Abstract

Nephrologists increasingly face the emerging challenge of managing sickle cell disease (SCD) patients with End-stage Renal Disease (ESRD). The increasing cohort of patients is due to improvements in SCD treatment, meaning that more people go on to develop complications such as Sickle Cell-associated Nephropathy (SCAN). Nigeria has the highest burden of SCD in the world, with rising cases of SCAN. In this patient cohort, some authors have recommended kidney transplantation as the choice of renal replacement therapy (RRT). However, the outcomes of renal transplantation in SCD patients have been variable with conflicting data presented in individual studies. We decided to conduct a systematic review to critically evaluate the outcomes of kidney transplantation in sickle cell disease patients and compare data from other countries with Nigeria. We expected that the results of our review would shed more light on kidney transplant outcomes in patients with SCD as well as identify gaps and areas of interest for future studies. We conducted a systematic search of MEDLINE via PubMed to identify studies that reported on outcomes of kidney transplantation in adult SCD patients, restricting results to those published from the year 2000. We then screened papers based on eligibility criteria in stages. First, we screened titles and abstracts based on eligibility. Common reasons for exclusion were studies with participants who did not have SCD and those without any reported outcome measure. Full texts of the remaining articles were subsequently read and selected based on eligibility criteria. We extracted data using a standardised data extraction form and presented our findings in a narrative synthesis. To ensure rigour, we adhered to the Preferred Reporting Items of Systematic Reviews and Meta-Analyses (PRISMA) guidelines in conducting and reporting this review. Database search conducted on 1st march 2020 found 88 papers. We also identified 6 additional articles that met the inclusion criteria following bibliographic review of eligible studies. We screened 94 records by title and abstract and removed 76 that did not meet the inclusion criteria. We screened the full-text versions of 18 articles and identified 9 for inclusion in this review. 6 articles were case reports and 3 were statistical studies using registry data. Insights generated from included studies relate to short term outcomes of renal transplant recipients who have SCD, with patient survival ranging from to 90.5% to 100% in the first year post-transplant. However, long term outcomes are significantly lower and can be as low as 55.7% at six years post-transplant. Renal transplantation in SCD presents both benefits and complications which need to be communicated to patients to ensure informed consent. We can mitigate adverse outcomes with adequate preoperative care and multidisciplinary team approach in managing complications on a case-by-case basis.

Full Text
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