Dr Claire Sharpe, King’s College London / King’s College Hospital NHS Foundation Trust, UK
There are currently over 12,000 sufferers of sickle cell disease (SCD) living in the UK, the majority of whom live in London. Glomerulopathy and renal failure are recognised complications in patients with SCD and these are becoming more prevalent as improvements in medical care have led to an ever-increasing life expectancy for people with SCD. Early features of sickle cell nephropathy (SCN) include an increase in glomerular filtration rate (GFR), an inability to concentrate urine (hyposthenuria) and proteinuria. Repeated medullary ischaemia leads to relative proximal tubular “hyperfunction” as demonstrated by increased creatinine secretion and reabsorption of beta2-microglobulin. Prolonged hyperfiltration and raised glomerular pressure leads to chronic renal failure with a reduced GFR (<60 ml/min) in approximately 4–20% of patients, many of whom will go on to develop end-stage renal failure.
This talk will highlight the underlying pathophysiology that gives rise to renal impairment and the rationale around treatment options. It will be illustrated with examples drawn from our experience gained in King’s College Hospital in caring for adult patients with sickle cell nephropathy over the past 15 years. It will cover the spectrum of renal disease from early monitoring in the outpatient clinic to the care of patients with end-stage renal failure including dialysis and renal transplantation.