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2016, Number 1

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Acta Pediatr Mex 2016; 37 (1)

Quality of life in patientes with antibody immunodeficiencies

Muriel-Vizcaíno R, Treviño-Garza G, Murata C, Staines-Boone AT, Yamazaki-Nakashimada MA, Espinosa-Padilla SE, Espinosa-Rosales FJ

Language: Spanish
References: 38
Page: 17-25
PDF size: 637.93 Kb.


ABSTRACT

Primary immunodeficiencies (PID) are genetic diseases affecting immunological normal response. In the great majority of cases affected individual sare predisposed to recurrent and / or severe infections with impaired quality of life (QoL). The most frequent PID are defects in the production of antibodies. When diagnosis and treatment are not done properly, permanent damage may occur in affected organs and further affecting QoL.
Objective: To evaluate QoL in patients with antibody PID compared with QoL of healthy controls.
Methods: Cross-sectional study with the application of the instrument "Pediatric Quality of Life Inventory" (PedsQL) in its Mexican Spanish validated version. The PedsQL score difference between children with PID and healthy children was determined by the Student t test. The effect of a delayed in diagnosis was evaluated by analysis in a covariance model. 28 patients were included. The median age was 5 years and 5 months.
Results: The average age at diagnosis was 6 years and 3 months. The median diagnostic delay was 3 years 3 months. The average QoL in the patients was 74.1 (SD ± 13.8) and 83.3 (SD ± 10.1) for controls (p = 0.005). The relationship between QoL, delayed diagnosis and presence of complications was analyzed using a linear model that was marginally significant (p = 0.056).
Negative correlation between levels of IgG in the last year and the quality of life was found. It was observed that in patients with bronchiectasis, QoL decreased significantly as the duration of delayed diagnosis (p = 0.007).
Conclusions. We consider necessary to assess QoL of patients with PID and to monitoring it during treatment in order to have an impact not only in reducing the number of infections, hospitalizations and complications, but also in improving their QoL. It should be designed a QoL measuring instrument specific for patients with PID


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