Article Text

PO-1016 Phenotype Variety In Patients With Selective Iga Deficiency
  1. SI Iurian1,
  2. S Iurian2,
  3. C Cazan1,
  4. D Fintina3
  1. 1Pediatric Clinic, "Lucian Blaga" University Pediatric Hospital, Sibiu, Romania
  2. 2Clinical Laboratory, Pediatric Hospital, Sibiu, Romania
  3. 3Pediatric Clinic, Pediatric Hospital, Sibiu, Romania

Abstract

Background Selective IgA deficiency (SIgAD) is the most frequent primary antibody deficiency.

Aims Authors emphasise the phenotype diversity among SIgAD patients included in our clinic casuistry.

Methods Authors analysed clinical and biochemical features of patients that fulfilled SIgAD diagnosis criteria. They’ve been evaluated concerning previous respiratory and gastro-intestinal diseases severity (necessity for intravenous antibiotics and/or intensive care measures). Serology investigations performed: complete blood count and evaluations for immunoglobulin isotype, IgG subclass, total IgE, specific IgE for cow’s milk precipitins. Autoimmune status estimation included antibodies against: red blood cells, basement membrane, smooth muscle, thyroglobulin, thyroid microsomal antigens, transglutaminase IgG/endomysium and nuclear proteins. Authors accomplished microbiological tests for patients with sinopulmonary infections and analysed stools in order to exclude Giardia/ Cryptococcus infections. Susceptibility for brochiectasis justified chest X-ray.

Results Among 8 SIgAD patients followed in our clinic, 3 patients were considered as asymptomatic. The others were diagnosed with: recurrent knee arthritis in context of Epstein-Barr virus reactivation confirmed by quantitative PCR for DNA-EBV in serum (1 patient), celiac disease (2 patients), giardiasis (2 patients), asthma and allergic rhinitis (1 patient), cow milk allergy (1 patient). Sinopulmonary infections/ purulent otitis were identified for all symptomatic cases. One patient was diagnosed with tonsil phlegmon. No immunological thrombocytopenic purpura or autoimmune hemolytic anaemia were confirmed.

Conclusions 1. In spite of small number of SIgAD patients, authors revealed a quite large range of clinical manifestations: from asymptomatic status to recurrent arthritis due to EBV reactivations; 2. Three patients presented more than 1 clinical feature.

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