Clinical characteristics of proximal tubular acidosis induced by deferasirox in children with β-thalassemia major
Objective To analyze the clinical characteristics of proximal tubular acidosis induced by deferasirox in children with β-thalassemia major.Methods This study retrospectively analyzed the clinical manifestations,laboratory test results,radiological features,treatment methods and outcomes of proximal tubular acidosis in 5 children with β-thalassemia major.Results Five patients aged 5 to 16 years,included 2 males and 3 females.Two patients were diagnosed with Fanconi syndrome.Four patients received deferasirox doses exceeding the conventional dose,including one with a rapid decline in ferritin levels.Gastrointestinal symptoms were the initial manifestations in 3 patients,while only mild anorexia was observed in 2 patients.There were significant correlations between the severity of clinical manifestations,the degree of acidosis and its duration.All patients had abnormally elevated u-rine β2-microglobulin levels,and 4 patients had hepatic iron deposition.After reducing or discontinu-ing deferasirox and providing symptomatic treatment,the renal tubular function of all 5 patients gradu-ally recovered.Conclusion Proximal tubular acidosis induced by deferasirox in patients withβ-thalassemia major can manifest as partial or complete proximal tubular acidosis,which may be asso-ciated with high-dose deferasirox administration and rapid decreases in body iron levels.Regular moni-toring of relevant indicators is recommended for children receiving long-term deferasirox treatment.Once proximal tubular acidosis is detected,drug dosage adjustment or discontinuation should be promptly implemented,and renal tubular function can recover in most patients.
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