High Incidence of Early Cholelithiasis Detected by Ultrasonography in Children and Young Adults with Hereditary Spherocytosis

Hannah Tamary, Shraga Aviner, Enrique Freud, Hagit Miskin, Tatyana Krasnov, Michael Schwarz, Isaac Yaniv

Research output: Contribution to journalArticlepeer-review

50 Scopus citations


Objectives: 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-IA) gene polymorphism was also determined. Patients and Methods: 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. Results: 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. Conclusions: 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.

Original languageEnglish
Pages (from-to)952-954
Number of pages3
JournalJournal of Pediatric Hematology/Oncology
Issue number12
StatePublished - 1 Dec 2003
Externally publishedYes


  • Biliary ultrasonography
  • Cholelithiasis
  • Spherocytosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology


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