Abstract

BackgroundEarly detection and interventions have enabled patients with sickle cell disease (SCD) to live well into adulthood. Consequently, the chronicity of SCD allows for the insidious manifestation of multisystem complications, including renal damage. Cystic renal lesions are commonly incidentally discovered on ultrasound and computerised tomography (CT) imaging of the abdomen. Most are benign simple cysts, however, difficulties may be encountered if infection, rupture, haemorrhage or cancerous changes develop. We aimed to determine whether patients with SCD have a higher prevalence of simple renal cysts compared to non-SCD individuals.MethodsData for a group of 223 patients with SCD who had undergone an ultrasound and/or CT imaging of the abdomen were extracted for comparison with 180 control patients (haemoglobin genotype unknown), matched for age and ethnicity. Scans were evaluated for 198 SCD patients and 180 controls.ResultsRenal cysts were found in 58% of the SCD group and 20% of the controls (OR 5.4 (CI 2.6–11.0), RR 2.8 (CI 1.9–4.2)). Bilateral renal cysts were found in 28% of the SCD participants in comparison with 5% of the control group. In those who had one or more cysts identified, the average number of cysts was 3.76 for the SCD group and 1.94 for the controls. Men with SCD were more likely to develop cysts than women (66% vs 53%), as were men without SCD (22% vs 17%).ConclusionsSimple renal cysts occur more frequently, are more abundant and develop at a younger age in patients with SCD than ethnically-matched controls. Further study of the mechanism underlying cyst formation may shed light on both sickle cell nephropathy and other cystic renal diseases.

Highlights

  • Detection and interventions have enabled patients with sickle cell disease (SCD) to live well into adulthood

  • Patients with an estimated Glomerular Filtration Rate of less than 60 ml/min were not included in the study as both the presence of SCD and renal cysts are associated with chronic kidney disease

  • Bilateral renal cysts were found in 28% of the SCD participants in comparison with 5% of the control group

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Summary

Introduction

Detection and interventions have enabled patients with sickle cell disease (SCD) to live well into adulthood. The chronicity of SCD allows for the insidious manifestation of multisystem complications, including renal damage. Sickle cell disease (SCD) is one of the most common inherited blood disorders worldwide [1]. It is caused by the presence of haemoglobin S (HbS) which results from the substitution of glutamic acid by valine in the sixth codon of the beta haemoglobin chain. Improved general medical management has helped patients with SCD live considerably longer, allowing for the insidious manifestation of multisystem complications and end-organ damage [3, 4]. Renal involvement affects approximately 60% of patients with sickle cell disease (HbSS) at some point during their life only 10 to 15% of these patients will develop end stage renal failure [5]

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