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Gall stones in Jamaican children with homozygous sickle cell disease
D K Webb1, J S Darby, D T Dunn
1Medical Research Council Laboratories, University of the West Indies, Kingston, Jamaica.
Insights
Gallstones are common in children with sickle cell disease (SCD). While associated with pain crises, gallstones themselves rarely cause specific symptoms, and pain crises alone aren't an indication for surgery.
Area of Science:
- Pediatric Hematology
- Gastroenterology
- Medical Imaging
Background:
- Sickle cell disease (SCD) is a genetic blood disorder associated with various complications.
- Gallstone formation (cholelithiasis) is a known complication, particularly in patients with chronic hemolysis.
- Understanding the prevalence and clinical significance of gallstones in pediatric SCD is crucial for management.
Purpose of the Study:
- To determine the prevalence of gallstones in children with homozygous sickle cell disease.
- To investigate the association between gallstones and hematological parameters, bilirubin levels, and clinical manifestations like abdominal pain crises.
- To evaluate whether gallstones or associated pain crises warrant surgical intervention in this pediatric population.
Main Methods:
- A cohort study involving 226 children with homozygous sickle cell disease, aged 5-13 years, followed from birth.
- Ultrasonography was used to detect gallstones.
- Hematological parameters (hemoglobin, fetal hemoglobin) and bilirubin concentrations were analyzed.
- Association with abdominal pain crises was assessed.
Main Results:
- Gallstones were detected in 13% (30 of 226) of children.
- Children with gallstones exhibited lower hemoglobin and fetal hemoglobin levels and higher bilirubin concentrations.
- The association with hemoglobin levels was secondary to bilirubin concentrations.
- Abdominal pain crises were significantly associated with gallstones, reflecting overall clinical severity rather than a direct causal link.
Conclusions:
- Gallstones are prevalent in children with homozygous sickle cell disease.
- The presence of gallstones is linked to hemolysis markers but not directly causal for pain crises.
- Abdominal pain crises alone should not be considered an indication for gallstone surgery in pediatric SCD patients.
Abstract:
Gall stones were detected by ultrasonography in 30 of 226 (13%) children with homozygous sickle cell disease aged 5-13 years participating in a cohort study from birth. Children with gall stones had significantly lower total haemoglobin and fetal haemoglobin and higher bilirubin concentrations, but further analysis showed that the apparent effects of haemoglobin and fetal haemoglobin concentration were secondary to their relationship with bilirubin concentrations. Abdominal pain crises were significantly associated with gall stones but both factors appeared to reflect an increased clinical severity and were probably not causally related. No patients had symptoms specific of gall stones and an association with abdominal pain crisis should not, of itself, be considered an indication for surgery.