Journal Article

Renal function in patients with β-thalassaemia major: a long-term follow-up study<sup>†</sup>

Maria Eliana Lai, Alessandra Spiga, Stefania Vacquer, Maria Paola Carta, Claudia Corrias and Claudio Ponticelli

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 27, issue 9, pages 3547-3551
Published in print September 2012 | ISSN: 0931-0509
Published online June 2012 | e-ISSN: 1460-2385 | DOI: http://dx.doi.org/10.1093/ndt/gfs169
Renal function in patients with β-thalassaemia major: a long-term follow-up study†

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Background

Little information is available about the kidney's involvement in patients with β-thalassaemia major (TM). In particular, there are no studies reporting the outcome of renal function over time.

Methods

In this retrospective study, we evaluated the changes in estimated glomerular filtration rate (eGFR) in 81 adult patients with TM followed for 10 years. Only patients who had an eGFR of >90 mL/min/1.73 m2 at presentation were admitted to the study. All patients were regularly followed for at least 10 years.

Results

At 10 years, 66 patients showed a mild decline in eGFR that remained, however, within a normal range (from 119.9 to 113.6 mL/min/1.73 m2, P = 0.636). In the remaining 15 patients (18.5%), eGFR decreased to <90 mL/min (from 98.1 to 78.2 mL/min/1.73 m2; P = 0.004). The repeated-measures models showed that the decline in eGFR over time was significantly higher (P = 0.0068) in patients with baseline phosphaturia >1000 mg/24 h (P = 0.0068), while eGFR tended to decline more rapidly in patients with baseline uricuria >700 mg/24 h than in those with lower uricuria (P = 0.0783). Univariate Cox's proportional regression models showed that abnormal levels of calcaemia were associated with the risk of kidney damage [hazard ratio (HR) 0.30, 95% confidence interval 0.09–0.97 for calcaemia 8.4–10.2 mg/dL versus HR not estimable for calcaemia <8.4 or >10.2 mg/dL].

Conclusions

In most adults with TM, the eGFR tends to remain within a normal range after 10 years. However, patients with elevated phosphaturia, elevated uricuria and/or abnormal levels of calcaemia show a significant decline in eGFR over time, suggesting that tubular damage acquired in childhood caused by either TM or its treatment may eventually result in abnormal eGFR. Further studies in a larger cohort of TM patients are needed to further elucidate the long-term impact of TM on renal function.

Keywords: anaemia and renal function; iron and renal function; thalassaemia and renal function; thalassaemia major

Journal Article.  3246 words.  Illustrated.

Subjects: Nephrology

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