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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 (>10 mg/dL ) of serum and secretory IgA with normal levels of IgG and IgM. Most patients with IgA deficiency are asymptomatic because of compensatory increases 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 at early diagnosis, supportive management and reducing its impact on the patient’s health.
This study comprised 120 infants and children from The New Valley Governorate in the period from February to October 2006.
Enrollment of patients was done according to:
• The clinical evaluation of immunocompetence. This included a clinical history and physical examination.
• Inclusion criteria:
1. Two or more systemic or serious bacterial infections (e.g., sepsis, osteomyelitis, or meningitis).
2. Three or more serious respiratory or documented bacterial soft tissue infections (e.g., cellulitis, draining otitis media, or lymphadenitis) within one year.
3. Infections occurring at unusual sites (e.g., liver or brain abscess).
4. Infections with common childhood pathogens but with unusual severity.
5. Increased frequency of infections.
6. Prolonged duration of infections.
7. Repeated infections without a symptom free interval
8. Increased dependency on antibiotics.
9. Unexpected or severe complications of infections
• All children included in the study were subjected to the following laboratory investigations which were done according to the requirement of each case:
1.ESR by Westergren method.
2.CBC by Coulter counter.
3.Plain X-ray.
4.Culture from infection sites.
5.Assay of serum level of IgA by turbidimetry. Patients were considered to have complete IgA deficiency if serum IgA was <5 mg/dL and were regarded as having partial deficiency if serum IgA was 5-30 mg/dL.
6- Radial Immunodiffusion was used to determine the exact level in patients who proved to have low IgA (below the level of detection) by turbidimetry.
6. Patients with proved IgA deficiency were assayed for IgG and IgM to settle the diagnosis of either selective IgA deficiency or other defects of immunoglobulins.
Of the 120 patients enrolled, 65 (54%) were males and 55 (46%) were females. The mean age at presentation was 39.6 ± 30.7 months (range, 9 to 144 months ) and the mean age of onset of recurrent infections was 19.5 ± 17.1 months (range, 2 to 72 months). Their weights ranged between 7– 40 kg with a mean value of 14.9 ± 7.2 kg. Their heights ranged between 60-130 cm with a mean value of 87.9 ± 16.7 cm.
IgA assay revealed deficiency in 7 % (8 patients), three patients had complete IgA deficiency, and 5 patients had partial IgA deficiency. Those 8 patients with IgA deficiency have been assayed for IgG and IgM.
It was found that 3 patients had increased IgG levels above normal ranges for age and 4 patients had their IgG levels within normal ranges for age. Two patients had their IgM levels within normal ranges for age and 5 patients had increased IgM levels above normal ranges for age so the result is that 7 of the IgA deficient patients (with IgA levels < 10 mg/dL) were diagnosed as selective IgA deficiency (SIgAD).