Abstract

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

Methods: All doctors working at UK CF centres were asked if they had been involved with the management of a patient with CF who had developed ARF. Those responding positively were 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.

Results: Responses were received from 55 of 56 centres with 64 reports, 9 of which were duplicates, leaving 55 cases. Consent was obtained for data extraction in 26 cases, of which 24 fitted the criteria for ARF (verified data). 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. 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%).

Conclusions: ARF is increasingly being recognised in patients with CF. There is significant morbidity with most patients requiring dialysis. This study implicates intravenous aminoglycosides, particularly gentamicin, in the aetiology of ARF in CF.