Sickle Cell Hepatopathy: Prevalence and Presentation in Children with Sickle Cell Anemia at Enugu State University Teaching Hospital, Parklane, Enugu

Abstract

Background: Sickle cell hepatopathy (SCH) are liver pathologies related mainly to sickling. These conditions present similarly yet have varied prognosis, high index of suspicion is therefore needed for early and proper diagnosis. In Nigeria literature on SCH is variable and scarce. Aim: To evaluate the clinical and laboratory evidences of SCH in Nigerian children with SCA. Study Design:  Cross-sectional, observational study. Place and Duration: SCD clinic, Department of Paediatrics, Enugu State University Teaching Hospital, Enugu, South-East Nigeria. Three months. Methods: A presumptive diagnosis of SCH based on icteric or anicteric hepatomegaly with or without right hypo-chondrial pain was made in thirty (30%) of 100 patients, 15 males and 15 females in a ratio of 1:1. Only 10 out of the 30 (33.3%) patients had complete data with final sample size of 10. History of scleral icterus, abdominal pain, multiple blood transfusions and frequent crises were obtained. Patients were examined for sclera icteria, hepatomegaly and right upper quadrant tenderness. LFT, blood film, CBC, hepatitis virus serology, haemoglobin quantitation, serum ferritin and abdominal ultrasonography (USS) were obtained. A definitive diagnosis of SCH was based on significant markedly elevated ALP, ALT and AST, marked total bilirubin with conjugated bilirubin > 50% total for AIHC (but < 50% for AHSS and AHSCC). Moderately elevated AST, ALT, mildly elevated ALP for AHSCC, moderately elevated ALP, normal ALT, normal or mildly elevated AST and significant sudden decline from steady state haemoglobin for AHSS. Results: Using appropriate statistical tools and software at 5% level of significance, we found that eighty percent (8 out of 10) had mild-to-moderate derangement of LFT but only 1 patient (10%) had LFT suggestive of AIHC. One (10%) patient’s LFT suggested AHSCC. One patient (10%) had features of AHSS. Conclusions: SCH is rare in children but may present as AHSCC, AHSS and AIHC.