Hospital General Universitario Gregorio Marañón

About: Hospital General Universitario Gregorio Marañón is a healthcare organization based out in Madrid, Spain. It is known for research contribution in the topics: Population & Transplantation. The organization has 11975 authors who have published 12386 publications receiving 244847 citations.

Defining enthesitis in spondyloarthritis by ultrasound: results of a Delphi process and of a reliability reading exercise.

Abstract: Objective: To standardize ultrasound (US) in enthesitis. Methods: An initial Delphi exercise was undertaken to define US-detected enthesitis and its core components. These definitions were subsequently tested on static images taken from spondyloarthritis patients in order to evaluate their reliability. Results: Excellent agreement (>80%) was obtained for including hypoechogenicity, increased thickness of the tendon insertion, calcifications, enthesophytes, erosions, and Doppler activity as core elementary lesions of US-detected enthesitis. US definitions were subsequently obtained for each elementary component. On static images, the intraobserver reliability showed a high degree of variability for the detection of elementary lesions, with kappa coefficients ranging from 0.13-1. The interobserver kappa values were variable, with the lowest kappa coefficient for enthesophytes (0.24) and the highest coefficient for Doppler activity at the enthesis (0.63). Conclusion: This is the first consensus-based US definition of enthesitis and its elementary components and the first step performed to ensure a higher degree of homogeneity and comparability of results between studies and in daily clinical work.

Estudio MASCARA (Manejo del Síndrome Coronario Agudo. Registro Actualizado). Resultados globales

Abstract: Introduccion y objetivos Determinar el perfil clinico, el manejo del sindrome coronario agudo (SCA) y el efecto de la estrategia intervencionista precoz (EIP) en el SCA sin elevacion del ST (SCASEST) y del intervencionismo coronario percutaneo (ICP) primario en el SCA con elevacion del ST (SCACEST). Metodos Inclusion prospectiva en 50 hospitales seleccionados aleatoriamente segun nivel asistencial, durante 9 meses entre 2004 y 2005, y seguimiento a 6 meses de la mortalidad o el reingreso por SCA. Se analizo el efecto ajustado de las estrategias de reperfusion. Resultados Tras control de calidad, se analizaron los datos de 32 hospitales, correspondientes a 7.923 acontecimientos coronarios (4.431 SCASEST [56%], 3.034 SCACEST [38%] y 458 SCA inclasificable [6%]) de 7.251 pacientes. Respecto a registros anteriores, destaca un incremento del ICP primario en el SCACEST (del 10,7 al 36,8% de los reperfundidos) y la EIP en el SCASEST (del 11,1 al 19,6%). La mortalidad hospitalaria total fue del 5,7% (intervalo de confianza [IC] del 95%, 5,1%-6,2%); del SCACEST, el 7,6% (IC del 95%, 6,7%-8,7%); del SCASEST, el 3,9% (IC del 95%, 3,3%-4,6%), y del indeterminado, el 8,8% (IC del 95%, 6,2%-12,2%). No se observo, en el total de la poblacion, relacion con el pronostico (mortalidad a 6 meses) de la EIP en el SCASEST (hazard ratio [HR] = 0,94; IC del 95%, 0,66-1,3) ni del ICP primario en el SCACEST (HR = 1; IC del 95%, 0,7-1,5). Se observaron resultados similares con la variable muerte o reingreso por SCA a 6 meses. Conclusiones En 2004-2005 se registro en Espana un aumento de estrategias invasivas. Se observo una insuficiente adecuacion de estas al riesgo basal de los pacientes.

Interleukin-7 receptor alpha (IL-7Ralpha) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients.

Abstract: Analysis of gene-targeted mice and patients with severe combined immunodeficiency due to mutations of the alpha chain of the interleukin-7 receptor (IL-7Ralpha) has shown important differences between mice and humans in the role played by IL-7 in lymphoid development. More recently, it has been shown that IL-7Ralpha is also shared by the receptor for another cytokine, thymic stromal lymphopoietin (TSLP). In this review, we discuss recent advances in IL-7- and TSLP-mediated signaling. We also report on the clinical and immunological features of 16 novel patients with IL-7Ralpha deficiency and discuss the results of hematopoietic stem cell transplantation.

DQB1 locus alone explains most of the risk and protection in narcolepsy with cataplexy in Europe.

Abstract: STUDY OBJECTIVE: Prior research has identified five common genetic variants associated with narcolepsy with cataplexy in Caucasian patients To replicate and/or extend these findings, we have tested HLA-DQB1, the previously identified 5 variants, and 10 other potential variants in a large European sample of narcolepsy with cataplexy subjects DESIGN: Retrospective case-control study SETTING: A recent study showed that over 76% of significant genome-wide association variants lie within DNase I hypersensitive sites (DHSs) From our previous GWAS, we identified 30 single nucleotide polymorphisms (SNPs) with P < 10(-4) mapping to DHSs Ten SNPs tagging these sites, HLADQB1, and all previously reported SNPs significantly associated with narcolepsy were tested for replication PATIENTS AND PARTICIPANTS: For GWAS, 1,261 narcolepsy patients and 1,422 HLA-DQB1*06:02-matched controls were included For HLA study, 1,218 patients and 3,541 controls were included MEASUREMENTS AND RESULTS: None of the top variants within DHSs were replicated Out of the five previously reported SNPs, only rs2858884 within the HLA region (P < 2x10(-9)) and rs1154155 within the TRA locus (P < 2x10(-8)) replicated DQB1 typing confirmed that DQB1*06:02 confers an extraordinary risk (odds ratio 251) Four protective alleles (DQB1*06:03, odds ratio 017, DQB1*05:01, odds ratio 056, DQB1*06:09 odds ratio 021, DQB1*02 odds ratio 076) were also identified CONCLUSION: An overwhelming portion of genetic risk for narcolepsy with cataplexy is found at DQB1 locus Since DQB1*06:02 positive subjects are at 251-fold increase in risk for narcolepsy, and all recent cases of narcolepsy after H1N1 vaccination are positive for this allele, DQB1 genotyping may be relevant to public health policy

Impact of hematologic malignancy and type of cancer therapy on COVID-19 severity and mortality: lessons from a large population-based registry study.

Abstract: Patients with cancer have been shown to have a higher risk of clinical severity and mortality compared to non-cancer patients with COVID-19. Patients with hematologic malignancies typically are known to have higher levels of immunosuppression and may develop more severe respiratory viral infections than patients with solid tumors. Data on COVID-19 in patients with hematologic malignancies are limited. Here we characterize disease severity and mortality and evaluate potential prognostic factors for mortality. In this population-based registry study, we collected de-identified data on clinical characteristics, treatment and outcomes in adult patients with hematologic malignancies and confirmed severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection within the Madrid region of Spain. Our case series included all patients admitted to 22 regional health service hospitals and 5 private healthcare centers between February 28 and May 25, 2020. The primary study outcome was all-cause mortality. We assessed the association between mortality and potential prognostic factors using Cox regression analyses adjusted for age, sex, comorbidities, hematologic malignancy and recent active cancer therapy. Of 833 patients reported, 697 were included in the analyses. Median age was 72 years (IQR 60–79), 413 (60%) patients were male and 479 (69%) and 218 (31%) had lymphoid and myeloid malignancies, respectively. Clinical severity of COVID-19 was severe/critical in 429 (62%) patients. At data cutoff, 230 (33%) patients had died. Age ≥ 60 years (hazard ratios 3.17–10.1 vs 2 comorbidities (1.41 vs ≤ 2), acute myeloid leukemia (2.22 vs non-Hodgkin lymphoma) and active antineoplastic treatment with monoclonal antibodies (2·02) were associated with increased mortality; conventional chemotherapy showed borderline significance (1.50 vs no active therapy). Conversely, Ph-negative myeloproliferative neoplasms (0.33) and active treatment with hypomethylating agents (0.47) were associated with lower mortality. Overall, 574 (82%) patients received antiviral therapy. Mortality with severe/critical COVID-19 was higher with no therapy vs any antiviral combination therapy (2.20). In this series of patients with hematologic malignancies and COVID-19, mortality was associated with higher age, more comorbidities, type of hematological malignancy and type of antineoplastic therapy. Further studies and long-term follow-up are required to validate these criteria for risk stratification.