RENAL COMPLICATIONS OF SICKLE CELL DISEASE IN AN AUSTRALIAN COHORT

Dr Louise Keamy1, Dr Sara Dohrmann2, Ms Claire Dowsing2, A/Prof Kylie Mason1,2, Prof Kathy Nicholls1

1Royal Melbourne Hospital, Parkville, Australia, 2Peter MacCallum Cancer Centre, Parkville, Australia

Biography:

Louise is a final year Nephrology Advanced Trainee in Victoria, currently working at Western Health

Aim:

To document the frequency of renal disease in patients with sickle cell disease (SCD) at The Royal Melbourne Hospital and to quantify the impact of renal disease on quality of life (QoL).

Background:

The prevalence of SCD in Australia has increased with changing migration patterns. SCD is an autosomal recessive disease characterized by the presence of haemoglobin S, which causes vaso-occlusive disease (VOD) and can result in renal injury. The prevalence of chronic kidney disease (CKD) is significantly higher than in the general population. Renal involvement leads to poor prognosis and reduced life expectancy. Screening for albuminuria is recommended annually from age 10. Treatment with hydroxyurea prevents VOD and is associated with lower rates of albuminuria.

Methods:

A single-centre, prospective cohort study in which demographic, clinical, biochemical and quality of life data were collected

Results:

20 patients were consented with a mean age 39. Of these, 15 completed QoL questionnaires, and 11 had screening performed within the past year. Regarding treatment, 18 had regular red cell exchange and 13 were on hydroxyurea. 25% had a prior renal event, including either acute kidney injury, haematuria or urine infection. The mean creatinine was 71 (CI 49-92), and 37.5% had albuminuria. In the QoL data, 73.3% had better QoL compared to a population matched for age/sex (AQoL-8D score), while the mean RQ-5D score was 0.8.

Conclusion:

This SCD cohort had elevated rates of renal events and albuminuria despite their relatively young age. Routine screening was frequently incomplete, and 35% were not receiving hydroxyurea. There were inconsistent QoL data between surveys, however there was a correlation between renal disease and poorer QoL indices.

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