Aeroallergen immunotherapy associated with reduced risk of severe COVID-19 in 1095 allergic patients.

Introduction: Allergen immunotherapy (AIT) brings along changes in the immune system, restoring dendritic cell function, reducing T2 inflammation and augmenting the regulatory cell activation. Coronavirus disease (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections, interferes with the immune system causing immune suppression during the first phase and over-activation in more advanced disease. We decided to explore the interaction of both in a real-world observational trial. Methods: We registered COVID-19 outcomes in patients with allergic disorders in Latin America, treated with and without AIT. The registry was conducted during the first 1.3 years of the pandemic, with most of the data collected before COVID-19 vaccination was concluded in most countries. Data collection was anonymous via a web-based instrument. Ten countries participated. Results: 630/1095 (57.6%) of the included patients received AIT. Compared to patients without AIT, those treated with AIT had a reduced risk ratio (RR) for COVID-19 lower respiratory symptoms (RR 0.78, 95% CI: 0.6703-0.9024; p = 0.001662) and need for oxygen therapy (RR 0.65, 95% CI: 0.4217-0.9992; p = 0.048). In adherent patients on maintenance sublingual immunotherapy/subcutaneous immunotherapy (SLIT/SCIT) the RR reduction was larger [RR = 0.6136 (95% CI 0.4623-0.8143; p < 0.001) and RR: 0.3495 (95% CI 0.1822-0.6701; p < 0.005), respectively]. SLIT was slightly more effective (NS). We excluded age, comorbidities, level of health care attendance, and type of allergic disorder as confounders, although asthma was related to a higher frequency of severe disease. When analyzing patients with allergic asthma (n = 503) the RR reduction favoring AIT was more pronounced with 30% for lower respiratory symptoms or worse (RR 0.6914, 95% CI 0.5264 to 0.9081, p = 0.0087) and 51% for need of oxygen therapy or worse (RR 0.4868, 95% CI 0.2829-0.8376, p = 0.0082). Among severe allergic patients treated with biologics (n = 24) only 2/24 needed oxygen therapy. There were no critical cases among them. Conclusion: In our registry AIT was associated with reduced COVID-19 severity.

Compromising between European and US allergen immunotherapy schools: Discussions from GUIMIT, the Mexican immunotherapy guidelines.

BACKGROUND: Allergen immunotherapy (AIT) has a longstanding history and still remains the only disease-changing treatment for allergic rhinitis and asthma. Over the years 2 different schools have developed their strategies: the United States (US) and the European. Allergen extracts available in these regions are adapted to local practice. In other parts of the world, extracts from both regions and local ones are commercialized, as in Mexico. Here, local experts developed a national AIT guideline (GUIMIT 2019) searching for compromises between both schools. METHODS: Using ADAPTE methodology for transculturizing guidelines and AGREE-II for evaluating guideline quality, GUIMIT selected 3 high-quality Main Reference Guidelines (MRGs): the European Academy of Allergy, Asthma and Immunology (EAACI) guideines, the S2k guideline of various German-speaking medical societies (2014), and the US Practice Parameters on Allergen Immunotherapy 2011. We formulated clinical questions and based responses on the fused evidence available in the MRGs, combined with local possibilities, patient's preference, and costs. We came across several issues on which the MRGs disagreed. These are presented here along with arguments of GUIMIT members to resolve them. GUIMIT (for a complete English version, Supplementary data) concluded the following. RESULTS: Related to the diagnosis of IgE-mediated respiratory allergy, apart from skin prick testing complementary tests (challenges, in vitro testing and molecular such as species-specific allergens) might be useful in selected cases to inform AIT composition. AIT is indicated in allergic rhinitis and suggested in allergic asthma (once controlled) and IgE-mediated atopic dermatitis. Concerning the correct subcutaneous AIT dose for compounding vials according to the US school: dosing tables and formula are given; up to 4 non-related allergens can be mixed, refraining from mixing high with low protease extracts. When using European extracts: the manufacturer's indications should be followed; in multi-allergic patients 2 simultaneous injections can be given (100% consensus); mixing is discouraged. In Mexico only allergoid tablets are available; based on doses used in all sublingual immunotherapy (SLIT) publications referenced in MRGs, GUIMIT suggests a probable effective dose related to subcutaneous immunotherapy (SCIT) might be: 50-200% of the monthly SCIT dose given daily, maximum mixing 4 allergens. Also, a table with practical suggestions on non-evidence-existing issues, developed with a simplified Delphi method, is added. Finally, dissemination and implementation of guidelines is briefly discussed, explaining how we used online tools for this in Mexico. CONCLUSIONS: Countries where European and American AIT extracts are available should adjust AIT according to which school is followed.

[MASK (Mobile Airways Sentinel Network), a mobile App with ARIA's comprehensive solution in Spanish-speaking countries]. / MASK (Mobile Airways Sentinel Network), una app móvil con la solución integral de ARIA en países de habla hispana.

Although there are high quality clinical guidelines about allergic rhinitis, many patients receive deficient treatment, partly due to the high level of self-medication. MASK (Mobile Airways Sentinel Network) is an integral part of a project against chronic diseases which it is focused on active and healthy aging and is supported by the European Union. It forms the third phase of ARIA (Allergic Rhinitis and its Impact on Asthma) in which, through a mobile app on a smart device, the purpose is to guide patients in the control of their multimorbidity, allergic rhinitis or conjunctivitis, or asthma. The "Allergy Diary" app by MACVIA-ARIA is free and it is available for Android and iOS; on it, patients indicate how unpleasant the symptoms are on a daily basis through five screens with an analogous visual scale; two more screens were recently added (sleep affectation). With the app, it is also possible to download the information of the "Allergy Diary" on the physician's computer through a QR code at the moment of the medical consultation. In this article, we review the first year of experience in Spain, Mexico and Argentina, where the Spanish version is used.

Aunque existen guías clínicas de alta calidad sobre rinitis alérgica, numerosos pacientes reciben tratamiento deficiente, en parte debido al alto grado de automedicación. MASK (Mobile Airways Sentinel Network) forma parte integral de un proyecto apoyado por la Unión Europea contra las enfermedades crónicas y enfocado al envejecimiento activo y saludable. Constituye la tercera fase de ARIA (Allergic Rhinitis and its Impact on Asthma), en la cual mediante una aplicación móvil en un dispositivo inteligente se intenta guiar al paciente en el control de su multimorbilidad, rinitis o conjuntivitis alérgicas o asma. La aplicación Diario de Alergia por MACVIA-ARIA es gratuita y está disponible para Android e iOS; en ella, los pacientes indican diariamente cuánto les molestan los síntomas a través de cinco pantallas con una escala visual análoga; recientemente se agregaron dos pantallas más (afectación del sueño). La aplicación también permite descargar los datos del "Diario de alergias" en la computadora del médico en el momento de la consulta a través de un código QR. En este artículo reseñamos el primer año de experiencia en España, México y Argentina, que utilizan la versión española.

[GUIMIT 2019, Mexican Guideline on Immunotherapy. Guideline on the diagnosis of IgE-mediated allergic disease and immunotherapy following the ADAPTE approach]. / GUIMIT 2019, Guía Mexicana de Inmunoterapia. Guía de diagnóstico de alergia mediada por IgE e inmunoterapia aplicando el método ADAPTE.

BACKGROUND: In Mexico, allergen immunotherapy (AIT) and immunotherapy with hymenoptera venom (VIT) is traditionally practiced combining aspects of the European and American school. In addition, both types of extracts (European and American) are commercially available in Mexico. Moreover, for an adequate AIT/VIT a timely diagnosis is crucial. Therefore, there is a need for a widely accepted, up-to-date national immunotherapy guideline that covers diagnostic issues, indications, dosage, mechanisms, adverse effects and future expectations of AIT (GUIMIT 2019). METHOD: With nationwide groups of allergists participating, including delegates from postgraduate training-programs in Allergy/Immunology-forming, the guideline document was developed according to the ADAPTE methodology: the immunotherapy guidelines from European Academy of Allergy and Clinical Immunology, German Society for Allergology and Clinical Immunology, The American Academy of Allergy, Asthma and Immunology and American College of Allergy, Asthma, and Immunology were selected as mother guidelines, as they received the highest AGREE-II score among international guidelines available; their evidence conforms the scientific basis for this document. RESULTS: GUIMIT emanates strong or weak (suggestions) recommendations about practical issues directly related to in vivo or in vitro diagnosis of IgE mediated allergic diseases and the preparation and application of AIT/VIT and its adverse effects. GUIMIT finishes with a perspective on AIT modalities for the future. All the statements were discussed and voted on until > 80 % consensus was reached. CONCLUSIONS: A wide and diverse group of AIT/VIT experts issued transculturized, evidence-based recommendations and reached consensus that might improve and standardize AIT practice in Mexico.

Antecedentes: En México, la inmunoterapia con alérgenos (ITA) y con veneno de himenópteros (VIT) se practica tradicionalmente combinando criterios de las escuelas europea y estadounidense; los dos tipos de extractos están comercialmente disponibles en México. Para una ITA adecuada es crucial un diagnóstico oportuno. Objetivo: Presentar GUIMIT 2019, Guía Mexicana de Inmunoterapia 2019, de base amplia, actualizada, que abarca temas de diagnóstico, indicaciones, dosificación, mecanismos, efectos adversos de la ITA y expectativas con esta modalidad de tratamiento. Método: Con la participación de múltiples grupos mexicanos de alergólogos, que incluían los centros formadores universitarios en alergia e inmunología, se desarrolló el documento de la guía según la metodología ADAPTE. Las guías de inmunoterapia de la European Academy of Allergy and Clinical Immunology, The American Academy of Allergy, Asthma and Immunology, German Society for Allergology and Clinical Immunology y del American College of Allergy, Asthma, and Immunology se seleccionaron como guías fuente, ya que recibieron la puntuación AGREE-II más alta entre las guías internacionales disponibles; su evidencia conforma la base científica de GUIMIT 2019. Resultados: En GUIMIT 2019 se emiten recomendaciones fuertes o débiles (sugerencias) acerca de temas directamente relacionados con el diagnóstico in vivo o in vitro de las enfermedades alérgicas mediadas por IgE, la preparación y aplicación de ITA o VIT y sus efectos adversos; se incluye la revisión de las modalidades de ITA para el futuro. Todos los argumentos que se exponen fueron discutidos y votados con > 80 % de aprobación. Conclusión: Un grupo amplio y diverso de expertos en ITA y VIT emitió recomendaciones transculturizadas basadas en evidencia, que alcanzaron consenso; con ellas se pretende mejorar y homologar la práctica de la inmunoterapia en México.

[MASK (Mobile Airways Sentinel Network). ARIA's comprehensive solution for mobile app for the multimorbidity of allergic rhinitis and asthma]. / MASK (Mobile Airways Sentinel Network). La solución integral de ARIA por app móvil para la multimorbilidad de rinitis alérgica y asma.

The vast majority of patients with allergic rhinitis (AR) do not receive the proper management which is recommended by the guidelines, but they frequently self-medicate. MASK (Mobile Airways Sentinel Network) is an integral part of a project that is supported by the European Union against chronic diseases and focused on active and healthy aging. MASK represents the third phase of ARIA (Allergic Rhinitis and its Impact on Asthma), in which, by using a mobile application in a smart device, the objective is to guide the patient in the control of his/her multi-morbidity, AR and/or allergic conjunctivitis (AC) and/or asthma. The mobile app Allergy Diary by MACVIA-ARIA is free and it is available for both Android and iOS platforms. After it is downloaded to the patient's cell phone, it first requests some information about the patient's profile, allergic pathologies and medication; afterwards, through a visual analog scale, the patient is invited to determine the degree of affectation in the nose, eyes, and bronchi, and its influence on their productivity at work / school. After analyzing the data generated by filling the Allergy Diary, it became clear there is a new clinical entity: allergic rhinitis+ allergic conjunctivitis +asthma, with greater effect; in addition to a high level of self-medication: in general, the patient takes medication on days when symptoms are present. The app has already been deployed in 23 countries, including several Spanish-speaking countries.

La mayoría de los pacientes con rinitis alérgica no recibe el manejo idóneo, sino que se automedica. MASK (Mobile Airways Sentinel Network) forma parte integral de un proyecto apoyado por la Unión Europea contra las enfermedades crónicas y enfocado al envejecimiento activo y saludable. Constituye la tercera fase de ARIA (Allergic Rhinitis and its Impact on Asthma), en la cual mediante una aplicación móvil en un dispositivo inteligente se intenta guiar al paciente en el control de su multimorbilidad, rinitis o conjuntivitis alérgicas o asma. La aplicación Diario de Alergia por MACVIA-ARIA es gratuita y está disponible para Android e iOS. Al descargarla al celular del paciente, a este se le piden datos de su perfil, patologías alérgicas y medicación; posteriormente, mediante una escala visual analógica se le invita a determinar el grado de afectación en nariz, ojos y bronquios y su influencia sobre su productividad laboral/escolar. Con los datos del Diario de Alergia se observa que existe un nuevo patrón de presentación: rinitis alérgica + conjuntivitis alérgica + asma, con mayor afectación, así como un alto nivel de automedicación: en general, el paciente toma medicación cuando presenta síntomas. La app se ha desplegado en 23 países, incluyendo varios países hispanohablantes.

[Executive Summary of ARIA 2019: Integrated care pathways for allergic rhinitis in Argentina, Spain and Mexico]. / Resumen ejecutivo de ARIA 2019: Vías integradas de atención para la rinitis alérgica en Argentina, España y México.

The health and economic impact of allergic diseases are increasing rapidly, and changes in management strategies are required. Its influence reduces the capacity of work and school performance by at least a third. The ICPs of the airways (integrated care pathways for respiratory diseases) are structured multidisciplinary healthcare plans, promoting the recommendations of the guidelines in local protocols and their application to clinical practice. This document presents an executive summary for Argentina, Mexico, and Spain. Next-generation ARIA guidelines are being developed for the pharmacological treatment of allergic rhinitis (AR), using the GRADE-based guidelines for AR, tested with real-life evidence provided by mobile technology with visual analogue scales. It is concluded that in the AR treatment, H1-antihistamines are less effective than intranasal corticosteroids (INCS), in severe AR the INCS represent the first line of treatment, and intranasal combination INCS + anti-H1 is more effective than monotherapy. However, according to the MASK real-life observational study, patients have poor adherence to treatment and often self-medicate, according to their needs.

El impacto sanitario y económico de las enfermedades alérgicas está aumentando rápidamente y se necesitan cambios en las estrategias para su manejo. Su influencia reduce al menos en un tercio la capacidad de desempeño laboral y escolar. Los ICP (Vías Integradas de Atención) de las enfermedades de las vías respiratorias son planes de atención estructurados y multidisciplinarios, que promueven las recomendaciones de las guías en protocolos locales y su aplicación a la práctica clínica. En este documento se presenta un resumen ejecutivo para Argentina, México y España. Se desarrollan las guías ARIA de próxima generación para el tratamiento farmacológico de la rinitis alérgica (RA) utilizando las pautas basadas en GRADE para RA, probadas con evidencia de la vida real proporcionada por tecnología móvil basada en escalas visuales analógicas. Se concluye que en el tratamiento de la RA, los antihistamínicos anti-H1 son menos efectivos que los corticoides intranasales (CINS), que en la rinitis gravelos CINS representan la primera línea de tratamiento, y que la combinación intranasal de CINS + anti-H1 es más eficaz que la monoterapia. Sin embargo, según el estudio MASK observacional en vida real, los pacientes tienen pobre adherencia al tratamiento y frecuentemente se automedican de acuerdo con sus necesidades.

[Clinical and therapeutic features in patients with common variable immunodeficiency from two third-level care hospitals from Mexico City]. / Características clínicas y terapéuticas de pacientes con inmunodeficiencia com˙n variable en dos hospitales de tercer nivel de atención de la Ciudad de México.

BACKGROUND: Common variable immunodeficiency (CVID) is one of the most common antibody deficiencies, is characterized by low serum immunoglobulins, defective antibody response and increased susceptibility to chronic and recurrent infections. OBJECTIVE: we present the clinical findings of patients with CVID in two hospitals in Mexico City. METHODS: We performed a retrospective study of patients who filled CVID criteria. We collected the following information, demographic data, age at onset, age at diagnosis, family history, infection, autoimmunity, lymphoproliferative disease, allergy, malignancy, immunoglobulin levels at diagnosis, route of administration, dosage and frequency of IVIG of each patient. Data were analyzed with descriptive statistics. RESULTS: Amongst 26 patients who filled CVID criteria, 14 were men and 12 women. The mean diagnosis delay was 48 months (22-128), serum immunoglobulins at diagnosis in mg/dL were IgG 216 (114-316), IgM 21 (12-121), IgA 21 (6-26) and IgE 4.6 (1.8) IU/mL. 81% of patients suffered pneumonia. There was a decrease in the number of pneumonias before and after treatment with gammaglobulin (p = 0.028). 27% of the patients had autoimmune diseases, 35% allergies, 35% chronic diarrhea, 62% bronchiectasis, 73% chronic cough, 50% lymphadenopathy. One patient had lymphoproliferative disease and none developed malignancy. CONCLUSIONS: We found that the delay in the diagnosis and initiation of gammaglobulin replacement affects the occurrence of complications such as bronchiectasis.

[Validation of the PRIDE Scale (Primary Immunodeficiency Disease Evaluation) to Determine High Level of Suspicion for Primary Immunodeficiency Disease in Mexican Population]. / Validación de la escala PRIDE (Primary Immunodeficiency Disease Evaluation) para determinar nivel alto de sospecha de inmunodeficiencia primaria en población mexicana.

BACKGROUND: Primary immunodeficiencies have a low prevalence, but their severity and high risk of mortality require a clinical approach based in diagnostic instruments that allow their early detection. OBJECTIVE: To validate the PRIDE scale to determine a high level of suspicion for primary immunodeficiency diseases in Mexican population. METHODS: Seventy five patients with suspicion of primary immunodeficiencies (13.9 ±11.8 years-old) were evaluated with the PRIDE scale by specialists in Allergy and Immunology in this cross-sectional study, and their results were compared with the confirmed diagnosis of primary immunodeficiency. RESULTS: Kappa concordance coefficient with clinical diagnosis was 0.872, with an standard error of 0.072; CI 95% [0.73-1.013]. The scale showed sensitivity 98.3%, specificity 86.6%, positive predictive value 96.7%, negative predictive value 92.8%, positive odds ratio 7.3358 and negative odds ratio 0.0196. CONCLUSIONS: PRIDE scale was concordant with the diagnosis of primary immunodeficiency in the majority of patients with a previous confirmed diagnosis. The characteristics of diagnostic accuracy provide reliability to this instrument in the scrutiny of patients with probable immunodeficiency.

[Neumopathies in patients with primary immunodeficiencies in treatment with intravenous gammaglobulin]. / Neumopatías en pacientes con inmunodeficiencias primarias en tratamiento con gammaglobulina intravenosa.

BACKGROUND: The primary immunodeficiency diseases cause a deficit in the production of antibodies. The chronic sinopulmonary disease is common and their clinic symptoms are diverse (pneumonia, bronchiectasis). OBJECTIVE: To know the frequency and type of pulmonary abnormalities in patients with primary immunodeficiency in treatment with intravenous immunoglobulin. MATERIAL AND METHODS: 24 files of patients with primary immunodeficiency were selected. Age, sex, primary immunodeficiency type, time of immunoglobulin treatment, chest X-ray finding, pulmonary computed tomography of high resolution (HRCT) and pulmonary function tests were registered. Measures of central tendency were calculated. RESULTS: There was no predominance of gender; the average age was 14 years old. The common variable immunodeficiency and the Bruton's hypogammaglobulinemia represented 91% of the patients. The X-ray of thorax was abnormal in 33%, although there were not bronchiectasis. The high-resolution computed tomography scan (HRCT) was abnormal in 67%, and 75% had bronchiectasis. CONCLUSIONS: The pulmonary complications are common despite therapy with intravenous immunoglobulin. The HRCT is better than X-ray in these patients.

[Diminished apoptosis in nasal mucosa of patients with persistent allergic rhinitis]. / Disminución de la apoptosis en la mucosa nasal de pacientes con rinitis alérgica persistente.

OBJECTIVE: To assess apoptosis frequency in the nasal mucosa of patients with perennial allergic rhinitis. PATIENTS AND METHODS: Eleven patients and five healthy subjects accepted to participate, from 10 to 16 years old. None of them had evidence of acute infectious disease, tobacco exposure or use of corticosteroids, antileukotrienes, antihistamines or immunotherapy. After a clinical evaluation and a skin prick testing, a nasal symptoms questionnaire was applied and a nasal washing was performed. Two hundred cells were examined (Giemsa and eosin yellowish) and apoptosis was identified by immunohistochemistry (Active-Caspase-3) (p20L18, sc-1225. Santa Cruz Biothecnology, Inc. Santa Cruz). RESULTS: The most common allergen was Dermatophagoides sp in 10 patients (91%). The total score of the nasal symptom questionnaire was 3 to 8 (median 6) in patients and 1 to 4 (median 2) in healthy subjects (p < 0.05, Mann Whitney U test). Frequency of eosinophils was 11 to 80% in patients and 0 to 1% in healthy subjects (p < 0.05, Z for proportions). Apoptosis was less frequent in patients (0 to 5, median 2) than in healthy subjects (4 to 16, median 8) (p < 0.01, Mann Whitney U test). CONCLUSIONS: Perennial allergic rhinitis may be associated with decreased apoptosis in the nasal mucosa.

[Evaluation of a questionnaire for measuring nasal symptoms in subjects with allergic rhinitis]. / Evaluación de un cuestionario para la medición de síntomas nasales en sujetos con rinitis alérgica.

BACKGROUND: For the assessment of nasal symptoms in allergic rhinitis patients, most of the questionnaires evaluate the impact of symptoms on patient's quality life. OBJECTIVE: To assess the consistent and reliability of a nasal symptom questionnaire in patients with allergic rhinitis. MATERIAL AND METHODS: 224 patients (10 to 19 years old) accepted to participate in the study. All the patients completed a questionnaire, which evaluates the severity and frequency of four nasal symptoms using a maximum total score of 12 points. The consistent was studied after the questionnaire was administered to 62 patients with allergic rhinitis and 62 patients without it. The reliability after one to four weeks was studied on 100 healthy subjects with the Bland and Altman's method. RESULTS: The total score of patients with allergic rhinitis was significantly different to the score of the patients without it (7.1 +/- 2 and 1.9 +/- 1, respectively). The alpha coefficient for the total score was 0.86. The repeated measures showed a repeatability of 96% with a repeatability coefficient of 2. CONCLUSION: The nasal symptom questionnaire is a reliable tool for the follow up of allergic rhinitis during periods of one to four weeks.

Prevalencia de hernia hiatal y esofagitis crónica en pacientes asmáticos adultos / Prevalence of hiatus hernia and chronic esophagitis in adult asthmatic patients

Antecedentes: la concomitancia entre asma, hernia hiatal, esofagitis y reflujo gastroesofágico se ha reportado como factor causal que altera la evolución clínica del asma. Material y método: estudio prospectivo, longitudinal y comparativo efectuado con un grupo de 90 pacientes con asma leve y moderada persistente, de origen alérgico, con una edad promedio de 35.9 años. Al cabo de seis meses de tratamiento inmunoalérgico se valoró la respuesta clínica que se clasificó en dos grupos: el grupo A lo integraron 40 pacientes con asma leve intermitente y el B 50 individuos con asma moderada severa persistente. A los dos grupos se les realizó esofagogastroscopía para poder establecer el diagnóstico de la afección esofágica, con biopsia en los tres tercios esofágicos. Se registraron los síntomas de reflujo gastroesofágico. Resultados: se encontró hernia hiatal en 33 pacientes, 12 del grupo A y 21 del B. Esofagitis proximal en 21 pacientes, 8 del grupo A y 13 del B. Síntomas de reflujo gastroesofágico en 71 pacientes, 29 del grupo A y 42 del B. La asociación de hernia hiatal-esofagitis-reflujo gastroesofágico se observó en 12 pacientes, dos del grupo A y 10 del B (p< 0.05). Conclusiones: la asociación de síntomas de reflujo gastroesofágico, hernia hiatal y esofagitis proximal es mayor en los pacientes con mala respuesta al tratamiento antiasmático. En el grupo de pacientes con mala respuesta y síntomas de reflujo gastroesofágico se justifica la realización de la endoscopía y la toma de biopsias del esófago proximal cuando no se cuenta con pH-metría de 24 horas.

Síndrome de adhesión leucocitaria deficiente: informe de un caso / Leukocyte adhesion deficiency syndrome. A case report

Introducción. El síndrome de adhesión leucocitaria deficiente (ALD) es una alteración de la fagocitosis caracterizada por la deficiencia de una o varias intregrinas a la superfamilia de receptores de la superficie celular y de moléculas de adhesión. Objetivo. Describir las características clínicas de una inmunodeficiencia primaria de prevalencia rara. Caso clínico. Recién nacido masculino de 19 días de vida extrauterina referido al servicio de infectología pediátrica por un cuadro clínico de 15 días de evolución caracterizado por caída tardía del cordón umbilical, fiebre y lesiones dérmicas pápuloeritematosas en el área periumbilical, hipogástrica, inguinal, glútea y en el muslo derecho, misma que progresaron a celulitis y necrosis tisular. Ingresó al hospital con diagnóstico de septicemia secundaria a onfalitis. Hubo respuesta parcial al menejo inicial con antimicrobianos. Posteriormente tuvo infecciones bacteriana y micóticas recurrentes de las vías respiratorias y el tracto gastrointestinal. Tuvo retardo psicomotriz y deterioro severo de su estado nutricional. Falleció por septicemia a los cinco meses de edad. De los exámenes de laboratorio, la biometría hemática mostró leucocitosis persistente entre 42,000 y 133,00 células/mm3, con predominio de neutrófilos, con variaciones entre 64 y 68 por ciento. Se encontró deficiencia de la quimiotaxis de neutrófilos. Por citometría de flujo se observó deficiencia de las adhesinas CD11/18. El resto de los estudios inmunológicos, biopsia de médula ósea y viral fueron normales. Conclusión. Si bien su prevalencia es rara, los defectos de adhesión leucocitaria deben considerarse en los pacientes con caída tardía de cordón umbilical, infecciones severas recurrentes y neutrofilia muy elevada y persistente en quienes se hayan descartado otras inmunodeficiencias primarias y secundarias

Conceptos actuales de autoinmunidad. Fisiología / Current concepts about autoimmunity. Physiopathology

La enfermedad autoinmune se caracteriza por representar una pérdida de la autotolerancia. Puede ser órgano específica y órgano inespecífica. Existe una interrelación entre elementos genéticos, ambientales e infecciosos, entre otros, para su génesis. La muerte celular programada o apoptosis está baja o incrementada en diversas entidades clínicas