Cholelithiasis in patients with paediatric sickle cell anaemia in a Saudi hospital.

OBJECTIVE: Sickle cell disease is one of the most common inherited hemoglobinopathies in the world. Chronic haemolysis predisposes individuals to the development of bilirubinate cholelithiasis, which can be asymptomatic or can result in cholecystitis, choledocholithiasis, cholangitis, and gallstone pancreatitis. We aimed to determine the prevalence of cholelithiasis and associated gallstone disease among patients with paediatric sickle cell disease in a Saudi hospital. METHODS: This retrospective study was conducted among all patients aged between 2 and 18 years. We reviewed the medical records of patients diagnosed with sickle cell anaemia. Mean and standard deviation were calculated for quantitative variables, and the Student t-test was used to compare means. The chi-square test was used to assess those risk factors possibly associated with cholelithiasis. A P-value of ≤0.05 was considered statistically significant. RESULTS: Approximately 75% of participants developed cholelithiasis (27.5%) at a mean age of 6.9 ± 3.4 years. The frequency of cholelithiasis was significantly higher with increasing age (40.8% in participants 12 years and older) and among those with high levels of haemoglobin S (Hb S) and mean corpuscular volume (MCV). Moreover, cholelithiasis was more frequent among males than females, Saudis than non-Saudis, and in those with sickle cell disease than in those with sickle thalassemia. However, these differences were not statistically significant. CONCLUSION: In this study, the prevalence of cholelithiasis among children with sickle cell anaemia was found to be high. This association was significantly increased with age and high levels of MCV and Hb S.

Ceftriaxone induced acute multi-organ failure syndrome in a Saudi boy with sickle cell disease.

Multi-organ failure syndrome (MOFS) is a rare life threatening complication of sickle cell disease. It is precipitated by severe vaso-occlusive episodes. We report a Saudi boy with sickle cell anemia, who developed acute MOFS following anaphylaxis to ceftriaxone administration. He had a dramatic recovery after red blood cell exchange transfusion and peritoneal dialysis.