Abstract
INTRODUCTION: sickle cell disease is a genetic disease with autosomal inheritance associated with haemoglobin structure abnormality which causes the formation of hemoglobin S. The purpose of our study was to collect data on digestive diseases in patients with sickle cell disease in Lubumbashi and to highlight their epidemiological and clinical features. METHODS: We conducted a retrospective, descriptive, cross-sectional study at the Research Center for Sickle Cell Disease in Lubumbashi. All the records of patients on follow-up for sickle cell disease with digestive disease during our 3-year period (January 2015 to December 2017) were analyzed. Data were collected using a survey taking into account different study parameters including: age, sex, the reason for consultation, diagnosis, the type of vaso-occlusive crisis, the paraclinical examinations made, hydroxyurea treatment. RESULTS: out of a total of 403 medical records examined we found 206 cases (n=206) of sickle cell disease associated with digestive disease, accounting for a rate of 51,11% of patients with sickle cell disease who suffered from digestive diseases. Both sexes were represented with a slight female predominance (51.94%) and a sex ratio M/F of 0.92. The most represented age ranges 1-6 years (32.52%), the average age was 11.8 years; the standard deviation was 21.9; the extreme ages were 13 months and 38 years. The reason for consultation was dominated by fever (60,67%), abdominal pain (44.66%) and digestive disorders (30,09%). Vaso-occlusive abdominal crises were found in 65 patients (31.55%) among whom 36 had only 1 crisis, 24 had 2 crises and 5 had 3 crises. Intestinal diseases were found in 121 patients (69,41%) dominated by intestinal parasites (found in 58 patients whose collection of stool samples showed 4 parasites: Yersinia enterocolitis, Entamoeba histolytica, Giardia intestinalis and Clostridium difficile). Gastric diseases were found in 105 patients ( 50,97%) divided into peptic ulcer (45 patients) and gastritis (60 patients); biliary vesicular disease was found in 40 patients (19.41%) including vesicular lithiasis without cholecystitis (32 patients), lithiasic cholecystitis (5 patients) and lithiasis in the main biliary tract (3 cases); there was 1 single case diagnosed with acute pancreatitis. The most common associated diseases in our study were respiratory diseases (169 cases;82,03%), oto-rhino-laryngological diseases (157 cases;76.21%), bony, vaso-occlusive crises (146 cases; 70,87%), urogenital diseases (64 cases; 31.06%) and malaria (51 patients; 24.75%). Hepatic diseases and diseases of the spleen were found in 18 cases (8.73%) and 47 cases (22,81%) respectively. Ultrasound was requested in 79 patients but only 31 of them underwent it because of the lack of financial means (it costs 20 U.S. dollars). In the case of clinically obvious splenomegaly, the search for Howell-Jolly bodies was requested in 23 patients but it was only performed in 2 patients because it costs 10 U.S. dollars). Routine blood count, hemoglobin, hematocrit, inflammatory assessment and thick drop examination were performed in all our patients but liver assessment, tests done on stool samples, urine test were recommended based on patient's complaint. Out of 206 patients, only 60 were under hydroxyurea treatment (29,16%). CONCLUSION: digestive diseases are common in patients with sickle cell disease and account for almost half of patients with diagnosed sickle cell disease. Unfortunately, best management is limited by poverty leading to less very useful paraclinical examinations in patients with digestive diseases resulting from sickle cell disease.