الفهرس 
Primary Immunodeficiency DiseasesOnly 14 pages are availabe for public view
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Abstract
IgA is the major protective immunoglobulin of external secretions, that is those of the gastrointestinal, respiratory and urogenital tracts, but it is also present in the circulation against bacterial and viral microorganisms.
Selective IgA deficiency is characterized by an isolated absence or near absence (>4 mg/dL ) of serum and secretory IgA with normal levels of IgG and IgM. Most patients with IgA deficiency are asymptomatic because of compensatory increase of IgG and IgM, but some may suffer from frequent mainly respiratory infections.
The aim of our study was to determine the frequency of IgA deficiency in these children aiming for early diagnosis, supportive management and reducing its impact on the patient’s health.
This study comprised 04 infants and children from Assuite Governorate in the period from December 7414 to July 7411. Patients included in the study had 7 or more signs of the 14 warning signs for suspecting primary SIgA deficiency. However, patients with any 7ry cause of immune deficiency or receiving immunosuppressive drugs were excluded.
Of the 04 patients enrolled, 34 (443) were males and 34
(44%) were females. The mean age at presentation was 36.1 ±
34.0 months with a range from 8 to 144 months. The mean age of onset of recurrent infections was 16.4 ± 10.1
months,with a range from 7 to 07 months. Their weight ranged between 0– 34 kg, with a mean value of 14.6 ± 0.7 kg. Their height ranged between 14-137 cm with a mean value of 80.6 ±
11.0 cm. IgA assay revealed deficiency in 1.47 % (1 patient) who had partial IgA deficiency.In this study, 843 were below 4 years old , 143 were below the 44th percentile for body weight, and 843 were below 44th percentile for height. Chest infections represented 08.13
of our sample with bronchitis and Pneumonia being more frequent. Also in this study the frequency of infections was high with a range from 14.1-14.1 time per year, with longer durations of infections and earlier onset of recurrent infections. Patients with severe infections had significantly lower level of serum IgA, and repeated infections without a symptom free interval more than those of mild and moderate infections.
Serum level of IgA was positively correlated to the age, body weight , body height, and inversely correlated to the duration of infections. No gender effects on serum IgA level were noticed. Serum level of IgA had a significant variation among the different types of infection being lowest in bronchiolitis and bronchopneumonia with significant difference between the diffrenet types of infection, IgA serum level had no significant relation to laboratory data.
It is concluded that, in this study the prevalence of selective IgA deficiency (SIgAD) in our sample was 1.473. SIgAD among patients with repeated, systemic, severe, unusual or prolonged infections, who are suspected of having primary immunodeficiency is uncommon in our country. And we recommend a replication of this study on a large scale of population, aiming for early diagnosis of selective IgA deficiency, supportive management and reducing it’s impact on the patient’s health.