Title: The significance of selective IgA deficiency in malabsorptive disorders in children
- Wrzesław Romańczuk, Bogdan M. Woźniewicz, Ryszard Korczowski
- Contemporary Pediatrics, Gastroenterology, Hepatology and Child Feeding
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- selective IgA deficiency, malabsorptive syndromes, children
Selective immunoglobuline A deficiency (IgAD) is the most common primary cause of immunodeficiency. The meaning of IgAD as a reason of intestinal malabsorption is still questioned. Some authors believe that blood IgAD is pathogenic for malabsorption in children olderthan 2-3 years. In years 1989-1999 ninety-six children - 50 girls (mean age 2.2 years) and 46 boys (mean age 3.0 years) - with IgAD have been observed. Fifty-five children inhabited urban area. At least two measurements of serum IgA with results below 5 mg/dL, performed morę than 6 months apart, were assumed as diagnostic criterion of IgAD. 17 cases with IgAD lasting longer than 2 years were classified as persistent selective IgA deficiency (pIgAD). The remaining (79 children) formed the group of transient IgA deficiency (IgAD). In 8 girls (aged 8 months to 8.8 years; mean 3.2 years) and 9 boys (aged 8 months to 12.5 years; mean 5.1 years) pIgAD was diagnosed. In 6 girls villousatrophygrade IV and presenceof IgG-ARA were detected. In 3 boys with pIgAD intestinal biopsy revealed villous atrophy IV° but in 2 other boys there were no such intestinal lesions. In all boys, as well as in girls, including cases with normal intestinal mucosa, elevated blood IgE was detected. Group of children with tIgAD was much morę heterogeneous. In 11 girls (26.2%) and 2 boys (5.4%) villous atrophy III/IV° were observed; in 7 cases (8.9%) there were no structural mucosal abnormalities. In 35 girls and 29 boys blood IgE concentration was above normal. These observations indicate that selective IgA deficiency, both persistent and transient, is inevitably associated with high blood IgE level and development of atopic reactions. IgAD-evoked malabsorption syndrome should be always considered in cases of persistent IgA deficiency.