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Alerg Asma Inmunol Pediatr 2008; 17 (1)
Language: Spanish
References: 32
Page: 14-34
PDF size: 937.43 Kb.
ABSTRACT
Most of the physicians that deal with patients with recurrent infections will have to elucidate the diagnosis
among an allergic disease, immunodeficiency or the combination. Patients with primary enferimmunodeficiency (PID) and allergic disease will present recurrent infections symptoms. There are reports of the humoral immunodeficiency and atopy association. The atopy has been report in a 15-20% of the children population and may produce a chronic inflammation of the respiratory mucosa, being an important factor that triggers the recurrent infections. It has been demonstrated that a good control of the allergic symptoms decreases the frequency of the bacterial infections, but this is not the case, when the patient has immunodeficiency, so it is necessary to treat the allergic disease simultaneously.
The PID can be abnormalities such as the lack of maturation, function or growth or the immune cells system. Patients with PID, have higher risk of infections, allergies, autoimmune and neoplasic problems. The cytometry has contributed to the more complete classification in the molecular and genetic defects of the PID, with a report of more than 100 PIDs, with an increasing number of those.
There are necessary elements to establish the exact diagnostics of the PIDS, such as a good clinical history, to think in the clinical possibility of an immunodeficiency and a specialized laboratory.
This article is oriented to the most recent advances in the molecular aspects of the immunodeficiencies where we analyze, the signaling defects, growth factors, development of the T lymphocytes (BL) and B lymphocytes (BL) and its surviving deficiencies in the T and B cells interaction, such as those in the two genes CD40L and CD40, the costimulators molecules ICOS y ICOS-L.
Defects of the phagocytic cells function such as the neutrophil defects, severe congenital neutropenia, leucocytes adhesion deficiency type I, LAD II deficiency, LAD II deficiency, gene CYBB link to X, deffects in the IFN-gamma receptors or its signaling transduction or the signaling transduction components (Stat1).
Complement system, and the genetic defect such as those in the genes RAG1 and RAG2 in BL and TL,
Artemis and DNA IV ligase genes, RFX5, RFXAP, RFXANK, CIITA, ADA and PNP deficiency, dysregulation of the immune system with the protein immunodeficiency’s involved in the granules exocytosis, SAP deficiency, Lyst deficiency, such as the complement system defects and the immune system basis in the
correlations with the clinical application. Also we describe the clinical specific laboratory tests, therapeutic indications and treatment of the patients with PIDs and allergic disease combination.
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