Cholelithiasis in children and adolescents with sickle cell disease: Experience in a resource-limited setting

Abstract

Background: Sickle cell disease (SCD) remains prevalent in Nigeria and can be complicated by cholelithiasis even in children. There is still a dearth of knowledge about the occurrence of cholelithiasis in these children. The present study is aimed to determine the prevalence of cholelithiasis in pediatric SCD in Lagos and documents relevant socio-demographic and clinical correlates. Subjects and Methods: This was a cross-sectional study of children and adolescents aged 1–19 years with SCD attending the Paediatric Haematology Clinic of the Lagos University Teaching Hospital. One hundred and forty-seven children were consecutively recruited into the study over 3 months and they all had sonographic examination of the gall bladder. The association between cholelithiasis, sociodemographic data, clinical symptoms, laboratory parameters, and the use of hydroxyurea was also documented. Results: The median age (range) of the study participants was 9.0 (1–19) years and majority were males (59.9%). The prevalence of cholelithiasis was 13.6% and the condition was most prevalent in adolescents (21.4%) compared to the younger children (6.5%). All the children with cholelithiasis were asymptomatic. Age and the frequency of crisis were significantly associated with cholelithiasis on multivariate analysis ( P = 0.03, 0.045, respectively). The use of hydroxyurea was not significantly related to the occurrence of cholelithiasis. Conclusion: The prevalence of cholelithiasis observed in this study is high. Routine screening of older children and adolescents with SCD, especially with the frequent crisis is suggested. Longitudinal studies to establish the relationship between hydroxyurea and cholelithiasis is also advocated.

Predisposing factors for advanced liver fibrosis in patients with sickle cell disease

“…Both tests have been widely used in patients with chronic viral hepatitis (HCV and HBV) and non‐alcoholic steatohepatitis (NASH) for the diagnosis of advanced fibrosis (≥F3) and cirrhosis with high efficacy 6 . In advance, they have been recently used in SCD patients in small cohorts, as a reliable screening tool for fibrosis when liver elastography and liver biopsy are not available 7 …”

“…6 In advance, they have been recently used in SCD patients in small cohorts, as a reliable screening tool for fibrosis when liver elastography and liver biopsy are not available. 7 Imaging evaluation included: upper abdomen ultrasound and triplex evaluation assessing signs and features of cirrhosis (inhomogeneous nodular liver parenchyma, increased size of caudate lobe and nodular liver surface), portal hypertension (diameter of portal vein, blood flow and presence of collaterals), echocardiographic assessment for right heart failure and estimation of pulmonary artery systolic pressure (PASP) with tricuspid regurgitation velocity (TRV) measurement, transient elastography (Fibroscan) and liver MRI for liver iron concentration (LIC).…”

Sickle Cell Disease (SCD) Leading to Pulmonary Arterial Hypertension (PAH) and Cholelithiasis (CL)

“…Sickle cell disease (SCD) is a diverse set of hereditary hemoglobinopathies linked to alterations in the beta component of the hemoglobin (Hb) molecule. Since it is an autosomal recessive disease, disease states and complications would only be present in individuals who were homozygous for the mutant allele or who were found to be compound heterozygotes with mutant alleles linked to other hemoglobinopathies (such as HbC disease, sickle cell Hb/beta-thalassemia, etc) [ 1 ]. For diagnosing SCD , certain Hb tests like Hb electrophoresis and sickling test are needed.…”

“…Chronic hemolysis, which causes considerable bilirubin generation, can lead to gallstone formation. One of the key symptoms of the condition is pigment gallstone development, which is caused by the bilirubin generated [ 1 ]. Gallstone development in SCD patients may also be influenced by changes in the function of the gall bladder or bile acid metabolism.…”

“…One of the most significant gastrointestinal signs of SCD is the development of gallstone [ 1 , 2 ]. which is mostly brought on by too much cholesterol in bile.…”