Naslov
Immunological deficits in children with recurrent pneumonia

Autori
Zenić, Lucija ; Miculinić, Andrija ; Polančec, Denis ; Živković, Jelena ; Turkalj, Mirjana

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
EAACI Congress 2017 : abstracts / - , 2017, Xx-xx

Skup
EAACI Congress 2017

Mjesto i datum
Helsinki, Finska, 17.06.2017. - 21.06.2017

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
immunofenotyping, recurrent pneumonia, children

Sažetak
As a part of routine diagnostics performed at our Department, most of the children submitted to immunofenotyping are suspected of having primary immunodeficiency. These immune disorders characterized by the loss of innate and/or adaptive immune system might account for recurrent pneumonia, defined as two or more episodes of pneumonia in a six-month period or tree or more episodes in a lifetime. The aim of this study was to evaluate if a partial or a complete lymphocyte deficit is seen in children with recurrent pneumonia received at our Hospital. Flow cytometry-based immunophenotyping of peripheral blood was performed in children suffering from recurrent pneumonia, and screened for T cells, NK cells, B cells (our basic panel) as well as for the eight B cell subpopulations (expanded panel). A retrospective study of the immunophenotyping analyses performed within one year involved 22 patients (age 3-13, both sexes equally represented) and the results were summarized according to the corresponding referent age values. Fourteen patients had a decreased number of double-negative B cell subset, nine had a decrease of plasmablasts, and six patients were deficient in the switched memory B-cell subpopulation, as compared to the reference values of their respective age. In some patients, an increase of naive (4 patients), transitional (4 patients) and non-switched (4 patients) B cell subsets was present. Furthermore, among the T lymphocyte population, activated T lymphocytes (mainly involving CD8 T cells) showed an increased frequency (5 patients), while both an increase (2 patients) and a decrease (2 patients) was noticed in the NK-cell population. Immunophenotyping by flow cytometry remains a valuable and indicative method for the diagnosis and monitoring of primary immunodeficiency in children affected by recurrent pneumonia. As suspected, most patients presented with a deficit of the B-cell population, namely terminally differentiated B-cell subsets, while the accumulation of less differentiated B-cell subsets was pronounced. This immunologic pattern reflects a defective progression of the B-cell differentiation process and implies a genetic mutation as an underlying mechanism of the immunodeficiency disease. Therefore, these patients qualify as candidates for the genetic analysis by the next generation sequencing technique that is being introduced at our Department as an inevitable part of translational and clinical research.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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