Hepatobiliary involvement of Henoch-Schönlein purpura in children.

Abstract

Hepatobiliary manifestation and its evolution in children with Henoch-Schönlein purpura (HSP) had been scarcely reported. Over a ten-year period between June 1988 and November 1998, medical charts of 225 children with the diagnosis of HSP encountered at Chang Gung Children's Hospital were reviewed. Those with the evidence of hepatobiliary involvement were enrolled in the study. The patients with hepatobiliary involvement were defined by having an elevated serum alanine transaminase (ALT) or gamma-glutamyl transferase (GGT), and the presence of abnormal sonographic findings involving liver, biliary system, and gallbladder. The patients included 9 boys and 11 girls with range of age from 3 to 11 years. The mean age of these patients was 6.5 +/- 3.7 years. Preceding upper respiratory tract infection was common in these patients (35%). The main clinical manifestation were distinct from typical abdominal symptoms of HSP, and included right upper quadrant pain (80%), nausea (45%), lethargy (20%), and vomiting (15%). Elevated serum ALT was noted in 15 cases (75%) and GGT in 6 cases (30%). Abdominal ultrasonography revealed hepatomegaly (15/20, 75%), and gallbladder wall thickening (5/20, 25%). No specific laboratory parameters were correlated with the hepatobiliary manifestation of HSP. Fourteen children (70%) received steroid therapy and disappearance of symptoms and sonographic resolution of hepatobiliary lesion was noted within 3-7 days of steroid therapy. All patients recovered completely except for one patient with hepatobiliary recurrence manifested as purpuric rash, abdominal pain, elevated GGT, and gallbladder wall thickening during 2-year follow-up.