输血依赖型重型地中海贫血患者发生多发性神经病的频率和潜在风险因素:一项横断面研究

IF 0.5 Q4 PEDIATRICS Egyptian Pediatric Association Gazette Pub Date : 2024-03-25 DOI:10.1186/s43054-024-00257-3
Amr I. Risha, Mervat A. Hesham, Usama R. Elsafy, Yosria A. El Taweel, Mohammed M. Omar, Sara F. Saadawy, Diana Hanna
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引用次数: 0

摘要

据报道,β-地中海贫血患者出现了神经系统并发症,包括外周多发性神经病,这对他们的生活质量造成了负面影响。长期缺氧、铁超载、平均年龄和铁螯合剂引起的神经毒性可能是导致神经病变的原因。然而,导致这一并发症的罪魁祸首仍不明确。我们的目的是研究β地中海贫血患者发生多发性神经病的频率和潜在风险因素。我们对 150 名输血依赖型重型β地中海贫血患者进行了横断面研究,他们的平均年龄为 16.44 ± 3.32 岁。我们对运动神经和感觉神经进行了电生理研究。我们发现,31.3%的病例有神经系统表现,这与年龄、病程和频繁输血有很大关系。在 47 例有神经系统表现的患者中,12 例(25.5%)神经传导速度(NCV)异常。10.6%、10.6%和14.9%的患者发现正中神经、腓神经和胫神经运动幅度异常。分别有 4.3%、2.2% 和 10.6% 的患者发现正中神经、腓总神经和胫神经感觉振幅异常。除了 NCV 异常与年龄较大有明显关系外,与其他临床和实验室参数没有明显关系。我们在 B 型地中海贫血患者中发现了高频率的运动和感觉多发性神经病。多发性神经病主要发生在年龄较大的患者身上,这说明地中海贫血患者的神经病可能与年龄有关。包括病程、输血频率和铁超载在内的其他因素可能也有影响,但本研究无法证实。还需要进一步的研究来验证 B 型地中海贫血患者发生多发性神经病的频率和预测因素。
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Frequency and potential risk factors of polyneuropathy in transfusion-dependent Β-thalassemia major patients: a cross-sectional study
Neurological complications, including peripheral polyneuropathy, have been reported in β-thalassemia patients that negatively impact their quality of life. Chronic hypoxia, iron overload, average age, and iron chelators-induced neurotoxicity might contribute to the development of neuropathy. However, the leading offender of this complication remains not clear. We aimed to study the frequency and potential risk factors of polyneuropathy in β-thalassemia patients. We performed a cross-section study on 150 transfusion-dependent β-thalassemia major patients with a mean age of 16.44 ± 3.32 years. We performed electrophysiological studies for motor and sensory nerves. We found that 31.3% of cases had neurological manifestations with significant relation to age, duration of the disease, and frequent transfusion. Out of 47 patients with neurological manifestations, 12 (25.5%) had abnormal nerve conduction velocity (NCV). Abnormal median, peroneal, and tibial nerve motor amplitudes were detected in 10.6%, 10.6%, and 14.9% of patients respectively. Abnormal median, peroneal, and sural nerve sensory amplitudes were detected in 4.3%, 2.2%, and 10.6% of patients respectively. Apart from a significant relation between abnormal NCV and older ages, no significant relation was detected with other studied clinical and laboratory parameters. We detected a high frequency of motor and sensory polyneuropathy in B-thalassemia patients. Polyneuropathy was predominately detected in older ages highlighting that neuropathy in thalassemia patients is probably age-dependent. Other factors including disease duration, transfusion frequency, and iron overload might have a contributing effect, however, that could not be confirmed in this study. Further studies are needed to verify the frequency and predictors of polyneuropathy in B-thalassemia patients.
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来源期刊
自引率
0.00%
发文量
32
审稿时长
9 weeks
期刊介绍: The Gazette is the official journal of the Egyptian Pediatric Association. The main purpose of the Gazette is to provide a place for the publication of high-quality papers documenting recent advances and new developments in both pediatrics and pediatric surgery in clinical and experimental settings. An equally important purpose of the Gazette is to publish local and regional issues related to children and child care. The Gazette welcomes original papers, review articles, case reports and short communications as well as short technical reports. Papers submitted to the Gazette are peer-reviewed by a large review board. The Gazette also offers CME quizzes, credits for which can be claimed from either the EPA website or the EPA headquarters. Fields of interest: all aspects of pediatrics, pediatric surgery, child health and child care. The Gazette complies with the Uniform Requirements for Manuscripts submitted to biomedical journals as recommended by the International Committee of Medical Journal Editors (ICMJE).
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