Papillary stenosis and sclerosing cholangitis in an immunodeficient child

Sclerosing cholangitis, an inflammatory disease of the biliary tree that occurs infrequently in childhood, has been recognized in combination with papillary stenosis in adults with the acquired immunodeficiency syndrome. A 10-yr-old child with a familial immunodeficiency syndrome characterized by defective T-cell function and deficiencies of immunoglobulins A and G developed papillary stenosis and sclerosing cholangitis associated with cryptosporidium enteritis. The patient presented with fever, jaundice, right upper quadrant pain, and elevated serum concentrations of transaminases and alkaline phosphatase. The pain and jaundice resolved after endoscopic sphincterotomy, but the biochemical abnormalities persisted. This case demonstrates that the combination of papillary stenosis and sclerosing cholangitis can occur in children as well as adults and may be associated with immunodeficiency syndromes other than the acquired immunodeficiency syndrome. Endoscopic sphincterotomy can provide symptomatic treatment for papillary stenosis in children with this condition, although the effect of sphincterotomy on the natural history of the sclerosing cholangitis is uncertain.