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Outcomes for children with gallbladder abnormalities and sickle cell disease
Authors:Suell Mary Nell  Horton Terzah M  Dishop Megan K  Mahoney Donald H  Olutoye Oluyinka O  Mueller Brigitta U
Affiliation:Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, 77030, USA. mnsuell@texaschildrenshospital.org
Abstract:OBJECTIVE: To determine whether elective cholecystectomy is justifiable in children with sickle cell disease (SCD), gallbladder abnormalities, and minimal clinical symptoms. STUDY DESIGN: A retrospective review comparing clinical presentations and abdominal ultrasound results with outcomes in 146 children with SCD. RESULTS: Ultrasound examination showed sludge or stones in 83 of 146 children (57%). This was found during a diagnostic ultrasound in 59 patients (71%) and during a screening ultrasound in 24 asymptomatic patients (29%). Fifty-four (65%) children with a positive ultrasound underwent cholecystectomy; 13 of these were initially asymptomatic patients who had subsequent development of clinical symptoms. Of the patients with cholecystectomy, 93% had histopathologic evidence of cholecystitis. Perioperative complications were rare, and there were no episodes of postoperative acute chest syndrome. Children who underwent elective surgery had an average 12-days-shorter overall hospital stay than those who underwent emergent surgery (4 vs 16 days, P <.001). CONCLUSIONS: Elective laparoscopic cholecystectomy may be safely performed in children with SCD. Surgery should be strongly considered at the time of gallstone diagnosis before symptoms or complications develop. Histopathologic chronic cholecystitis does not correlate with clinical symptoms.
Keywords:Hb-SS, Homozygous hemoglobin SS genotype   SCD, Sickle cell disease   UGT1A, Uridine diphosphate (UDP)-glucuronosyltransferase 1A
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