Spanish: Primary Immunodeficiency Disease

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</div><h3></h3><img alt="Enfermedades de Inmunodeficiencia Primaria" src="/Aaaai/media/MediaLibraryRedesign/Conditions%20Treatments/Afecciones%20y%20Tratamientos/86537295-Football-cropped.jpeg" /> <p>Las enfermedades de inmunodeficiencia ocurren cuando parte del sistema inmunitario est谩 ausente o no est谩 funcionando de manera adecuada. Cuando la causa de esta inmunodeficiencia es hereditaria o gen茅tica, se habla de enfermedades de inmunodeficiencia primaria (IP). Los investigadores han identificado m谩s de 250 clases diferentes de IP.<br /> <br /> El sistema inmunitario est谩 compuesto por diferentes tipos de c茅lulas y prote铆nas que trabajan en conjunto para proteger de los ataques de invasores “extra帽os”, como bacterias, virus y hongos. Los leucocitos se forman en la m茅dula 贸sea y viajan por el torrente sangu铆neo y ganglios linf谩ticos. Los anticuerpos son prote铆nas que nacen en respuesta a la infecci贸n o inmunizaci贸n y ayudan a luchar contra las infecciones. El complemento es otra prote铆na de la sangre que juega un papel de protecci贸n en el sistema inmunitario.   <br /> <br /> En las IP m谩s comunes, diferentes formas de estas c茅lulas o prote铆nas est谩n ausentes o no funcionan adecuadamente. Esto crea un patr贸n de infecciones repetidas, infecciones graves y/o infecciones que son excepcionalmente dif铆ciles de curar. Estas infecciones pueden afectar distintos sistemas de 贸rganos como la piel, el aparato respiratorio y gastrointestinal, los o铆dos, el cerebro, la m茅dula espinal o puede afectar m煤ltiples sistemas. En algunos casos, las IP atacan 贸rganos, gl谩ndulas, c茅lulas y tejidos espec铆ficos y/o m煤ltiples. Por ejemplo, en algunas IP se presentan defectos card铆acos. Otras IP alteran las caracter铆sticas faciales, algunas detienen el crecimiento normal, e incluso otras est谩n relacionadas con trastornos autoinmunitarios, como la artritis reumatoide.  <br /> <br /> <strong>Enfermedad de inmunodeficiencia primaria. S铆ntomas y diagn贸stico</strong><br /> Seg煤n las c茅lulas y las prote铆nas afectadas, las IP se pueden presentar a diferentes edades. Por ejemplo, las inmunodeficiencias graves de linfocitos T o combinadas por lo general se presentan en el primer a帽o de vida. Sin embargo, algunas deficiencias de anticuerpos pueden presentarse en ni帽os mayores o adultos. A menudo se debe establecer un patr贸n de infecciones recurrentes antes de que se sospeche la existencia de IP.<br /> <br /> Estos son algunos de los signos que pueden indicar la existencia de una IP:<br /> • Infecciones recurrentes, inusuales o dif铆ciles de tratar<br /> • Crecimiento deficiente o p茅rdida de peso<br /> • Neumon铆a recurrente, infecciones del o铆do o sinusitis<br /> • Que se requieran m煤ltiples tomas de antibi贸ticos o antibi贸ticos IV para limpiar las infecciones<br /> • Abscesos profundos recurrentes de los 贸rganos o de la piel<br /> • Antecedentes familiares de IP<br /> • Inflamaci贸n de las gl谩ndulas linf谩ticas o hipertrofia del bazo<br /> • Enfermedad autoinmunitaria<br /> <br /> Algunos trastornos de inmunodeficiencia no son primarios (hereditarios o gen茅ticos). Las enfermedades de inmunodeficiencia secundaria se producen cuando el sistema inmunol贸gico se ve comprometido debido a un factor ambiental. Ejemplos de esas causas externas incluyen la infecci贸n (VIH), medicamentos (quimioterapia o esteroides sist茅micos) quemaduras graves o desnutrici贸n.<br /> <br /> <strong>Enfermedad de inmunodeficiencia primaria – Tratamiento y control</strong><br /> La investigaci贸n en materia de inmunodeficiencia primaria est谩 dando grandes avances, mejorando las opciones de tratamientos y mejorando la calidad de vida de la mayor铆a de las personas que padecen de estas complejas afecciones. Las opciones de tratamiento apuntan a defectos inmunitarios espec铆ficos y pueden incluir transplantes (de m茅dula 贸sea, c茅lulas madre, timo), reemplazo de inmunoglobulina (anticuerpo), antibi贸ticos preventivos y estrategias para controlar la enfermedad autoinmunitaria. La terapia de genes puede ser exitosa en tipos espec铆ficos de IP.   <br /> <br /> Si usted o su ni帽o tienen s铆ntomas de estas afecciones a menudo cr铆ticas, necesita el mejor cuidado posible. Un alergista/inmun贸logo o un inmun贸logo cl铆nico tiene la capacitaci贸n especializada y la experiencia para diagnosticar, con precisi贸n, y coordinar un plan de tratamiento para las IP.<br />  </p> <p><em>* Geha R, Notarangelo L, Casanova JL, Conley ME, Chapel ME, Fischer A, Hammerstrom L, Nonoyama S, Ochs H, Puck J, Roifman C, Seger R, Wedgwood J.   Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting.</em></p> <p><em>Journal of Allergy and Clinical Immunology 2007; 120(4):776-794.</em></p> <h3>CURRENT TOPICS</h3> <p><a href="/Conditions-Treatments/Afecciones-y-Tratamientos/Enfermedades-de-Inmunodeficiencia-Primaria/Cuestionario-sobre-Enfermedades-de-Inmunodeficienc">Cuestionario sobre Enfermedades de Inmunodeficiencia Primaria (PIDD) - Primary Immunodeficiency Diseases (PIDD) Quiz »</a></p> <p><a href="/Tools-for-the-Public/Biblioteca-de-condiciones/Biblioteca-de-inmunodeficiencias/Preparacion-para-la-terapia-de-infusion-de-inmunog">Preparaci贸n para la terapia de infusi贸n de inmunoglobulina intravenosa (IVIG) (Preparing for Intravenous Immunoglobulin (IVIG) Infusion Therapy) »</a></p> <p><a href="/Tools-for-the-Public/Biblioteca-de-condiciones/Biblioteca-de-asma/Introduccion-a-las-terapias-biologicas">Introducci贸n a las terapias biol贸gicas (An Introduction to Biologic Therapies) </a><a href="/conditions-and-treatments/library/asthma-library/SP-biologic-therapies">»</a><br /> <br /> <a href="/Tools-for-the-Public/Biblioteca-de-condiciones" target="_blank">Biblioteca de condiciones (Conditions Library)</a></p> <div> <div class="c-social-sharing"> <h4 class="c-social-sharing__heading">Share this page:</h4> <ul class="c-links"> <li class="c-link__item"> <a id="p_lt_ctl03_pageplaceholder_p_lt_ctl02_AAAAI_SocialSharing_faceBook" rel="noreferrer noopener" class="c-social-sharing__link 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