, and recovered incompletely in 17%. Antibodies against aquaporin-4 (AQP4-Abs) were negative in 73.3%. Magnetic resonance imaging of the brain was normal in 76.7%. None of the patients evolved to another demyelinating disease over time. Initial treatment was methylprednisolone in 100%, and plasmapheresis in 20%. Currently, all patients are on maintenance treatment with mycophenolate mofetil or rituximab with a decrease in relapsing rate. CONCLUSIONS: Diagnosis of CRION is challenging and should be kept in mind. Prompt diagnosis, adequate treatment and close follow-up are essential to prevent disabling sequelae in these patients.

[Late onset primary antiphospholipid syndrome in the older adult]. / Síndrome antifosfolípido primario con debut tardío en el adulto mayor.

BACKGROUND: Antiphospholipid syndrome (APS) is a systemic autoimmune disease, characterized by arterial or venous thrombosis and/or obstetric events in the presence of antiphospholipid antibodies (aPL). It is usually diagnosed in patients between the ages of 15 and 50 years, and there are 5 new cases per 100,000 people per year. It is reported a case of APS, which it is present in an older adult with an unusual clinical manifestation. CLINICAL CASE: Female patient without history of autoimmune diseases, at age 70 presented hemolytic anemia, Coombs direct positive, classified as autoimmune hemolytic anemia (AHAI) Coombs+, and severe thrombocytopenia. Other immunological, infectious, and lymphoid proliferative disorders and solid tumors were ruled out. Fisher-Evans syndrome (FES) was diagnosed with good response to treatment. Three months later, the patient presented deep venous thrombosis in the left pelvic limb, positive antiphospholipid antibodies (aPL) and positive aloantibodies were determined, establishing the diagnosis of primary APS and FES as its initial manifestation. Since then, the patient has been in treatment with acenocoumarol and prednisone without new recurrences of thrombosis, with persistence of moderate thrombocytopenia, without adding another clinical manifestation in 15 years of follow-up. CONCLUSION: The unusual presentation of this disease in older adults with comorbidities should not rule out the possibility of the development of a primary autoimmune disease, so it should be considered for diagnosis in this age group.

INTRODUCCIÓN: el síndrome antifosfolípido (SAF) es una enfermedad autoinmune sistémica, caracterizada por trombosis arterial o venosa, o eventos obstétricos en presencia de anticuerpos antifosfolípidos (aPL). Suele diagnosticarse entre los 15 y los 50 años, y hay cinco casos nuevos por cada 100 000 personas al año. Se reporta un caso de SAF que presenta una adulta mayor con manifestación clínica poco usual. CASO CLÍNICO: paciente mujer, sin antecedentes de enfermedades autoinmunes, que a los 70 años presentó anemia hemolítica y Coombs directo positivo, lo cual se catalogó como anemia hemolítica autoinmune (AHAI) Coombs+, y trombocitopenia severa. Se descartaron otros trastornos inmunológicos, infecciosos, linfoproliferativos y tumores sólidos, y se diagnosticó síndrome de Fisher-Evans (SFE) con buena respuesta al tratamiento. Tres meses después, la paciente presentó trombosis venosa profunda en miembro pélvico izquierdo. Se determinaron aPL positivos y aloanticuerpos positivos, y se estableció el diagnóstico de SAF primario y como su manifestación inicial el SFE. Desde entonces la paciente fue tratada con acenocumarina y prednisona sin recurrencias de trombosis, con persistencia de trombocitopenia moderada y sin nuevas manifestaciones clínicas en 15 años de seguimiento. CONCLUSIÓN: ante la presentación inusual de esta enfermedad en adultos mayores con comorbilidades no se debe descartar la posibilidad del desarrollo de una enfermedad autoinmune primaria, por lo cual se debe tener en cuenta para su diagnóstico en este grupo etario.

Transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing sweet syndrome.

The Sweet's syndrome, is an inflammatory skin disorder characterized by extensive infiltration of neutrophils in the dermis with extension to the subcutis, known as acute febrile neutrophilic dermatosis. It may occur as a paraneoplastic syndrome. To our knowledge, there are currently few reports about transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing Sweet syndrome in the literature. Herein we describe an unusual case in a young patient with these characteristics that evolved to a fatal outcome.

[Body composition and physical activity in resident physicians]. / Composición corporal y actividad física en médicos residentes.

BACKGROUND: Mexico is ranked second in obesity in adults worldwide and resident physicians are not exempt from this problem. Inadequate diet and physical inactivity are associated factors. Bioimpedance has greater precision than anthropometry. OBJECTIVE: To analyze the difference between degree of physical activity, caloric intake and body composition in Internal Medicine residents according to their degree of residence. MATERIAL AND METHODS: Cross-sectional study. Resident physicians of both sexes, from second, third and fourth degree of training were included; they were fasting. Vital signs were taken; a Rapid Assessment of Physical Activity (RAPA) questionnaire, anthropometry, a 24-hour reminder, and bioelectrical impedance analysis were administered. Descriptive statistics, Kruskal-Wallis and chi squared tests were used. RESULTS: 84 resident physicians were included, 48 were male. The median age was of 27 years (26-28). There was a prevalence of 46.4% of overweight and obesity, despite the hypocaloric diet in 89%. Bioelectrical impedance analysis showed that 72.6% of residents had elevated body fat, 71% sub-optimal degree of physical activity and 23.7% arterial hypertension. CONCLUSIONS: A high prevalence of overweight and obesity was found with differences in body composition and suboptimal level of physical activity. It is important to correct bad eating habits and improve physical activity to reduce risks in this population.

INTRODUCCIÓN: México es segundo lugar mundial en obesidad en adultos y los médicos residentes no están exentos de este problema. La dieta inadecuada y la inactividad física son factores asociados. La bioimpedancia tiene mayor precisión que la antropometría. OBJETIVO: analizar la diferencia entre grado de actividad física, ingesta calórica y composición corporal en residentes de Medicina Interna de acuerdo con su grado de residencia. MATERIAL Y MÉTODOS: estudio transversal. Se incluyeron médicos residentes de ambos sexos de segundo, tercero y cuarto grado, quienes participaron en ayuno. Se les tomaron signos vitales, se les otorgó el cuestionario Rapid Assesment of Physical Activity (RAPA), antropometría, recordatorio de 24 horas y medición de composición corporal por bioimpedancia. Se empleó estadística descriptiva, prueba de Kruskal-Wallis y chi cuadrada. RESULTADOS: se incluyeron 84 médicos residentes, 48 (57.14%) fueron del género masculino. La mediana de edad fue de 27 años (26-28). Se encontró una prevalencia de sobrepeso y obesidad de 46.4% a pesar de la dieta hipocalórica en el 89%. La medición por bioimpedancia indicó que 72.6% tuvo grasa corporal elevada, 71% grado de actividad física subóptimo y 23.7% hipertensión arterial. CONCLUSIONES: se encontró una alta prevalencia de sobrepeso y obesidad con diferencias en la composición corporal y el nivel subóptimo de actividad física. Es importante corregir los malos hábitos alimenticios y mejorar la actividad física para reducir riesgos en esta población.

Development and Evaluation of a Set of Spike and Receptor Binding Domain-Based Enzyme-Linked Immunosorbent Assays for SARS-CoV-2 Serological Testing.

The implementation and validation of anti-SARS-CoV-2 IgG serological assays are reported in this paper. S1 and RBD proteins were used to coat ELISA plates, and several secondary antibodies served as reporters. The assays were initially validated with 50 RT-PCR positive COVID-19 sera, which showed high IgG titers of mainly IgG1 isotype, followed by IgG3. Low or no IgG2 and IgG4 titers were detected. Then, the RBD/IgG assay was further validated with 887 serum samples from RT-PCR positive COVID-19 individuals collected at different times, including 7, 14, 21, and 40 days after the onset of symptoms. Most of the sera were IgG positive at day 40, with seroconversion happening after 14-21 days. A third party conducted an additional performance test of the RBD/IgG assay with 406 sera, including 149 RT-PCR positive COVID-19 samples, 229 RT-PCR negative COVID-19 individuals, and 28 sera from individuals with other viral infections not related to SARS-CoV-2. The sensitivity of the assay was 99.33%, with a specificity of 97.82%. All the sera collected from individuals with infectious diseases other than COVID-19 were negative. Given the robustness of this RBD/IgG assay, it received approval from the sanitary authority in Mexico (COFEPRIS) for production and commercialization under the name UDISTEST-V2G®.

Standard versus extended criteria graft procurement. Experience at the Hospital de Especialidades La Raza. / Procuración de injertos estándar frente a extendidos. Experiencia en el Hospital de Especialidades La Raza.

BACKGROUND: In Mexico, there are 23 158 patients waiting for an organ or tissue transplant. The increasing demand of grafts justifies the use of expanded criteria donors; however, not even all standard grafts have been procured. OBJECTIVE: To identify the associated factors to the decision of not procuring grafts from brain death donors whose donation was consented. METHOD: Retrospective cohort, univariate and multivariate analysis. 35 donation files with brain death were included from 2014 to 2019. Groups in which the heart wasn't procured versus those in which it was procured were compared; same comparisons were made for liver, kidney, skin, bone tissue and corneas. RESULTS: 20 women (57.1%), 15 men (42.9%) average age of 43.8 ± 16.4 years. High-dose of inotropic or norepinephrine use increased the probability of cardiac procurement (odds ratio [OR] 0.57; 95% confidence interval [95% CI]: 0.0-0.5). It was not detected a sensitive and specific variable for decision making at liver procurement. Implementation of two or more diagnostic methods for BD were associated with kidney loss (OR: 10; 95% CI: 1.2-78.1). Organs and tissues met standard criteria; however, 76 (41.5%) were not procured. CONCLUSIONS: Non-procurement associated factors were different from the standard donor established criteria. It is necessary to follow clear procurement criteria, in order to reduce viable grafts loss.

INTRODUCCIÓN: En México se registraron 23,158 personas en espera de un órgano o tejido para trasplante durante el año 2019. El constante aumento de la demanda sustenta el empleo de donantes con criterios extendidos; sin embargo, no todos los injertos estándar se procuran. OBJETIVO: Identificar los factores asociados a la decisión de no procurar injertos provenientes de donantes con muerte encefálica en quienes se consintió la donación. MÉTODO: Cohorte retrospectiva, análisis univariado y multivariado. Se incluyeron 35 expedientes de donación concretada con muerte encefálica de 2014 a 2019. Se compararon los grupos en los que no se procuró corazón, en los que sí, y para hígado, riñón, tejido óseo, piel y córneas. RESULTADOS: Se incluyeron 20 mujeres (57.1%) y 15 hombres (42.9%) con una edad media de 43.8 ± 16.4 años. El uso de inotrópico a dosis altas o norepinefrina aumentó la probabilidad de procuración cardiaca (razón de momios [RM]: 0.57; intervalo de confianza del 95% [IC95%]: 0.0-0.5). No se detectó ninguna variable sensible y específica para la toma de decisión en la procuración hepática. El empleo de dos o más métodos diagnósticos de muerte encefálica se asoció a pérdida del riñón (RM: 10; IC95%: 1.2-78.1). Cumplieron con criterios estándar 183 órganos y tejidos (74.6%); sin embargo, 76 (41.5%) no fueron procurados. CONCLUSIONES: Los factores asociados a la decisión de no procuración fueron distintos a los criterios de donante estándar establecidos. Es necesario seguir criterios claros de procuración para disminuir la pérdida de injertos viables.

The Systemic Sclerosis Questionnaire (SySQ): Validation of the translation of the original German version into Spanish and its relationship to the disease and to quality of life. / Cuestionario de funcionalidad para esclerosis sistémica (SySQ): validación en español del original en alemán y su relación con la enfermedad y la calidad de vida.

OBJECTIVE: Translation, transculturation and validity of the self-administered questionnaire for functionality (Systemic Sclerosis Questionnaires [SySQ]) for use in Spanish patients with systemic sclerosis and its relationship to the severity of the disease and to quality of life. PATIENTS AND METHODS: We conducted an observational analytical study to perform a cross-cultural validation of the self-administered questionnaire on functionality in scleroderma. The validity of the form and content was evaluated by an expert panel. The method included: a) adaptation into Spanish of the construct for translation and back translation, and transculturation; b) internal consistency with the SySQ (Cronbach's alpha), and c) reproducibility was assessed taking into account all occasions in which the test was performed with Cohen's kappa. Additionally, we calculated the Spearman correlation coefficient with the Medsger severity scale, Health Assessment Questionnaire score and SF-36 score. RESULTS: We included 70 patients with systemic sclerosis: age 17-78 (51±12) years, 65 (93%) were women, diffuse/limited subtype 64/36%, disease duration of 0.5-40 years. Optimal internal consistency for all categories of the final version of SySQ (Cronbach's α of 0.961) and intraobserver reliability in 2 tests over a 2-week interval (Cohen's kappa coefficient 0.618) and optimal interobserver reliability in 2 tests on the same day (Cohen's kappa coefficient 0.911). Moderate correlation between functionality by SySQ and by Health Assessment Questionnaire (r=0.573, P<.0001). Inverse correlation between SySQ and quality of life mental health domain SF-36 (r=-0.435, P<.001) and physical domain SF-36 (r=-0.638, P<.001). Medsger severity scale (tendon, heart, lung, vascular) also showed significant correlation with SySQ. CONCLUSIONS: SySQ in this validated Spanish version is a suitable instrument to measure functional status in patients with systemic sclerosis. Reduced functionality is related to greater tendon and peripheral vascular involvement and to a poorer quality of life.

Myocardial function in primary antiphospholipid syndrome using speckle-tracking echocardiography.

Antiphospholipid syndrome (APS) patients have high cardiovascular risk. Speckle-tracking echocardiography (STE) detects myocardial function. To evaluate the myocardial function in primary APS (PAPS) patients using two-dimensional echocardiography (2-D) obtaining values of left ventricle global longitudinal strain (GLS) by STE. Patients with PAPS, > 16 years, both genders, without signs and symptoms of heart disease were recruited and matched with healthy controls by age and gender. Demographic, clinical data, and cardiovascular risk factors were recorded. Images from the two-, three-, and four-chamber views were recorded and analyzed with STE and values of GLS. Descriptive and analytic statistics were applied. Thirty-eight PAPS patients and 21 controls were included. Age 46.7 ± 10, disease evolution 13.06 ± 6.69 years. Obesity and dyslipidemia were more frequent in PAPS patients in comparison with controls. Even though the proportion of obesity was greater in PAPS patients than in the control group, we did not find significant differences, neither in patients with/without metabolic syndrome. Regarding patients with and without obesity with lower GLS values, there were no differences either. Average GLS was lower in PAPS than in controls. Segments and myocardial regions showed lower values of myocardial deformation in PAPS patients than controls, including apical segments suggesting underlying heart disease. GLS is reduced in PAPS compared with healthy subjects. STE is a suitable method to detect cardiac affection in patients without apparent cardiac involvement. Studies with this technique to monitor progression of heart disease in PAPS are necessary.

Splenectomy in systemic lupus erythematosus and autoimmune hematologic disease: a comparative analysis.

The objective of the study is to analyze the efficacy and safety of splenectomy in the management of refractory autoimmune thrombocytopenia (AT)/autoimmune hemolytic anemia (AIHA) associated or not with systemic lupus erythematosus. Thirty-four patients after splenectomy due to severe AT and/or AIHA were divided into group 1 (G1) 18 SLE/APS patients: 9 AT/SLE patients, 6 SLE/antiphospholipid syndrome (APS), and 3 primary APS. Group 2 (G2): 16 patients without SLE/APS: 2 Fisher-Evans syndrome and 14 AIHA. Surgery approach when (1) platelets ≤ 50,000/ml despite 2 weeks on medical therapy, (2) medically dependent, and (3) medically intolerant or after two hemolytic crises in AIHA patients. Splenectomy response: (1) complete (CR): ≥ 150,000 platelets/ml, (2) partial: 50,000-149,000/ml, or (3) none: ≤ 50,000/ml. CR for AIHA: hemoglobin ≥9 g/dl. STATISTICAL ANALYSIS: descriptive statistics and chi-square test. The mean age was 34.6 years; mean follow-up: 28.5 months. Open splenectomy in 15/34 vs laparoscopy in 19/34 (p = NS). CR in 15/34, G1: 4/18, G2: 11/16, (p = 0.006). Complications in 6/34, 5 from G2 vs 1 from G1 (p = 0.05). Relapse in 7/18 patients in G1 and 3/16 in G2 (p = 0.05). Open and laparoscopic splenectomies in SLE and AT patients are as effective as in those without SLE; however, patients with SLE and APS had more relapses.

Autoimmune/inflammatory syndrome induced by mineral oil: a health problem.

Autoimmune/inflammatory syndrome induced by adjuvant (ASIA) includes the following conditions: siliconosis, Gulf War syndrome, macrophagic myofasciitis syndrome, and post-vaccination phenomena. Afterward, other syndromes have been recognized, such as in ASIA by mineral oil (ASIA-MO). These conditions are triggered by adjuvants and they are the result of the interplay of genetic and environmental factors. ASIA-MO is defined as the infiltration of oily type modeling substances for cosmetic purposes. It has been reported in many countries and used surreptitiously. Pathogenesis of ASIA-MO is not clear, but is characterized by chronic granulomatous inflammation, like the pristane model in mice, with increase of proinflammatory cytokines: type I interferons (IFNα and IFNß), systemic lupus erythematosus (SLE), and erosive arthritis. In humans, an increase of interleukin 1 (IL-1) has been found. Clinical spectrum of ASIA-MO is heterogeneous, varying from mild to severe and being local and systemic. The systemic manifestations can be non-specific and specific, meeting criteria for any autoimmune disease (AID), i.e., SLE, rheumatoid arthritis, and systemic sclerosis, among others. The areas of the body where the mineral oil is mostly applied include the following: buttocks (38-72%), breasts (12-16%), lower extremities (18-22%), and face (6-10%). The penis augmentation is also common. Treatment is focused on local and systemic manifestations and requires medical and surgical management representing a challenge for the physician.

Antiphospholipid antibodies disappearance in primary antiphospholipid syndrome: Thrombosis recurrence.

OBJECTIVE: To evaluate the clinical outcome after aPL (antiphospholipid antibodies) disappearance in primary APS patients. METHODS: From a cohort of 70 patients with primary APS, we selected patients with positive aPL determinations at onset and ≥2 subsequent negative aPL determinations during the last 5years. To corroborate the immunologic profile, we determined IgG/IgM aCL antibodies, IgG/IgM antiß2GPl, anti-annexin A5 antibodies and lupus anticoagulant (LA). All patients continued treatment with oral anticoagulants. Clinical data and aPL determinations at onset/after disappearance were obtained. STATISTICAL ANALYSIS: descriptive statistics and Kaplan-Meier analysis. RESULTS: We found 24 patients with persistently negative aPL, including the last immunologic profile, 17 females, 7 males, mean age 51.7, disease evolution 16.3years, mean of 4 aPL previous positive determinations. aCL was positive at onset in 87.5%, 29% had double aPL positivity at onset (aCL/LA). Deep venous thrombosis (DVT) and ischemic stroke in 33% and pulmonary embolism in 12.5% were the most frequent manifestations at onset. INR range: 2-3. Time with aPL positive 109.4±80.7months. After 60months of follow-up since aPL disappearance, 45.8% of patients presented thrombosis recurrence, DVT in 9 patients, ischemic stroke in 1, pulmonary artery hypertension in 1. Other non-thrombotic APS manifestations were chronic ulcers in lower extremities and severe thrombocytopenia. CONCLUSIONS: This study suggest, that in primary APS, persistent negative aPL profile is not an indication to interrupt oral anticoagulant therapy. However, there is a subset of patients that remained asymptomatic. Other studies are necessary in order to elucidate this controversy.