Source: Annual Congress 2006 - Pneumonia and other invasive pulmonary infections in children
Disease area: Paediatric lung diseases

Abstract

To determinate the outcome of pulmonary disease in pts with primary humoral immunodeficiencies we evaluated prospectively (follow-up of 3-15 yrs) the clinical records, chest High Resolution Computed Tomography (HRCT) and pulmonary function tests (PFT) in 7 pts with X-linked agammaglobulinemia (XLA) and in 3 pts with Common Variable Immunodeficiency (CVID).The mean age at the diagnosis was 4.8 yrs (0.7- 15 yrs) for XLA pts and 8.5 yrs (3.6-16.5 yrs) for CVID pts. After diagnosis the pts received IVIG replacement therapy every 3-4 wks, chest physiotherapy with PEP-Mask and antibiotic therapy during acute respiratory infections. In XLA group the mean number of months/yrs with productive cough (cough index) was 7.1 at the beginning of the study and 5.4 at the end of the follow-up; in the CVID group the cough index was 5.6 and 2.3 respectively. Before diagnosis the 57% of the XLA pts and the 67% of the CVID pts were suffering from recurrent pneumonia while during the follow-up only one XLA pt had a documented pneumonia.The mean HRCT general score,evaluated using Bhalla‘s system, was 8.1 at the beginning and at the end of the study in XLA pts and 1.6 at the beginning and 4.5 at the end in CVID pts. PFT showed a moderate obstruction of the small airways in 4 pts at the beginning, but in 3 of these pts PFT values were normal at the end of follow-up. The Bhalla‘s score was stable in 5 pts, improved in 3 and worsened in one XLA with severe pulmonary disease at the diagnosis and, unespectedly, in one CVID with a good clinical outcome. HRCT is the method of choice in monitoring pulmonary changes.Systematic treatment seams to be effective fo a favourable outcome.

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Citations should be made in the following way:
M. F. Patria, N. Liotto, R. M. Dellepiane, A. Lopopolo, M. C. Pietrogrande (Milan, Italy). Outcome of pulmonary disease in patients with primary humoral immunodeficiencies. Eur Respir J 2006; 28: Suppl. 50, 1792

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