Las inmunodeficiencias primarias (IDP) son enfermedades de baja prevalencia. Existen signos de alarma para su sospecha clínica. Los objetivos fueron describir características clínicas y signos de alarma de pacientes con IDP y comparar diferencias clínicas entre déficit selectivo de inmunoglobulina A (IgA) y otras IDP. Se estudiaron 89 pacientes, con mediana de edad, al momento del diagnóstico, de 6 años (4,08-11,67). 53 (59,5%) pacientes, varones. 54 (60,7%) pacientes, con déficit selectivo de IgA, y 35 (39,3%), con otras IDP. Las principales manifestaciones clínicas fueron rinofaringitis en 65 (73,03%) pacientes y atopia en 39 (43,82%) pacientes. Presentaron signos de alarma 24 (26,97%) pacientes, ninguno con déficit selectivo de IgA. En los pacientes con otras IDP, se observó mayor incidencia de infección respiratoria baja, sepsis, infecciones cutáneas, candidiasis mucocutánea, alteraciones dentales, malformaciones cardiovasculares, angioedema, internaciones y mortalidad. 10 (28,57%) pacientes recibieron gammaglobulina endovenosa; 15 (42,85%), antibiótico-profilaxis; y 2 (2,24%), antimicóticoprofilaxis.
Primary immunodeficiencies (PID) are low-prevalence diseases. There are warning signs that may raise clinical suspicion. The objectives of this study were to describe the clinical characteristics and warning signs of patients with PID and to compare the clinical differences between selective immunoglobulin A (IgA) deficiency and other PIDs. Eighty-nine patients were studied; their median age at the time of diagnosis was 6 years old (4.08-11.67). Fifty-three (59.5%) patients were male. Fifty-four (60.7%) patients had selective IgA deficiency, and 35 (39.3%) had other PIDs. The main clinical manifestations were rhinopharyngitis in 65 (73.03%) patients and atopy in 39 (43.82%). Twenty- four (26.97%) patients showed warning signs, and none had selective IgA deficiency. Patients with other PIDs had a higher incidence of lower respiratory tract infection, sepsis, skin infections, mucocutaneous candidiasis, dental alterations, cardiovascular malformations, angioedema, hospitalizations and death. Ten (28.57%) patients received intravenous gammaglobulin, 15 (42.85%) antibiotic prophylaxis, and 2 (2.24%) antifungal prophylaxis.
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