Cholelithiasis in Children with Immunoglobulin A Deficiency: A New Gastroenterologic Syndrome

In view of the role of immunoglobulin A (IgA) in the local host defenses of the gut and biliary tract, and the association of IgA deficiency with various intestinal disorders, an ultrasonographic study was performed to determine the prevalence of gallstones in selective IgA deficiency. Eight of 13 children (three girls and five boys) with selective IgA deficiency had abnormal biliary sonograms. Four had cholelithiasis, and four had biliary sludge only. IgA deficiency may predispose to gallbladder infection, and thus to the development of gallstones. Decreased bile acid pool due to intestinal losses, and biliary stasis due to reduced hormonal gallbladder stimulation may also play a role in some cases, but further studies are needed. This association adds a new gastrointestinal abnormality to those known to be associated with selective IgA deficiency.