Time Course of Mechanical Ventilation Driving Pressure Levels in Pediatric Acute Respiratory Distress Syndrome: Outcomes in a Prospective, Multicenter Cohort Study From Colombia, 2018-2022.

OBJECTIVES: High driving pressure (DP, ratio of tidal volume (Vt) over respiratory system compliance) is a risk for poor outcomes in patients with pediatric acute respiratory distress syndrome (PARDS). We therefore assessed the time course in level of DP (i.e., 24, 48, and 72 hr) after starting mechanical ventilation (MV), and its association with 28-day mortality. DESIGN: Multicenter, prospective study conducted between February 2018 and December 2022. SETTING: Twelve tertiary care PICUs in Colombia. PATIENTS: One hundred eighty-four intubated children with moderate to severe PARDS. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The median (interquartile range [IQR]) age of the PARDS cohort was 11 (IQR 3-24) months. A total of 129 of 184 patients (70.2%) had a pulmonary etiology leading to PARDS, and 31 of 184 patients (16.8%) died. In the first 24 hours after admission, the plateau pressure in the nonsurvivor group, compared with the survivor group, differed (28.24 [IQR 24.14-32.11] vs. 23.18 [IQR 20.72-27.13] cm H2O, p < 0.01). Of note, children with a Vt less than 8 mL/kg of ideal body weight had lower adjusted odds ratio (aOR [95% CI]) of 28-day mortality (aOR 0.69, [95% CI, 0.55-0.87]; p = 0.02). However, we failed to identify an association between DP level and the oxygenation index (aOR 0.58; 95% CI, 0.21-1.58) at each of time point. In a diagnostic exploratory analysis, we found that DP greater than 15 cm H2O at 72 hours was an explanatory variable for mortality, with area under the receiver operating characteristic curve of 0.83 (95% CI, 0.74-0.89); there was also increased hazard for death with hazard ratio 2.5 (95% CI, 1.07-5.92). DP greater than 15 cm H2O at 72 hours was also associated with longer duration of MV (10 [IQR 7-14] vs. 7 [IQR 5-10] d; p = 0.02). CONCLUSIONS: In children with moderate to severe PARDS, a DP greater than 15 cm H2O at 72 hours after the initiation of MV is associated with greater odds of 28-day mortality and a longer duration of MV. DP should be considered a variable worth monitoring during protective ventilation for PARDS.

Minimally Invasive Surgery for Managing Grade IV and V Spondylolisthesis.

Surgical treatment of high-grade spondylolisthesis is controversial and aims at restoring the spinopelvic sagittal balance through complete or partial reduction of the listhesis. Nerve decompression and interbody fusion are necessary for patients presenting with neurological deficit, severe pain, lower limb asymmetry, or deformities. We present the case and the results of a patient with high-grade spondylolisthesis, in whom minimally invasive management was performed. A narrative review in this topic is also provided. We performed a literature review of high-grade spondylolisthesis to compare our technique to current surgical alternatives. We included articles from PubMed, Embase, Scopus, Ovid, and Science Direct published between 1963 and 2022 that were written in English, German, and Spanish. The terms used were the following: "high grade spondylolisthesis," "spondyloptosis," "surgical management," "interbody fusion," and "arthrodesis." In all, 485 articles were displayed, from which we filtered 112 by title and abstract. At the end, 75 references were selected for the review. Different interbody fusion techniques can be used to correct the lumbosacral kyphosis and restore the spinopelvic parameters. A complete reduction of the listhesis is not always required. The surgical procedure carried out in our patient corresponds to the first known case of minimally invasive circumferential arthrodesis with iliac screws and sacral fixation in a high-grade dysplastic spondylolisthesis. This approach guarantees the correction of the lumbosacral kyphosis and a complete reduction of the listhesis. Further studies are required to determine whether the results of this case can be extrapolated to other patients with high-grade spondylolisthesis.

Suramin, a drug for the treatment of trypanosomiasis, reduces the prothrombotic and metastatic phenotypes of colorectal cancer cells by inhibiting hepsin.

Recently, our group identified serine-protease hepsin from primary tumor as a biomarker of metastasis and thrombosis in patients with localized colorectal cancer. We described hepsin promotes invasion and thrombin generation of colorectal cancer cells in vitro and in vivo and identified venetoclax as a hepsin inhibitor that suppresses these effects. Now, we aspire to identify additional hepsin inhibitors, aiming to broaden the therapeutic choices for targeted intervention in colorectal cancer. METHODS: We developed a virtual screening based on molecular docking between the hepsin active site and 2000 compounds from DrugBank. The most promising drug was validated in a hepsin activity assay. Subsequently, we measured the hepsin inhibitor effect on colorectal cancer cells with basal or overexpression of hepsin via wound-healing, gelatin matrix invasion, and plasma thrombin generation assays. Finally, a zebrafish model determined whether hepsin inhibition reduced the invasion of colorectal cancer cells overexpressing hepsin. RESULTS: Suramin was the most potent hepsin inhibitor (docking score: -11.9691 Kcal/mol), with an IC50 of 0.66 µM. In Caco-2 cells with basal or overexpression of hepsin, suramin decreased migration and significantly reduced invasion and thrombin generation. Suramin did not reduce the thrombotic phenotype in the hepsin-negative colorectal cancer cells HCT-116 and DLD-1. Finally, suramin significantly reduced the in vivo invasion of Caco-2 cells overexpressing hepsin. CONCLUSION: Suramin is a novel hepsin inhibitor that reduces its protumorigenic and prothrombotic effects in colorectal cancer cells. This suggests the possibility of repurposing suramin and its derivatives to augment the repertoire of molecular targeted therapies against colorectal cancer.

Emergency department patient navigators successfully connect patients to care within a rapidly evolving healthcare system.

In the United States, adherence to follow up medical appointments among patients discharged from the emergency department varies between 26% and 56%, depending on the population. It is well known that patients face significant barriers to care within an increasingly complicated system of care. In an effort to better support patients, in 2020, NewYork-Presbyterian Queens implemented a Patient Navigator Program with 7 bilingual Patient Navigators who were trained to deliver culturally sensitive education and support, and to schedule follow up appointments for patients experiencing barriers to care. Between February 2020 and December 2022, 30,164 patients were supported by the 7 Patient Navigators. Ninety-four percent of patients without a primary care provider had a new provider and appointment upon discharge, and 81% of patients attended the appointment scheduled by the Patient Navigator. This study demonstrates that Patient Navigators can work alongside clinical colleagues, and as members of emergency department health care teams, to support patients to connect to care and to attend follow up appointments. It also highlights that Patient Navigators are uniquely qualified to build trust and to support patients to achieve appropriate, continuous care within a rapidly evolving health care system.

Venetoclax is a potent hepsin inhibitor that reduces the metastatic and prothrombotic phenotypes of hepsin-expressing colorectal cancer cells.

Introduction: Hepsin is a type II transmembrane serine protease and its expression has been linked to greater tumorigenicity and worse prognosis in different tumors. Recently, our group demonstrated that high hepsin levels from primary tumor were associated with a higher risk of metastasis and thrombosis in localized colorectal cancer patients. This study aims to explore the molecular role of hepsin in colorectal cancer. Methods: Hepsin levels in plasma from resected and metastatic colorectal cancer patients were analyzed by ELISA. The effect of hepsin levels on cell migration, invasion, and proliferation, as well as on the activation of crucial cancer signaling pathways, was performed in vitro using colorectal cancer cells. A thrombin generation assay determined the procoagulant function of hepsin from these cells. A virtual screening of a database containing more than 2000 FDA-approved compounds was performed to screen hepsin inhibitors, and selected compounds were tested in vitro for their ability to suppress hepsin effects in colorectal cancer cells. Xenotransplantation assays were done in zebrafish larvae to study the impact of venetoclax on invasion promoted by hepsin. Results: Our results showed higher plasma hepsin levels in metastatic patients, among which, hepsin was higher in those suffering thrombosis. Hepsin overexpression increased colorectal cancer cell invasion, Erk1/2 and STAT3 phosphorylation, and thrombin generation in plasma. In addition, we identified venetoclax as a potent hepsin inhibitor that reduced the metastatic and prothrombotic phenotypes of hepsin-expressing colorectal cancer cells. Interestingly, pretreatment with Venetoclax of cells overexpressing hepsin reduced their invasiveness in vivo. Discussion: Our results demonstrate that hepsin overexpression correlates with a more aggressive and prothrombotic tumor phenotype. Likewise, they demonstrate the antitumor role of venetoclax as a hepsin inhibitor, laying the groundwork for molecular-targeted therapy for colorectal cancer.

Feasibility of Food FARMacia: Mobile Food Pantry to Reduce Household Food Insecurity in Pediatric Primary Care.

Despite recommendations for systematic food insecurity screening in pediatric primary care, feasible interventions in clinical settings are lacking. The goal of this study was to examine reach, feasibility, and retention in Food FARMacia, a pilot clinically based food insecurity intervention among children aged <6 years. We examined electronic health record data to assess reach and performed a prospective, longitudinal study of families in Food FARMacia (May 2019 to January 2020) to examine attendance and retention. We used descriptive statistics and bivariate analyses to assess outcomes. Among 650 pediatric patients, 172 reported household food insecurity and 50 registered for Food FARMacia (child mean age 22 ± 18 months; 88% Hispanic/Latino). Demographic characteristics of Food FARMacia participants were similar to those of the target group. Median attendance rate was 75% (10 sessions) and retention in both the study and program was 68%. Older child age (retention: age 26.7 ± 18.7 months vs. attrition: age 12.1 ± 13.8 months, p = 0.01), Hispanic/Latino ethnicity (retention: 97% vs. attrition: 69%, p < 0.01), and larger household size (retention: 4.5 ± 1.1 vs. attrition: 3.7 ± 1.4, p = 0.04) correlated with retention. A clinically based mobile food pantry pilot program and study reached the target population and were feasible.

Assessment of a Fruit and Vegetable Prescription Program in the Northern Manhattan Community.

PURPOSE: This study aimed to assess whether produce prescription redemption was associated with food insecurity (FI), sociodemographics, and nutrition-related health measures, and to identify factors affecting participation. DESIGN: Retrospective, cross-sectional study. Patients, equally divided between groups who redeemed and did not redeem prescriptions, completed a follow-up survey. SETTING: Northern Manhattan, NY. SUBJECTS: 242 patients referred to Nutrition at an academic medical center between June and November 2019. INTERVENTION: All patients referred to Nutrition received prescriptions for produce at local Greenmarkets (patients with FI received $20; other patients received $10). MEASURES: We assessed patient satisfaction and factors impacting participation. Sociodemographics and nutrition-related health measures were extracted from medical records. ANALYSIS: The χ2 test for categorical data and Student's t-test for continuous variables. RESULTS: Prescription redeemers were significantly more likely to be very satisfied with the program (P < .001), have FI (P < .01), and have elevated hemoglobin A1C than non-redeemers (6.3 vs 5.5%, P < .001). Distance, time constraints, and forgetting or losing the prescription were common barriers, while convenience and valuing healthy eating facilitated redemption. CONCLUSION: Higher FI and worse hemoglobin A1c in patients who redeemed prescriptions suggests that our program reaches the target audience: patients needing food assistance and a healthier diet. Awareness of barriers offers areas for improvement. This provides a feasible model for hospital investment to increase access to produce to improve health and health equity.

Social Determinants of Health and COVID-19 Among Patients in New York City.

Background: Covid-19 testing and disease outcomes according to demographic and neighborhood characteristics must be understood. Methods: Using aggregate administrative data from a multi-site academic healthcare system in New York from March 1 - May 14, 2020, we examined patient demographic and neighborhood characteristics according to Covid-19 testing and disease outcomes. Results: Among the 23,918 patients, higher proportions of those over 65 years old, male sex, Hispanic ethnicity, Medicare, or Medicaid insurance had positive tests, were hospitalized, or died than those with younger age, non-Hispanic ethnicity, or private insurance. Patients living in census tracts with more non-White individuals, Hispanic individuals, individuals in poverty, or housing crowding had higher proportions of Covid-19 positive tests, hospitalizations, and deaths than counterparts. Discussion: Variation exists in Covid-19 testing and disease outcomes according to patient and neighborhood characteristics. There is a need to monitor Covid-19 testing access and disease outcomes and resolve racist policies and practices.

Distinct mechanisms regulate Cdx2 expression in the blastocyst and in trophoblast stem cells.

The first intercellular differences during mammalian embryogenesis arise in the blastocyst, producing the inner cell mass and the trophectoderm. The trophectoderm is the first extraembryonic tissue and does not contribute to the embryo proper, its differentiation instead forming tissues that sustain embryonic development. Crucial roles in extraembryonic differentiation have been identified for certain transcription factors, but a comprehensive picture of the regulation of this early specification is still lacking. Here, we investigated whether the regulatory mechanisms involved in Cdx2 expression in the blastocyst are also utilized in the postimplantation embryo. We analyzed an enhancer that is regulated through Hippo and Notch in the blastocyst trophectoderm, unexpectedly finding that it is inactive in the extraembryonic structures at postimplantation stages. Further analysis identified other Cdx2 regulatory elements including a stem-cell specific regulatory sequence and an element that drives reporter expression in the trophectoderm, a subset of cells in the extraembryonic region of the postimplantation embryo and in trophoblast stem cells. The cross-comparison in this study of cis-regulatory elements employed in the blastocyst, stem cell populations and the postimplantation embryo provides new insights into early mammalian development and suggests a two-step mechanism in Cdx2 regulation.

Ablación de taquicardia ventricular en displasia arritmogénica del ventrículo derecho / Ablation of tachycardia in arrhythmogenic right ventricular dysplasia

La displasia arritmogénica del ventrículo derecho es una cardiopatía de origen genético, su importancia radica en la capacidad de generar muerte súbita en pacientes en la tercera y cuartadécada de la vida, después de grandes esfuerzos por aumentar la sensibilidad y mejorar la capacidad diagnóstica continúa siendo un importante problema de salud pública. Los desfibriladores implantables han demostrado aumentar la supervivencia de quienes presentan arritmias letales asociadas, sin embargo, debido al carácter progresivo de la enfermedad un interrogante de importancia es cómo tratar a los pacientes con terapias apropiadas y frecuentes de estos dispositivos, la ablación por radiofrecuencia es una respuesta terapéutica seria a este dilema. En la actualidad disponemos de técnicas de ablación que combinan los métodos de la imagen TC cardiaca, la resonancia cardiaca, los mapas electroanatómicos y algunas herramientas de la electrofisiología convencional que permiten realizar ablaciones sin la inducción de arritmias ventriculares de forma sostenida y durante el ritmo sinusal, de modo similar el mejor entendimiento de la patogenia introdujo el uso de técnicas híbridas endo y epicárdica, la suma de cada uno de estos avances ha aumentado la tolerancia durante el procedimiento, ha mejorado los resultados en las etapas agudas postablación y en los seguimientos a mediano plazo, hoy los márgenes de seguridad y eficacia para esta técnica se incrementan, siendo de primera línea en diferentes situaciones de relevancia en esta compleja enfermedad.

Arrhythmogenic right ventricular dysplasia (ARVD) is a genetic disease associated with sudden cardiac death, affecting subjects in the 3rd and 4th decade of life. Despite great efforts made in order to improve its early diagnosis, ARVD remains as a major public health problem in Europe and America. Currently, risk stratification of sudden cardiac death in patients with ARVD remains challenging. Over the last decade implantable defibrillators have been shown to increase survival of patients with structural heart disease and risk factors for sudden cardiac death. However, there is no consensus about how to treat patients with recurrent appropriate implantable defibrillators therapies. Recent studies have shown that radiofrequency ablation is an effective treatment for patients with recurrent episodes of ventricular tachycardia. Specifically, substrate ablation techniques have been shown to be especially useful in the case of ARVD, as these techniques allow performing ablation during sinus rhythm. Additionally, emerging tools as electroanatomic navigation, CT or MRI have provided further knowledge of the pathogenesis of ARVD, allowing the development of novel therapeutic approaches. In this review epidemiologic, pathogenic, diagnostic and therapeutic features of ARVD are discussed, with special focus on the treatment of ventricular arrhythmias associated with ARVD.

Notch and hippo converge on Cdx2 to specify the trophectoderm lineage in the mouse blastocyst.

The first lineage choice in mammalian embryogenesis is that between the trophectoderm, which gives rise to the trophoblast of the placenta, and the inner cell mass, from which is derived the embryo proper and the yolk sac. The establishment of these lineages is preceded by the inside-versus-outside positioning of cells in the early embryo and stochastic expression of key transcription factors, which is then resolved into lineage-restricted expression. The regulatory inputs that drive this restriction and how they relate to cell position are largely unknown. Here, we show an unsuspected role of Notch signaling in regulating trophectoderm-specific expression of Cdx2 in cooperation with TEAD4. Notch activity is restricted to outer cells and is able to influence positional allocation of blastomeres, mediating preferential localization to the trophectoderm. Our results show that multiple signaling inputs at preimplantation stages specify the first embryonic lineages.

The impact of integrating community health workers into the patient-centered medical home.

OBJECTIVES: Establishing patient-centered medical homes (PCMHs) that deliver comprehensive care can be challenging for practices serving diverse populations. Integrating community health workers (CHWs) as members of the care team is one approach to deliver such care; however, little is known about this process. We study an approach of integrating CHWs into PCMHs with predominantly Latino, pediatric asthma patients and examine the impact on care delivery. METHODS: A case study was conducted to examine the integration of 8 CHWs into 5 PCMHs in an academic medical center located in a large, urban setting. Data associated with referrals to CHW practice-based education and support and to a care coordination program, as well as survey results from providers on the impact of CHWs in PCMHs were collected and analyzed over the study period. RESULTS: More than 750 families of children with asthma received education and support from CHWs from February 2011 through December 2013. The number of referrals to the care coordination program increased 7-fold during this time (P < .001). Of providers surveyed (n = 55), those who reported not referring to care coordination decreased from 22% to 6% and those who reported referring patients to care coordination increased from 67% to 79%. Additionally, 39% of providers reported giving a care plan to patients when prompted by the CHW. CONCLUSIONS: CHWs can be successfully integrated into the PCMH care team. They may also impact the identification of high-risk populations for care coordination and delivery of comprehensive care.

In-classroom physical activity and its impact on physical activity outside of school in a Hispanic community.

BACKGROUND: School-based physical activity programs can reach large populations of at-risk children however evidence for the sustainability of healthy behaviors as a result of these programs is mixed. Healthy Schools Healthy Families (HSHF) is a physical activity and nutrition program for elementary students in a predominantly minority community. The program includes short teacher led classroom-based physical activities, also known as Transition Exercises (TE). Our aim was to assess whether TE was associated with children's reported recreational physical activity outside of school. METHODS: We surveyed HSHF students in grade 5 (n = 383) about their recreational physical activity at the start and end of the school year. Multivariable analysis was used to determine what factors including TE contributed to their reported activity. RESULTS: Students were predominantly Hispanic with a mean age of 10 ± .03. There was an increase in reported recreational physical activity from the start to the end of the school year (73.6% to 82.4%, P < .05). Students who participated in more TE had a 2.75 times greater odds of reporting participation in recreational activity than students who participated in less TE. CONCLUSIONS: For students in HSHF, TE was significantly associated with an increase in recreational physical activity.

Lesión pulmonar inducida por talidomida: reporte de caso / Thalidomide-induced lung injury: case report

El síndrome de dificultad respiratoria aguda (SDRA) es una entidad con alta mortalidad y entre sus causas bien establecidas, están ciertos medicamentos. En este reporte de caso, un hombre de 52 años con antecedentes de mieloma múltiple estadio IIIA, en tratamiento con bortezomip, doxorrubicina y dexametazona (BAD) y manejo ambulatorio con talidomida (200 mg/día), consultó por tos y disnea progresiva, asociada a picos febriles de una semana de evolución. Al ingreso la tensión arterial fue de 90/70 mm de Hg, la FC de 80 x min, la FR de 26 x min, con auscultación de crépitos bibasales y presentaba, además, hipoxemia y alcalemia respiratoria en los gases arteriales (pH: 7,48, PaCO2: 24 mm de Hg, PaO2: 40 mm de Hg, Sat 80%, HCO3: 18, FiO2: 21%), hemograma sin leucocitosis pero con hipereosinofilia de 18% y radiografía de tórax con infiltrados alveolares bilaterales. Se consideró neumonía por gérmenes atípicos, iniciándose manejo antibiótico, pero el paciente presentó deterioro del estado general, con falla respiratoria y requerimiento de ventilación mecánica. Todos los cultivos fueron negativos, incluso los del lavado alveolar y la biopsia pulmonar reportó neumonía eosinofílica, posiblemente por medicamentos, por lo cual se retiró la talidomida y se manejó con metilprednisolona, con resolución de los infiltrados alveolares, y la hipoxemia, lo que permitió la extubación al cabo de cuatro días. Este caso cumple con los criterios de lesión pulmonar inducida por medicamentos (relación temporal entre los síntomas y el uso del medicamento, presencia de hallazgos histopatológicos en la biopsia, exclusión de otras causas de SDRA y desaparición de los síntomas al iniciar esteroides y suspender el medicamento), siendo la talidomida el probable agente causal, coincidiendo con dos reportes similares publicados en la literatura mundial. La búsqueda, el reporte de nuevos casos y la realización de estudios prospectivos para determinar la posible relación entre la talidomida y la lesión pulmonar permitirá definir exactamente si existe relación causal, de modo que pueda hacerse un diagnóstico temprano, para reducir o evitar la morbimortalidad asociada.

Diagnostic evaluation and classification criteria in Sjögren's Syndrome.

OBJECTIVES: Our objective is to carry out a clinical study of the performance of the preliminary European classification criteria for Sjögren Syndrome and that of the criteria proposed by the American European Consensus Group. METHODS: Eighty-eight patients who had undergone a biopsy of the salivary gland on suspicion that they were suffering from Sjögren Syndrome were studied by two independent rheumatologists. Two pathologists independently revised the biopsies without knowing the diagnosis. With all of this information, the clinicians established, or did not establish, a diagnosis of primary Sjögren Syndrome or secondary Sjögren Syndrome. Finally, it was analysed whether or not the patients met the American European Consensus Group classification criteria and the preliminary European criteria for primary Sjögren Syndrome and secondary Sjögren Syndrome, and their sensitivity and specificity with respect to the clinical diagnosis were determined. RESULTS: Clinicians estimated that 35 cases (39.8%) had primary Sjögren Syndrome (kappa 0.718) and 17 cases (19.3%) had secondary Sjögren Syndrome (kappa 0.761). The specificity and sensitivity of American European Consensus Group criteria, with regard to the clinical diagnosis, for primary Sjögren Syndrome were 97.2% and 48.6%, respectively. For secondary Sjögren Syndrome, the specificity was 97.2% and the sensitivity 64.7%. The preliminary European criteria for primary Sjögren Syndrome demonstrated a lesser specificity (75%), but a higher sensitivity (65.7%). In secondary Sjögren Syndrome the specificity reached 97.2% with sensitivity at 70.6%. CONCLUSIONS: These results underline the difficulty in applying the Sjögren Syndrome classification criteria from the American European Consensus Group and the preliminary European criteria, in the diagnosis of individual patients.

[Asymmetric septal hypertrophy associated with "subclinical hypothyroidism"]. / Hipertrofia septal asimétrica reversible asociada a "hipotiroidismo subclínico".

We report a case of hypothyroid cardiomyopathy manifested as reversible asymmetric septal hypertrophy in a 61-year-old white woman diagnosed 20 years previously with primary hypothyroidism.

[Role of coronary artery revascularization and aneurysmectomy in ventricular arrhythmias in the chronic phase of myocardial infarction]. / Papel de la revascularización coronaria y la aneurismectomía en las arritmias ventriculares asociadas a infarto de miocardio crónico.

INTRODUCTION AND OBJECTIVES: The influence of coronary artery revascularization on the control of ventricular arrhythmias in patients with chronic myocardial infarction is uncertain. However, ablation of the arrhythmogenic circuit in these patients by aneurysm resection is useful for controlling ventricular arrhythmias. We made a prospective analysis of our clinical strategy in patients who were candidates for coronary artery revascularization and/or aneurysmectomy to determine its influence on the recurrence of ventricular arrhythmias. PATIENTS AND METHOD: Prospective study of 17 consecutive patients with chronic myocardial infarction and ventricular arrhythmias unrelated with an acute ischemic event, who had coronary artery disease and/or ventricular aneurysm susceptible to aggressive treatment. We evaluated our clinical strategy and the recurrence of ventricular arrhythmias during a mean follow-up period of 33.64 months. RESULTS: Two groups of patients were studied: patients with ventricular aneurysm (group I: 12 patients) and patients without ventricular aneurysm (group II: 5 patients). Seven patients of group I underwent endoaneurysmorrhaphy and endocardial resection (4 of these patients had associated revascularization procedures). Three patients were not candidates for aneurysmectomy or revascularization procedures. Two patients underwent only revascularization procedures. All the patients in group II were revascularized. The patients who underwent aneurysmectomy did not have recurrence of arrhythmias. In 5 of the 6 patients who underwent programmed electrophysiological stimulation after aneurysmectomy, no sustained arrhythmia could be induced. Patients who were only revascularized had a high rate of recurrence of ventricular arrhythmias (57%), which were inducible after revascularization. CONCLUSION: Aneurysmectomy and endocardial resection constituted, in our experience, an effective tool for controlling ventricular arrhythmias associated with left ventricular aneurysm. Coronary artery revascularization in patients with ventricular arrhythmias and chronic myocardial infarction probably does not prevent the recurrence of ventricular arrhythmias.

Papel de la revascularización coronaria y la aneurismectomía en las arritmias ventriculares asociadas a infartode miocardio crónico / Role of Coronary Artery Revascularization and Aneurysmectomy in Ventricular Arrhythmias in the Chronic Phase of Myocardial Infarction

Introducción y objetivos. La influencia de la revascularización coronaria en el control de las arritmias ventriculares, en pacientes con infarto de miocardio crónico, es incierta y el control de éstas es más evidente cuando se consigue resecar el circuito arritmogénico de un aneurisma. En este sentido, revisamos prospectivamente nuestra estrategia clínica en estos pacientes subsidiarios de revascularización coronaria y/o aneurismectomía con el objetivo de comprobar su influencia en la recurrencia de la arritmia. Pacientes y método. Estudio prospectivo de 17 pacientes consecutivos con infarto de miocardio crónico y arritmias ventriculares no relacionadas con isquemia aguda, que presentaban enfermedad coronaria y/o aneurisma ventricular subsidiario de tratamiento intervencionista. Se analiza la estrategia desarrollada y la recurrencia de las arritmias ventriculares a lo largo de un seguimiento de 33,64 meses de media. Resultados. Se consideraron 2 grupos de pacientes, según presentaran (grupo I) o no (grupo II) aneurisma de ventrículo izquierdo. De los 12 pacientes del grupo I, en siete se realizó endoaneurismorrafia y resección endocárdica (además, cuatro fueron revascularizados). Tres pacientes no fueron intervenidos por presentar un aneurisma no resecable y no ser subsidiario de revascularización. Dos pacientes sólo fueron revascularizados. Todos los pacientes del grupo II (5 pacientes sin aneurisma) fueron revascularizados. Los enfermos sometidos a aneurismectomía no tuvieron recurrencia de la arritmia, y ésta no se indujo en cinco de los 6 pacientes a los que se realizó estudio electrofisiológico postintervención. Los pacientes exclusivamente revascularizados presentaron una alta recurrencia arrítmica (57 por ciento) y su arritmia podía ser inducible tras la revascularización. Conclusiones. La aneurismectomía complementada con resección endocárdica constituyó, según nuestra experiencia, una eficaz herramienta para el control de las arritmias ventriculares asociadas a aneurisma de ventrículo izquierdo. La revascularización coronaria en pacientes con arritmias ventriculares e infarto de miocardio crónico no parece influir en la prevención de recurrencia arrítmica (AU)

Association between a high-expressing interferon-gamma allele and a lower frequency of kidney angiomyolipomas in TSC2 patients.

Tuberous sclerosis complex (TSC) is a familial hamartoma syndrome in which renal involvement is common and, at times, life threatening. We have investigated the potential effect of a non-TSC gene on renal disease in a cohort of 172 TSC patients with TSC2 mutations. Patients were genotyped for an interferon-gamma (IFN-gamma) microsatellite polymorphism, within intron 1, for which one common allele (allele 2, with 12 CA repeats) has been shown to have a higher expression of IFN-gamma. A chi(2) analysis was used to examine the association between IFN-gamma allele 2 and the development of kidney angiomyolipomas (KAMLs) in this TSC2 cohort. Because of the age-dependent development of KAMLs in TSC, we initially focused on the 127 patients who were >5 years old. Additional subgroup analyses were done to investigate the influence of age and gender. The transmission/disequilibrium test (TDT) was also performed in a subset of this cohort (46 probands) for whom parent and/or sibling samples were available for analysis. Both chi(2) analysis and TDT suggested an association between IFN-gamma allele 2 and the absence of KAMLs in patients who have known TSC2 mutations. Among the 127 patients who were >5 years old, KAMLs were present in 95 (75%) and were absent in 32 (25%). In the group with KAML present, the frequency of IFN-gamma allele 2 was 56%; in the group with KAML absent, the frequency of IFN-gamma allele 2 was significantly higher, at 78% (P=.02, by chi(2) analysis). The family-based TDT analysis gave similar results, with a TDT statistic (TDT chi2=5.45) corresponding to a P value of.02. Subgroup analyses show that both age and gender may influence the impact of this association. Although these results should be replicated in other populations with TSC, the present study suggests that modifier genes play a role in the variable expression of TSC and also suggests a potential therapy for KAMLs in patients with TSC.

Erupción papulosa aislada y confluente en tronco y manos en varón con infección por el Virus de la Inmunodeficiencia Humana / Circumscribeb and confluent papular rash in the trunk and hands in male patient infected with the human immunodeficency virus

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