Zafeiriou D I, Kousi A A, Tsantali C T, Kontopoulos E E, Augoustidou-Savvopoulou P A, Tsoubaris P D, Athanasiou M A
First Pediatric Clinic, Aristotelian University of Thessaloniki, Greece.
Pediatr Neurol. 1998 May;18(5):420-4. doi: 10.1016/s0887-8994(98)00004-6.
Forty patients with beta-thalassemia major (BTM), between 11 and 19 years of age and maintained on long-term desferrioxamine (DFO) treatment, were examined by evoked potential and nerve conduction velocity studies to investigate a possible involvement of the auditory, visual, somatosensory, or peripheral nervous pathways. Pathologic findings in brainstem auditory-, visual-, and somatosensory-evoked potentials, and nerve conduction velocity studies were demonstrated in 25%, 15%, 7.5%, and 25% of the patients, respectively, whereas 15% demonstrated involvement of multiple neural pathways. Subclinical involvement of the auditory pathway was statistically associated with higher mean daily DFO dose and longer duration of DFO therapy, whereas abnormalities regarding the somatosensory pathways were related to older age, longer mean duration of DFO therapy, and lower serum copper levels. Involvement of the peripheral nervous system was related to lower serum copper levels. Multiple involvement of neural pathways was related to longer mean duration of DFO therapy. We conclude that risk factors related to long-term DFO treatment are only partly responsible for the subclinical involvement of neural pathways demonstrated in beta-thalassemia major patients.
对40例年龄在11至19岁之间、长期接受去铁胺（DFO）治疗的重型β地中海贫血（BTM）患者进行了诱发电位和神经传导速度研究，以调查听觉、视觉、躯体感觉或周围神经通路是否可能受累。脑干听觉诱发电位、视觉诱发电位和躯体感觉诱发电位以及神经传导速度研究的病理结果分别在25%、15%、7.5%和25%的患者中得到证实，而15%的患者表现为多条神经通路受累。听觉通路的亚临床受累与每日平均DFO剂量较高和DFO治疗持续时间较长在统计学上相关，而躯体感觉通路的异常与年龄较大、DFO治疗平均持续时间较长和血清铜水平较低有关。周围神经系统受累与血清铜水平较低有关。神经通路的多重受累与DFO治疗的平均持续时间较长有关。我们得出结论，与长期DFO治疗相关的危险因素仅部分导致了重型β地中海贫血患者中所显示的神经通路亚临床受累。
