Prevalence of peripheral neuropathy in children with transfusion-dependent thalassemia: A hospital-based cross-sectional study.

IF 1.1 Q4 PRIMARY HEALTH CARE
Aniruddha Rathore, Mukesh Dhankar, Sharmila B Mukherjee, Suvasini Sharma, Shailaja Shukla, Piali Mandal
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引用次数: 0

Abstract

Background: Our study aimed to determine the prevalence of Peripheral Neuropathy (using nerve conduction studies (NCS)) in children with transfusion-dependent thalassemia aged between 5 to 18 years and to study its correlation with chronic anemia, ferritin levels, chelation status, annual transfusion requirement, deficiency of serum Vitamin B12, and Folate levels.

Methods: In this hospital-based cross-sectional study, 100 eligible children were enrolled in a tertiary care teaching hospital in New Delhi, India. Neurological examinations focusing on peripheral neuropathy followed by NCS were performed on all the patients. Age-wise cutoff values outside of 2.5 SD of normal were taken as abnormal.

Results: None of the children had clinical features of peripheral neuropathy, although 77% had abnormalities in NCS. Of these, 33% had pure motor nerve changes, 7% had pure sensory nerve changes, 1% had abnormal F responses, and 26% had mixed nerve changes. These changes correlated significantly with chronic anemia and duration of iron chelation but not with other factors.

Conclusion: In children with transfusion-dependent thalassemia who do not exhibit any neurological signs or symptoms, however, it is not uncommon to observe abnormal NCS at an average hemoglobin (Hb) level of less than 9.5 g/dl. Further comprehensive case-control studies are necessary to determine if a more specific Hb target range of 9.5 to 10.5 g/dl is appropriate and to investigate the potential impact of chelation therapy on these changes.

输血依赖型地中海贫血患儿周围神经病变的患病率:一项基于医院的横断面研究。
背景:我们的研究旨在确定5 - 18岁输血依赖型地中海贫血儿童周围神经病变的患病率(使用神经传导研究(NCS)),并研究其与慢性贫血、铁蛋白水平、螯合状态、每年输血需求、血清维生素B12缺乏和叶酸水平的相关性。方法:在这项以医院为基础的横断面研究中,100名符合条件的儿童在印度新德里的一家三级保健教学医院登记。所有患者均行神经学检查,重点检查周围神经病变,然后行神经造影。年龄划分的截止值在正常的2.5 SD以外视为异常。结果:所有患儿均无周围神经病变的临床特征,但77%的患儿有NCS异常。其中,33%为纯运动神经改变,7%为纯感觉神经改变,1%为异常F反应,26%为混合性神经改变。这些变化与慢性贫血和铁螯合持续时间显著相关,但与其他因素无关。结论:输血依赖型地中海贫血患儿没有表现出任何神经系统体征或症状,然而,在平均血红蛋白(Hb)水平低于9.5 g/dl时观察到异常NCS并不罕见。进一步的全面病例对照研究是必要的,以确定更具体的Hb目标范围9.5至10.5 g/dl是否合适,并调查螯合治疗对这些变化的潜在影响。
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