Survey of acute renal failure in patients with cystic fibrosis in the UK

Carol Bertenshaw, Alan R Watson, Sarah Lewis and Alan Smyth

There has been a recent increase in the number of reported cases of acute renal failure (ARF) in cystic fibrosis (CF). The purpose of this study was to determine the incidence risk of ARF in patients with CF in the UK and to identify possible aetiological factors.

The authors asked all doctors working at UK CF centres if they had been involved with the management of a patient with CF who had then developed ARF and those responding positively were then asked to request informed consent for entry into the study and the patient’s case notes were then reviewed. The analysis was restricted to patients developing ARF between 1997 and 2004. A second questionnaire sought information on aminoglycoside prescribing practice. Responses were received from 55 of 56 centres with 64 reports, 9 of which were duplicates, leaving 55 cases; of which consent for entry into the study was obtained in 26 cases. Median age at presentation with ARF was 9.7 years (range 0.4–31.8) and 12 cases were male. The incidence risk of ARF was 4.6 (verified data) to 10.5 cases (all data)/10 000 CF patients/year. In 21 cases (88%) an aminoglycoside was prescribed at onset of ARF or in the preceding week; 16 (76%) of those receiving an aminoglycoside had gentamicin. The authors also noted that a renal biopsy was performed in 7 cases and histological examination revealed acute tubular necrosis in 6, all of whom had received gentamicin. Renal dialysis was required in 13 cases (54%). Complete recovery was seen in 22/24 patients (92%). 

In conclusion, ARF is increasingly being recognised in patients with CF with a significant morbidity rate as most patients require dialysis. This study implicates intravenous aminoglycosides, particularly gentamicin, in the aetiology of ARF in CF.

Thorax 2007 62: 541-545

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