Abstract

Background: Sickle cell disease has been reported from all over the world including India. The early detection of high bilirubin pattern and cholelithiasis & their treatment is essential for improving quality of life in sickle cell disease children.

Objective:  Current study aims at evaluating bilirubin pattern and incidence of cholelithiasis in cases of sickle cell disease in paediatric population attending RIMS, Ranchi.

Materials & Method: 56 consecutive cases admitted to hospital with sickle cell disease were studied by history, thorough examination & stepwise investigations including CBC with PBS, Liver function tests inclu. Serum bilirubin & ultrasonography of whole abdomen.

Results:  The fever, abdominal pain, tiredness, cough, bone pain, vomiting, headache and chest pain had been noted in 85.7% (48 cases), 33.9% (19 cases), 28.5% (16 cases), 23.2% (13 cases),23.2% (13 cases), 7.1% (4 cases), 1.7% (1 case) and 1.7% (1 case) of sickle cell disease patients respectively but no leg ulcer, priapism or stroke. Most of the patients were males (M:F ratio of 1.95:1), hindu religion(67.9%) & tribals (59%). The reticulocyte count was high (mean 6.67) in all patients and haemoglobin ranged between 4.8-9 gm/dl which was low. Hepatomegaly was present in all cases while 2 patients had splenectomy previously and another 3 patients had no palpable spleen. Patients with gallbladder abnormality in the form of stone or sludge had higher total and direct fraction of serum bilirubin as well as serum transaminases. The overall incidence of cholelithiasis was 7.14%. The incidence of gallstone increases with increase in age.

Conclusion:  There is an increasing incidence of gallstone as well as gallbladder sludge with increasing age among sickle cell disease patients but no difference in earlier age group.

Keywords:  Sickle cell hepatopathy, cholelithiasis.