Retrospective cholecystography studies in adults with hereditary spherocytosis (HS) suggested detectable gallstones in 37% to 43% of patients. Since longitudinal studies using biliary ultrasonography are unavailable, the aim of the present study was to determine the incidence of gallstone disease, as detected by biliary ultrasonography, in children and young adults with HS. As individuals with HS who co-inherit Gilbert syndrome have a greater risk of developing gallstones, uridine diphosphate-glucuronyl transferase (UGT-1A) gene polymorphism was also determined.
The authors retrospectively evaluated 44 patients aged 1.4 to 22 years with HS, 12 (27%) of whom underwent splenectomy. Ultrasonography was performed annually starting at the age of 4 years or at the time of diagnosis, if later.
Of the 44 patients, 18 (41%) developed cholelithiasis as demonstrated by gallbladder ultrasonography. In most patients (94%) the test first proved positive at age 4 to 13 years. Patients with HS and Gilbert syndrome tended to be younger at the time of cholelithiasis.
Early cholelithiasis was detected in children and young adults with HS. To identify this complication, the authors recommend early annual biliary ultrasonography in HS children, starting at about 4 years of age. In patients with Gilbert syndrome, closer follow-up may be indicated.
From the Department of Hematology-Oncology (Drs Tamary, Aviner, Miskin, and Yaniv), Department of Surgery (Dr Freud) and Ultrasound Unit (Dr Schwarz), Schneider Children's Medical Center of Israel, and Pediatric Hematology Laboratory (Dr Krasnov), Felsenstein Medical Research Center, Beilinson Campus, Petah Tikva and Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
Received for publication June 3, 2003; accepted August 14, 2003.
Reprints: Hannah Tamary, MD, Department of Pediatric Hematology-Oncology, Schneider Children's Medical Center of Israel, 14 Kaplan Street, Petah Tikva, 49 202 Israel (e-mail: firstname.lastname@example.org).