Journal
KIDNEY INTERNATIONAL
Volume 80, Issue 1, Pages 79-87Publisher
ELSEVIER SCIENCE INC
DOI: 10.1038/ki.2011.16
Keywords
genetic renal disease; glomerular disease; glomerulonephritis; Henoch-Schonlein purpura; IgA nephropathy
Categories
Funding
- American Philosophical Society
- National Center for Research Resources (NCRR), National Institutes of Health (NIH)
- NIH Roadmap for Medical Research
- National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) [DK082753, DK078244, DK080301, DK075868, DK083663, DK071802, DK077279]
- National Kidney Foundation
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Serum galactose-deficient immunoglobulin A1 (Gd-IgA1) is an inherited risk factor for adult IgA nephropathy (IgAN). In this paper, we determined the heritability of serum Gd-IgA1 levels in children with IgAN and Henoch-Schonlein purpura nephritis (HSPN), two disorders with clinical phenotypes sharing common pathogenic mechanisms. Serum Gd-IgA1 concentrations were quantified using a Helix aspersa-lectin-based enzyme-linked immunosorbent assay. As a group, 34 children with either disorder (20 with HSPN and 14 with IgAN) had significantly higher Gd-IgA1 levels compared with 51 age-and ethnicity-matched pediatric controls. Serum levels of Gd-IgA1 were also elevated in a large fraction of 54 first-degree relatives of pediatric IgAN and HSPN patients compared with 141 unrelated healthy adult controls. A unilineal transmission of the trait was found in 17, bilineal transmission in 1, and sporadic occurrence in 5 of 23 families when both parents and the patient were analyzed. There was a significant age-, gender-, and household-adjusted heritability of serum galactose-deficient IgA1 estimated at 76% in pediatric IgAN and at 64% in HSPN patients. Thus, serum galactose-deficient IgA1 levels are highly inherited in pediatric patients with IgAN and HSPN, providing support for another shared pathogenic link between these disorders.
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