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[地中海贫血患者去铁胺耳毒性的评估。5年随访及结果]

[Evaluation of desferrioxamine ototoxicity in thalassemic patients. Follow-up over a 5-year period and results].

作者信息

Sacco M, Meleleo D, Tricarico N, Greco Miani A, Serra E, Parlatore L

机构信息

Divisione di Pediatria, Ospedale Casa Sollievo della Sofferenza, IRCCS-S. Giovanni Rotondo, Foggia.

出版信息

Minerva Pediatr. 1994 May;46(5):225-30.

PMID:8090141
Abstract

Desferrioxamine (DFO), an iron-chelating drug, up to today has been essential for the care of thalassaemic patients. Unfortunately it presents some toxic effects. One of these is sensorineural hypoacusia, already reported some years ago. In 1987 we documented in our patients a prevalence of 19.4% (7 cases of 36) of sensorineural hypoacusia that proved to be significantly associated with the highest pro kg/die doses of DFO. We made a follow-up audiometry over a 5-year period after a general adjustment of the DFO dosage related to serum Ferritin levels. Four patients with mild hypoacusia (30-50 dB) showed normal audiometry after one year. The hypoacusia of the two severe cases (50-80 dB) proved stable until 5-years later in one case and increased in the other. Only a new case arose denovo. In pathologic patients "Therapeutic index" (DFO/serum Ferritin) was significantly higher than in normoacusic ones and the "threshold" value between the two populations was of 0.027. We think that the Therapeutic index may be a useful guideline to calculate safe doses of DFO about ototoxicity.

摘要

去铁胺(DFO)是一种铁螯合剂药物,直至今日一直是地中海贫血患者治疗的关键药物。不幸的是,它存在一些毒副作用。其中之一是感音神经性听力减退,早在几年前就有相关报道。1987年,我们在患者中记录到感音神经性听力减退的患病率为19.4%(36例中有7例),事实证明这与最高每千克/每日剂量的DFO显著相关。在根据血清铁蛋白水平对DFO剂量进行总体调整后,我们进行了为期5年的随访听力测定。4例轻度听力减退(30 - 50分贝)的患者在1年后听力测定结果恢复正常。2例重度病例(50 - 80分贝)中,1例的听力减退直至5年后仍保持稳定,另1例则有所加重。仅新出现了1例病例。在听力异常的患者中,“治疗指数”(DFO/血清铁蛋白)显著高于听力正常的患者,且这两组人群之间的“阈值”为0.027。我们认为治疗指数可能是计算关于耳毒性的DFO安全剂量的有用指导。

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