Source: Annual Congress 2001 - Immune deficiencies and the lung in childhood
Disease area: Respiratory infections

Abstract

Background: Primary Humeral ImmunoDeficiencies(PHID) are nowadays increasingly recognized. Patients with PHID frequently show respiratory complications, which are often the first manifestation of disease and the major cause of morbidity and mortality. Objectives: To determine the clinical spectrum of respiratory diseases in children with PHID. Method: We extracted data from the clinical files of patients with PHID, according to WHO criteria, who were enrolled in Iranian Primary Immunodeficiency Registry. Results: We analyzed 135 patients (91 males,44females) with the diagnoses of common-variable immunodeficiency (72pts), x-linked agammaglubolinemia (29pts), IgA deficiency (22pts), IgG-subclass deficiency (9pts), and hyper-IgM syndrome (3pts). Their mean age at the time of study was 11 years. Respiratory diseases were the first presentation in 88 patients (65%). In the evolution of their disease, 110 cases (81%) had upper respiratory tract infections, among which acute otitis media (73pts,54%), Sinusitis (64pts,47%), and pharyngitis (9pts,7%) are the more frequent. Among the lower respiratory tract infections, pneumonia was the most common (95pts,70%). The other complications included bronchiectasis (24pts,18%), bronchitis (7pts), tuberculosis (6pts), pneumocystis carinii pneumonia (5pts), and lung abscess (2pts). Conclusion: Respiratory infections constitute the most common presenting symptom. There maybe some differences in the type and frequency of infections in each of these disorders.

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A. Aghamohammadi, M. Moein, Z. Pourpak, K. Abolmaali, N. Rezaei, L. Amiri Kordestani, Z. Habibi (Tehran, Iran). The clinical spectrum of respiratory diseases in patients with primary humeoral immunodeficiency disorders; a clinical survey of patients from the Iranian primary immunodeficiency registry. Eur Respir J 2001; 16: Suppl. 31, 3430

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