A Study of the Relationship between Inflammatory Immune Function and Intestinal Flora in Adolescent Patients with First-Episode Depression

Background: Depression has become one of the most common mood disorders in adolescents, with an increasing incidence each year. Abnormal activation of peripheral immunity causes an increase in pro-inflammatory factors, which in turn affects neuroendocrine dysfunction and alters neurobiochemistry, leading to depression. In this study, we aimed to explore the relationship between inflammatory immune function and intestinal flora in adolescents with first-episode depression. Methods: A total of 170 cases of adolescent patients with first-episode depression who attended our hospital from January 2020 to March 2023 were retrospectively selected as the observation group. Simultaneously, 170 individuals who underwent a healthy physical examination during the same period were chosen as the control group. The enzyme-linked immunosorbent assay (ELISA) was employed to quantify the levels of monoamine neurotransmitters 5-hydroxytryptamine (5-HT), substance P (SP), neuropeptide Y (NPY), serum tumor necrosis factor-α (TNF-α), interleukin (IL)-1β, and IL-6 in the patients. Flow cytometry was utilized to assess the levels of T-lymphocytes CD3+, CD4+, and CD8+ cells. The levels of 16S ribosomal RNA (16SrRNA) method were used to determine the intestinal flora of the subjects in both groups. Inflammatory factor levels, immune function, and intestinal flora expression were observed, and correlation analysis was performed. Results: The levels of 5-HT and NPY in the observation group were lower than those in the control group. The SP level was significantly higher in the observation group compared to the control group (p < 0.05). The observation group demonstrated significantly higher TNF-α, IL-1β, and IL-6 levels than the control group (p < 0.05). The values of CD3+, CD4+, CD4+/CD8+ in the observation group were lower than those in the control group (p < 0.05), whereas the CD8+ values were notably higher (p < 0.05). ... (AU)

Documento de consenso sobre los medicamentos biosimilares en enfermedades inmunomediadas en España / Consensus statement on the use of biosimilar drugs in immune-mediated diseases in Spain

Objetivo: Mejorar el nivel de conocimiento sobre los medicamentos biosimilares y generar un marco consensuado sobre su uso. Métodos: Estudio cualitativo. Se seleccionó un grupo multidisciplinar de expertos en medicamentos biosimilares (una dermatóloga, un farmacéutico de hospital, un reumatólogo y un gastroenterólogo) que definieron los apartados y los temas del documento. Se realizó una revisión narrativa de la literatura en Medline para identificar artículos sobre los medicamentos biosimilares. Se seleccionaron revisiones sistemáticas de la literatura, estudios controlados pre-clínicos, clínicos y en vida real. Con esta información se generaron varios principios generales y recomendaciones. El grado de acuerdo con los mismos se estableció mediante un Delphi que se extendió a 66 profesionales de la salud que votaron de 1 (totalmente en desacuerdo) a 10 (totalmente de acuerdo). Se definió acuerdo si al menos el 70% de los participantes votaron ≥7. Resultados: La revisión de la literatura incluyó 555 artículos. Se votaron un total de 10 principios generales y recomendaciones. Todos alcanzaron el nivel de acuerdo establecido en el Delphi. El documento incluye datos sobre las características principales de los medicamentos biosimilares (definición, desarrollo, aprobación, extrapolación de indicaciones, intercambiabilidad, financiación y trazabilidad); sobre la evidencia publicada (biosimilitud, eficacia, efectividad, seguridad, inmunogenicidad, eficiencia, switch); sobre barreras y facilitadores a su uso, y datos sobre la información para pacientes. Conclusiones: Los medicamentos biosimilares autorizados reúnen todas las características de calidad, eficacia y seguridad. Además, ayudan significativamente a mejorar el acceso de los pacientes a las terapias biológicas y contribuyen a la sostenibilidad de los sistemas sanitarios. (AU)

Objective: To improve knowledge about biosimilar medicines and to generate a consensus framework on their use. Methods: Qualitative study. A multidisciplinary group of experts in biosimilar medicines was established (1dermatologist, 1hospital pharmacist, 1rheumatologist, and 1gastroenterologist) who defined the sections and topics of the document. A narrative literature review was performed in Medline to identify articles on biosimilar medicines. Systematic reviews, controlled, pre-clinical, clinical, and real-life studies were selected. Based on the results of the review, several general principles and recommendations were generated. The level of agreement was tested in a Delphi that was extended to 66 health professionals who voted from 1 (totally disagree) to 10 (totally agree). Agreement was defined if at least 70% of the participants voted ≥7. Results: The literature review included 555 articles. A total of 10 general principles and recommendations were voted upon. All reached the level of agreement established. The document includes data on the main characteristics of biosimilar medicines (definition, development, approval, indication extrapolation, interchangeability, financing, and traceability); published evidence (biosimilarity, efficacy, effectiveness, safety, immunogenicity, efficiency, switch); barriers and facilitators to its use; and data on information for patients. Conclusions: Authorized biosimilar medicines meet all the characteristics of quality, efficacy, and safety. They also significantly help improve patient access to biological therapies and contribute to health system sustainability. (AU)

Demographic and clinical characterization of pediatric group patients with inborn errors of the immune system in a Colombian tertiary hospital

Introduction: In recent decades, there has been a growing increase in the diagnosis of patients with inborn errors of the immune system, formerly known as primary immunodeficiency dis-orders (PIDs). Timely diagnosis remains a challenge due to low clinical suspicion and poor edu-cation on the subject. It is estimated that between 70% and 90% of these pathologies remain underdiagnosed in our environment.Objective: The objective of this study is to characterize the demographic and clinical presen-tation of pediatric group patients with inborn errors of the immune system in a Colombian tertiary hospital.Methods: Retrospective descriptive study of 306 patients with a diagnosis of innate errors of the immune system who consulted the PID clinic between 2011 and 2018 in a high-complexity institution in Cali, Colombia.Results: Three-hundred and six patients were included. The median age was 4 years (IQR 2.3–7.7 years), and 59.5% of the patients were male. According to the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency classification for inborn errors of the immune system, the most common group was antibody deficiency in 74.8% (n=229), especially in the age group between 1 and 5 years. The least frequent in our pop-ulation was complement deficiency. Of the warning signs stipulated for these pathologies, the most frequent were the (1) need for intravenous antibiotics (32%), (2) difficulty growing (15.7%), (3) four or more episodes of ear infection (10.8%), and (4) abscesses in organs or cuta-neous abscesses (12.7%). No patient reported two or more episodes of pneumonia or sinusitis, and only 5.8% of the patients received a bone marrow transplant (AU)

Treatment and Outcome in Deficiency of Adenosine Deaminase 2: A Literature Review

Objectives: Deficiency of adenosine deaminase 2 (DADA2) is a rare disease with varying phenotypes and disease outcomes. We evaluated the treatment of DADA2 and explored the factors associated with disease outcome.Methods: A systemic literature review of DADA2 was conducted. Cases were included if they had documented detailed genotypes, phenotypes, treatment protocols, and outcomes. Patients were categorized as having uncontrolled and controlled disease. Factors associated with disease outcome were analyzed using logistic regression models.Results: The study population comprised 242 DADA2 patients with data on treatment protocols and responses, of whom 17 required no treatment. Tumor necrosis factor a inhibitors (TNFi) were effective in 78.6% (103/131). Hematological abnormalities and increased acute phase reactants are independently associated with the effectiveness of TNFi (OR, 0.21 [95%CI, 0.07-0.661; P=.007] and 9.62 [95%CI, 2.31-40.00; P=.002, respectively). Among the 225 patients requiring active treatment, 157 (69.8%) had controlled disease and 68 (30.2%) uncontrolled disease. Neither age of disease onset nor genotype was associated with disease outcome. Increased acute phase reactant values, constitutional symptoms, neurological symptoms, and treatment with TNFi were independently associated with disease control, while recurrent infections and severe vascular events were the main causes of mortality (10/21 and 6/21, respectively).Conclusion: In patients requiring treatment, symptoms of systemic inflammation and vasculitis and treatment with TNFi are associated with disease control. Recurrent infections and severe vascular events should be treated intensively, as they are the main causes of death. Hematological abnormalities should be monitored, as they decrease the effectiveness of TNFi (AU)

Objetivos: El déficit de adenosina desaminasa 2 (DADA2) es una enfermedad rara con diferentes fenotipos y una evolución variable de laenfermedad. Nuestro objetivo es resumir los tratamientos de DADA2 y explorar los factores asociados con la evolución de la enfermedad.Métodos: Se realizó una revisión bibliográfica sistémica de DADA2. Los casos que se incluyeron fueron aquellos que habían documentadoel genotipo, fenotipos, protocolo de tratamiento y evolución. Los pacientes se clasificaron en grupos controlados y no controlados. Losfactores asociados con la evolución de la enfermedad se analizaron con modelos de regresión logística.Resultados: Se incluyeron un total de 242 pacientes con DADA2 con los protocolos de su tratamiento y la respuesta al mismo, 17 de los cualesno requirieron tratamiento. La eficacia general de los inhibidores de TNF-a (TNFi) fue del 78,6% (103/131). Las anomalías hematológicasy el aumento de los reactantes de fase aguda se asociaron de forma independiente con la eficacia del TNFi, OR = 0,21 (IC del 95%: 0,07 a0,661, p = 0,007) y 9,62 (IC del 95%: 2,31 a 40,00, p = 0,002), respectivamente. Entre los 225 pacientes que requirieron tratamiento activo,157 (69,8%) pacientes estaban en el grupo controlado y 68 (30,2%) en el grupo no controlado. Ni la edad de inicio de la enfermedad niel genotipo se asociaron con la evolución de la enfermedad. El aumento de los reactantes de fase aguda (APR), el deterioro constitucional,los síntomas neurológicos y el tratamiento con TNFi, se asociaron de forma independiente con el control de la enfermedad, mientras quelas infecciones recurrentes y los eventos vasculares graves fueron las principales causas de mortalidad (10/21 y 6/21, respectivamente).Conclusión: Los síntomas de inflamación sistémica, la vasculitis y el tratamiento con TNFi se asociaron con el control de la enfermedad enaquellos pacientes con DADA2 que requirieron tratamiento.

Características epidemiológicas, clínicas y respuesta al tratamiento en 113 pacientes con colitis microscópica / Epidemiological and clinical characteristics, and response to treatment in 113 patients with microscopic colitis

Objetivo: Estudiar las características epidemiológicas, clínicas y la respuesta al tratamiento en pacientes con colitis microscópica. Pacientes y método: Se recopilaron retrospectivamente los datos epidemiológicos, clínicos, analíticos y endoscópicos de 113 pacientes con colitis microscópica. La respuesta al tratamiento se analizó en 104 de ellos. La eficacia y la recidiva tras la administración de budesonida se evaluaron mediante curvas de supervivencia (Kaplan-Meier). Resultados: El 78% de los pacientes fueron mujeres, con una edad media de 65 ± 16 años. En los fumadores, la edad media fue 10 años menor. Un 48% tenía alguna enfermedad inmunomediada concomitante. El 60% sufrió un único brote de la enfermedad. La presentación clínica fue similar en ambos subtipos, aunque los pacientes con colitis colágena tuvieron con mayor frecuencia un curso crónico (48 vs. 29%, p = 0,047). La tasa de remisión con budesonida fue del 93% (IC 95%: 82-98). La incidencia acumulada de recidiva, tras una mediana de seguimiento de 21 meses, fue del 39% (IC 95%: 26-54%): 19% al año, 32% a los dos años y 46% a los tres años de seguimiento. No hubo diferencias en la respuesta clínica a la budesonida en función del tabaquismo o del subtipo de colitis microscópica. Conclusiones: La colitis microscópica es más frecuente en mujeres de edad avanzada. El tabaco se asoció a una aparición más precoz de la enfermedad, aunque no influyó en la evolución clínica o en la respuesta al tratamiento. La mayoría (> 90%) de los pacientes tratados con budesonida alcanzaron la remisión, aunque casi la mitad recidivaron posteriormente.(AU)

Objective: To study the epidemiological and clinical characteristics, and response to treatment in patients with microscopic colitis. Patients and method: Epidemiological, clinical, blood test and endoscopic data were retrospectively collected from 113 patients with microscopic colitis. Response to treatment was analyzed in 104 of them. Efficacy and relapse after treatment with budesonide were assessed using survival curves (Kaplan-Meier). Results: 78% of the patients were women, with a mean age of 65 ± 16 years. In smokers, the mean age was 10 years younger. 48% of them had some concomitant autoimmune disease; 60% suffered a single outbreak of the disease. The clinical presentation was similar in both subtypes, although patients with collagenous colitis had a chronic course more frequently (48% vs. 29%, p = 0.047). The remission rate with budesonide was 93% (95% CI 82-98). The cumulative incidence of relapse, after a median follow-up of 21 months, was 39% (95% CI 26-54%): 19% at one year, 32% at two years, and 46% at three years of follow-up. There were no differences in clinical response to budesonide based on smoking habit or microscopic colitis subtype. Conclusions: Microscopic colitis is more frequent in elderly women. Smoking was associated with earlier onset of the disease, although it did not influence the clinical course or response to treatment. The majority (> 90%) of patients treated with budesonide achieved remission, although nearly half subsequently relapsed.(AU)

22q11.2 deletion syndrome: 20 years of experience from two pediatric immunology units and review of clues for diagnosis and disease management

INTRODUCTION AND OBJECTIVES: The purpose of this study was to evaluate patients diagnosed with 22q11.2 deletion syndrome and determine the clues directing to diagnosis and evaluation of immunological findings for excellent management of the disease. MATERIAL AND METHODS: Thirty-three pediatric patients with 22q11.2 deletion syndrome diag­nosed between 1998 and 2019 at Pediatric Immunology Division of Ege University Faculty of Medicine and SBU Izmir Dr Behcet Uz Children's Education and Research Hospital were evaluated. RESULTS: This study includes the largest case series reported from Turkey. Congenital car­diac anomalies were the most common pathology associated with the syndrome (90.9%). Hypocalcemic symptoms were observed in 13 patients (40%). Twenty-two of the 33 (66.6%) patients were diagnosed before two years of age. Autoimmune diseases, dysmorphic facial findings, recurrent infections, growth retardation, and speech impairment were other clues for diagnosis in older patients. Clinical spectrum and immunological abnormalities of this syn­drome are quite variable. All T-cell subset counts were less than 5th percentile below median by age in one patient (3%) and 10 patients had normal all T-cell subset counts (30.3%). Overall, 69.6% of the patients had normal IgG, IgA, and IgM levels and two patients had panhypogam­maglobulinemia. Recurrent infections were revealed in 75.7% of the patients during follow-up. CONCLUSIONS: Presence of cardiac anomaly is more helpful in the diagnosis, especially under two years of age. Patients with immunologically high or standard risk did not show any differ­ence in terms of numbers and severity of infections and autoimmunity

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Causas de muerte fetal: Un revisión de 38 pacientes / Causes associated to fetal death: A review study of 38 women

INTRODUCCIÓN: La pérdida del embarazo que ocurre tras las veinte semanas de gestación, se denomina muerte fetal (MF); es un evento que causa un gran impacto psicoemocional en la pareja afectada. La literatura médica afirma que, en casi la mitad de estos casos, no hay una causa conocida. Las causas principales están relacionadas son: síndrome antifosfolípido obstétrico (SAF), otras alteraciones inmunológicas (OIA), otros factores que pueden causar infarto placentario por coagulación, rotura prematura de membranas, preeclampsia y trombosis en la circulación útero-placentaria. MÉTODOS: Revisamos cuidadosamente la historia clínica y los estudios inmunológicos de una cohorte de 38 pacientes que han sufrido MF. RESULTADOS: Treinta y ocho pacientes (edades 36-42 años) fueron estudiadas. En más de la mitad de los pacientes (57 %) se diagnosticó SAF. El hipotiroidismo autoinmune (26 %), el anticuerpo antinuclear (24 %) comprendió el grupo de OIA. Once de 38 pacientes mostraron diferentes mutaciones de trombofilias. La hiperhomocisteinemia estuvo presente en el 53 % de los pacientes. CONCLUSIÓN: Las alteraciones inmunológicas y la trombofilia se asociaron con una proporción significativa de nuestros casos de MF. El diagnóstico de las causas evitables es necesario para evitar complicaciones obstétricas en embarazos futuros

INTRODUCTION: Pregnancy loss that occurs after the twenty weeks of gestation, termed foetal death (FD), is a rare event of pregnancy causing great psycho-emotional impact on the affected couple. Medical literature states that in nearly half of these cases, there is no known cause. Leading, causes are related to obstetric antiphospholipid syndrome (APS), other immunological alterations (OIA), other factors that may cause clotting placental infarction, premature rupture of membranes, preeclampsia, and thrombosis in the utero-placental circulation with subsequent FD. METHODS: We carefully reviewed the complete medical records and immunological studies of a cohort of 38 patients that have suffered FD. RESULTS: Thirty-eight patients (ages 36 - 42 years) were studied. In more than half of the patients (57%) APS was diagnosed. Autoimmune hypothyroidism (26%), antinuclear antibody (24%) comprised the group of OIA. Eleven out of 38 patients showed different thrombophilia mutations. Hyperhomocysteinemia was present in 53% of patients. CONCLUSION: Immunological alterations and thrombophilia were associated with a significant proportion of our FD cases. Diagnosis of preventable causes of FD is necessary in order to avoid any obstetric complications in future pregnancies

Inmunoterapia, cáncer y enfermedades reumatológicas: revisión de la literatura y serie de casos en un hospital universitario / Immunotherapy, cancer and rheumatological diseases: a review of the literature and a series of cases in a university hospital

La aparición en el campo de la oncología de moléculas terapéuticas en forma de anticuerpos monoclonales, cuyo objetivo consiste en estimular el propio sistema inmune del paciente para que sea este el encargado de destruir las células cancerígenas, ha revolucionado el tratamiento de diversos cánceres en los últimos años. Este tipo de terapia, denominada inmunoterapia, se caracteriza además por presentar efectos secundarios en forma de enfermedades autoinmunes que todavía estamos empezando a conocer. Desde el punto de vista de los efectos secundarios inmunomediados reumatológicos, podemos encontrar manifestaciones musculoesqueléticas mecánicas, inflamatorias o enfermedad autoinmune sistémica. El manejo terapéutico de estos efectos secundarios se mantiene variable debido a la ausencia de ensayos clínicos y de recomendaciones validadas, siendo el manejo multidisciplinar fundamental para tratar con éxito dichos casos. En este artículo presentamos nuestra serie de casos clínicos de pacientes en tratamiento con inmunoterapia y efectos secundarios inmunomediados reumatológicos en un hospital universitario

The appearance in the field of oncology of therapeutic molecules in the form of monoclonal antibodies, whose objective is to stimulate the patient's own immune system to be responsible for destroying cancer cells, has revolutionized the treatment of many cancers in recent years. This type of therapy, called immunotherapy, is also characterized by presenting side effects in the form of autoimmune diseases that we are still beginning to understand. From the point of view of the immune-mediated rheumatological side effects, we can find musculoskeletal manifestations, mechanical, inflammatory or systemic autoimmune diseases. The therapeutic approach to these side effects remains uncertain due to the absence of clinical trials and validated recommendations. The multidisciplinary management is crucial to successfully treat such cases. In the following manuscript, we will describe our case reports of rheumatologic immune-related adverse events in a university hospital

CD40 Ligand Deficiency

CD40 ligand deficiency (CD40L), currently classified as an inborn error of immunity affecting cellular and humoral immunity, prevalently emerges in boys within the first two years of life. It manifests itself as a decrease in serum IgG, IgA and IgE, with normal or high IgM, defects in T cell proliferation, and decrease in soluble CD40L. These accompany sinopulmonary and/or gastrointestinal infections, and there may be infections caused by pyogenic bacteria, opportunistic infections, autoimmune diseases, and neoplasms. Mild and moderate cases of this deficiency may respond well to prophylactic antibiotic therapy or to human immunoglobulin replacement therapy, in addition to the early treatment of infections. Severe cases can be treated with hematopoietic stem cell transplantation, which allows the healing of such patients, rather than sequelae and a poor progression. Thus, its differential diagnosis with other inborn errors of immunity is essential, especially CD40 deficiency and variable common immunodeficiency; the reason why we have proposed the present literature review

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Riesgo infeccioso en los tratamientos biológicos utilizados en enfermedades de base inmunológica / No disponible

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Programa de intervenção de enfermagem na vacinação dos doentes submetidos a transplante de células progenitoras da hematopoiese / Nursing intervention program on the vaccination of patients undergoing hematopoietic stem cell transplant

The vaccination project for patients undergoing Hematopoietic Stem Cell Trans-plant (HSCT) arose because they lose the immunization provided by vaccines administered prior to transplantation and are extremely vulnerable to infection. This program began in 2012, was based on the organization of the post-HSCT vaccination schedule in nursing consultations aimed at providing the patient with vaccination and verifying compliance with the National Vaccination Plan. The aim of this article is to evaluate the results of the implementation of the vaccination project of patients undergoing hematopoietic stem cell transplant. The method used was a retrospective quantitative analysis of the application of the project methodology, through the defined outcome quality indicator - vaccination rate of patients undergoing HSCT with the National Vaccination Plan. From 2017 to 2018, the number of telephone contacts made in the scope of vaccination was defined as a process quality indicator and the content analyzed. We covered a population of 348 people, 229 patients undergoing allogeneic HSCT and 119 autologous. We achieved vaccination rates of 100. By implementing improvement strategies over the years we have been able to improve project effectiveness and vaccination for a larger number of patients.The project plays an important role in the area of public health and rehabilitation of patients undergoing HSCT as it allows the patient to be linked to the caregiver community, which contributes to a greater connection and proximity of the person to the health unit. enabling them to better integrate into everyday life in the various dimensions of the person

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Síndrome de Down: un enfoque de las patologías respiratorias asociadas / Down's syndrome: an approach to the associated respiratory conditions

INTRODUCCIÓN: El síndrome de Down es una alteración cromosómica en donde la mayoría de los casos son causados por la presencia de una copia extra del cromosoma 21. Los niños con este tipo de trastorno son afectados comúnmente por padecimientos respiratorios recurrentes, lo que se debe a las características fenotípicas e inmunológicas presentes. OBJETIVO: Realizar la revisión y descripción de las manifestaciones respiratorias más comunes en el niño con SD en 67 pacientes evaluados y bajo seguimiento en el Hospital de Especialidades Pediátricas de Tuxtla Gutiérrez, Chiapas, México, así como de los factores anatómicos, inmunológicos y fisiológicos que las predisponen, por medio de la búsqueda de artículos publicados en PubMed, Google académico, Scielo y EBSCOHost. CONCLUSIONES: Los padecimientos respiratorios son de origen multifactorial. Estos se deben a la predisposición fenotípica de alteraciones craneofaciales, laríngeas, pulmonares y a alteraciones del estado inmunológico, por lo que la prevención es sumamente importante para evitarlos. Es de suma importancia el seguimiento multidisciplinario para realizar de forma intencionada un seguimiento integral que permita a estos niños gozar de una excelente calidad de vida

INTRODUCTION: Down syndrome is a chromosomal alteration where most cases are caused by the presence of an extra copy of chromosome 21. Children with this type of disorder are commonly affected by recurrent respiratory conditions, which is due to the phenotypic and immunological characteristics present. OBJECTIVE: Performs the review and description of the most common respiratory manifestations in the child with SD in a cohort of 67 patients evaluated and monitored at the pediatric specialty hospital of Tuxtla Gutiérrez, Chiapas, Mexico. As well as the anatomical, immunological and physiological factors that predispose them, by searching for articles published in PubMed, Google academic, Scielo and EBSCOHost. CONCLUSIONS: Respiratory conditions are of multifactorial origin. These are due to phenotypic predisposition of skull-facial, laryngeal, pulmonary alterations, and immune state disturbances. So prevention is extremely important to avoid them. Multidisciplinary follow-up is of paramount importance in order to intentionally carry out comprehensive monitoring that allows these children to enjoy an excellent quality of life

Concordancia entre la prueba de la tuberculina y el Interferon Gamma Release Assay-IGRA en pacientes con enfermedades inflamatorias mediadas por la inmunidad / Concordance between the test of the tuberculin and Interferon Gamma Release Assay-IGRA in patients with immune-mediated inflammatory diseases

INTRODUCCIÓN: Las terapias inmunosupresoras en el tratamiento de las enfermedades inflamatorias mediadas por la inmunidad (EIMI) predisponen a la tuberculosis, por lo que el cribado de infección tuberculosa latente (ITL) y su tratamiento reduce la probabilidad de progresión a tuberculosis activa. El objetivo del estudio fue analizar la concordancia entre la prueba de la tuberculina (PT) e "Interferon Gamma Release Assay-IGRA" en relación con el tipo de EIMI y tratamiento inmunosupresor (IS). MATERIAL Y MÉTODOS: Estudio transversal en pacientes con EIMI candidatos o en tratamiento IS remitidos para cribado de ITL, de Abril del 2017 hasta Mayo del 2018. Variables resultado fueron PT e IGRA. Variables explicativas: EIMI, IS, edad, sexo, vacunación BCG previa y factores de riesgo de tuberculosis. RESULTADOS: Se estudiaron 146 pacientes (33 [22,6%] vacunados con BCG, 1 [0,7%] con diagnóstico previo de tuberculosis y 22 [15,1%] originarios de país endémico). Índice de Kappa (k) fue de 0,338 entre PT e IGRA para la totalidad de la muestra. Menor concordancia en pacientes con enfermedad de Crohn (k=0,125), en los tratados con corticoides (k=0,222), vacunados con BCG (k=0,122) y en pacientes procedentes de países endémicos de tuberculosis (k=0,128). CONCLUSIONES: La concordancia entre la PT y el IGRA se ve afectada en pacientes con EIMI y en mayor medida en la enfermedad inflamatoria intestinal, con la corticoterapia, con la vacunación con BCG o en los procedentes de países endémicos

INTRODUCTION: The immunosuppressive therapies in the treatment of the immune-mediated inflammatory diseases (EIMI) predispose individuals to the tuberculosis, so the screening of latent tuberculosis infection (ITL) and the treatment reduces the likelihood of a progression to an active tuberculosis. The aim of the study was to analyze the concordance between the test of the tuberculin (PT) and "Interferon Gamma Release Assay-IGRA" in relation to the type of EIMI and the immunosuppressive treatment (IS). MATERIAL AND METHODS: Transversal study of patients with EIMI candidates or in treatment IS forwarded to the ITL screening, from April 2017 until May 2018. The outcome variables were PT and IGRA. The explicative variables were: EIMI, IS, age, gender, prior BCG vaccination and tuberculosis risk factors. RESULTS: A total of 146 patients were analyzed (33[22.6%] vaccinated with BCG, 1 [0.7%] with a pre-diagnosis of tuberculosis, and 22 [15.1%] from an endemic country). Kappa index (k) was 0,338 between PT and IGRA for the whole sample. A lower concordance was found in patients with the Crohn's disease (k=0.125), in the ones treated with corticosteroids (k=0.222), vaccinated with BCG (k=0.122) and in patients from tuberculosis endemic countries (k=0.128). CONCLUSION: The concordance between PT and IGRA is affected in patients with EIMI, and to a greater extent to patients with the inflammatory bowel disease, with the corticotherapy, with the BCG vaccination, or in the ones from endemic countries

Red española de registros de enfermedades raras para la investigación: primeros resultados del proyecto Spain-RDR en Navarra / Spanish rare disease registries research network: first results of Spain-RDR's project in Navarre

Fundamento: La Red Española de Registros de Enfermedades Raras para la Investigación, Spain-RDR, fue un proyecto del Instituto de Salud Carlos III (2012-2015) en el que participaron todas las Comunidades Autónomas. Se presentan los primeros resultados de Navarra. Material y métodos: Se explotó el Conjunto Mínimo Básico de Datos de 2010-2011 para valorar la recogida de posibles casos de enfermedades raras en Navarra (estudio piloto) y después se amplió la información, tanto en tiempo (año 2012) como en fuentes de captación (Estadística de Mortalidad y Registro de Incapacidad Temporal). Resultados: En el estudio piloto, Navarra identificó 9.420 posibles casos de 8.141 residentes, pasando a 13.494 casos de 11.644 personas con la ampliación temporal y de fuentes. El 38% de los casos correspondió a enfermedades endocrinas, metabólicas e inmunes, y anomalías congénitas. Conclusiones: Es necesario ampliar las fuentes y el período de captación, así como validar los casos captados para conocer la magnitud real del problema en su conjunto y de cada enfermedad específica incluida en el registro

Background: The Spanish Rare Disease Registries Research Network, Spain-RDR, was a project of the Carlos III Health Institute (2012-2015) in which all the Autonomous Communities participated. The initial results for Navarre are presented. Methods: The Minimum Basic Data Set for 2010-2011 was explored to assess the collection of possible cases of rare diseases in Navarre (pilot study). The information was later extended in both time (the year 2012) and sources consulted (Mortality Statistics and Temporary Disability Registry). Results: Navarre identified 9,420 possible cases amongst the 8,141 residents in the pilot study, reaching 13,494 cases amongst the 11,644 people obtained with the extension of time and sources. Thirty-eight percent of the cases corresponded to endocrine, metabolic and immune diseases, and congenital anomalies. Conclusions: It is necessary to expand the sources and the period of data collection, as well as to validate the cases registered in order to know the real magnitude of the problem as a whole and for each specific disease included in the registry

Inhibidores de la vía de señalización JAK-STAT en el tratamiento de las enfermedades inmunomediadas / JAK-STAT inhibitors for the treatment of immunomediated diseases

La ruta de señalización de las proteínas de la familia Janus cinasa (JAK) está implicada en la patogenia de muchas enfermedades inflamatorias y autoinmunitarias, como la artritis reumatoide (AR), la psoriasis y la enfermedad inflamatoria intestinal. Una gran cantidad de citocinas implicadas en el desarrollo de estas enfermedades utilizan esta vía de señalización para transducir señales intracelulares. En los últimos años, la aparición de los inhibidores de las proteínas JAK (jakinibs) ha demostrado que los fármacos relacionados con esta ruta patogénica pueden tener gran aplicabilidad clínica. Tofacitinib y baricitinib, primeros jakinibs aprobados para el tratamiento de la AR, están en estudio para el tratamiento de otras enfermedades autoinmunitarias. Asimismo, otros jakinibs se encuentran en diferentes fases de desarrollo. En este trabajo se revisan los principales aspectos en cuanto a eficacia, interacciones farmacológicas y seguridad tanto de los jakinibs clásicos como de los de nueva generación

The Janus kinase (JAK) pathway is implicated in the pathogenesis of many inflammatory and autoimmune diseases including rheumatoid arthritis (RA), psoriasis, and inflammatory bowel disease. There are a lot of proinflammatory cytokines involved in such diseases using this pathway to transduce intracellular signals. In the last years, JAK inhibitors (jakinibs) have appeared with a great success, showing that these kinds of drugs have a great applicability in clinical practice. Tofacitinib and baricitinib, the first jakinibs approved for the treatment of RA, are being investigated also for treating other autoimmune systemic diseases. Likewise, other jakinibs are in several phases of development. This review analyses the safety and clinical efficacy of the jakinibs, starting with the classics and continuing with next-generation jakinibs

Leucoencefalopatía multifocal progresiva y disfunción inmune no especificada / Progressive multifocal leukoencephalopathy and non-specific immune dysfunction

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Features and roles of T helper 9 cells and interleukin 9 in immunological diseases

T helper 9 (TH9) cells are considered as newly classified helper T cells that have an important role in the regulation of immune responses. Since these cells preferentially produce IL-9, these cells are termed TH9 cells. Recently, the role of TH9 and its signature cytokine (IL-9) has been investigated in a wide range of diseases, including autoimmunity, allergy, infections, cancer and immunodeficiency. Herein, we review the most recent data concerning TH9 cells and IL-9 as well as their roles in disease. These insights suggest that TH9 cells are a future target for therapeutic intervention

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XVII Curso Internacional Inmunonutrición en la Salud y el Bienestar / No disponible

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Tiempos de pseudoInmunología / No disponible

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