Faculty Collaboration Database

Increased incidence of HLA-B40 group antigens in children with hemolytic-uremic syndrome. Nephron 1994;68(4):433-6

Date

01/01/1994

Pubmed ID

7870227

DOI

10.1159/000188303

Scopus ID

2-s2.0-0028104391 (requires institutional sign-in at Scopus site)   9 Citations

Abstract

Hemolytic uremic syndrome (HUS) develops in 25-30% of children infected with Escherichia coli strains that produce Shiga-like toxins, also known as verocytotoxins. Mild HUS also occurs in 1 in 4 of the other family members, suggesting a familial predisposition to HUS. To understand the possible genetic predisposition, the frequency of HLA antigens was evaluated in 30 children (12 boys, 18 girls; mean age 3.8 years) with HUS following a prodrome of bloody diarrhea. When compared to a blood donor population from the same geographic area and ethnic background, no significant differences were noted in the frequency of HLA-A, HLA-C, HLA-DR, and HLA-DQ antigens. However, the frequency of HLA-B40 and its splits (B60, 61, 41, 47) was significantly higher in the study population (corrected p < 0.005). The relative risk of developing HUS was 6.04 when HLA-B40 and HLA-B40 split products were present, and the risk increased to 8.5 when the analysis was extended to include the cross-reactive antigens B44 and B13. These HLA-B antigens share common amino acid sequences at positions 41-45 and 67-74 on the alpha-1 domain of the HLA class I molecule. Our data suggest that the inheritance of HLA-B40, its splits, and cross-reactive antigens increases the risk of developing HUS.

Author List

Sheth KJ, Gill JC, Leichter HE, Havens PL, Hunter JB

Author

Peter Havens MD Emeritus Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Child
Child, Preschool
Diarrhea
Female
HLA Antigens
HLA-B Antigens
HLA-B40 Antigen
Hemolytic-Uremic Syndrome
Humans
Infant
Male
Reference Values