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Pediatría (Asunción)

On-line version ISSN 1683-9803

Abstract

CABALLERO, Fabiola et al. Antibody deficiencies in children and adolescents with recurrent and/or severe infections. Pediatr. (Asunción) [online]. 2018, vol.45, n.2, pp.141-146. ISSN 1683-9803.  https://doi.org/10.31698/ped.45022018006.

Introduction:

Predominantly antibody deficiencies (PAD) are primary immunodeficiencies characterized by hypogammaglobulinaemia, a decrease of IgA, IgG and/or IgM serum levels, which predispose to recurrent and/or severe infections. The most frequent are the common variable immunodeficiency (CVID), X-linked agammaglobulinaemia (XLA), hyper IgM syndrome (HIgM) and IgA deficiency (IgAD). In Paraguay, the reports about these diseases are very few.

Objective:

to study serum immunoglobulin levels in patients with recurrent and/or severe infections searching for PAD.

Materials and Methods:

143 patients from 1 to 17 years old were included, registered in a data base from the Instituto de Investigaciones en Ciencias de la Salud from 2012 to 2017, with IgA, IgG and IgM serum levels dosages and clinical reference of recurrent and/or severe infections. An international diagnostic phenotypic algorithm was applied to the hypogammaglobulinaemia cases.

Results:

The average age of the patients was 6 ± 4 years, 54% (77/143) were males, 78% (112/143) were outpatients and 40% (57/143) had respiratory infections. In 90% (129/143) of the patients normal values of serum immunoglobulins were observed and in 10% (14/143) hypogammaglobulinaemia was found, corresponding to one case of XLA, 3 cases of CVID, 5 of IgAD, 2 of HIgM, 2 IgG subclasses deficiencies and one IgM selective deficiency.

Conclusions:

The frequency of DPA cases identified corresponds with data from other countries in which IgAD and CVID are the most common ones. It is important to suspect these pathologies in children and adolescents with recurrent, severe, opportunistic infections and evaluate their serum immunoglobulins for timely diagnostic.

Keywords : Antibody deficiencies; hypogammaglobulinaemia; primary immunodeficiencies..

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