Suppr超能文献

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。

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

作者信息

Rathore Aniruddha, Dhankar Mukesh, Mukherjee Sharmila B, Sharma Suvasini, Shukla Shailaja, Mandal Piali

机构信息

Department of Pediatrics, Lady Hardinge Medical College, New Delhi, India.

出版信息

J Family Med Prim Care. 2024 Dec;13(12):5847-5852. doi: 10.4103/jfmpc.jfmpc_289_24. Epub 2024 Dec 9.

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.

摘要

背景

我们的研究旨在确定5至18岁输血依赖型地中海贫血患儿周围神经病变的患病率(采用神经传导研究(NCS)),并研究其与慢性贫血、铁蛋白水平、螯合状态、年输血需求量、血清维生素B12缺乏及叶酸水平之间的相关性。

方法

在这项基于医院的横断面研究中,印度新德里一家三级护理教学医院招募了100名符合条件的儿童。对所有患者进行了侧重于周围神经病变的神经系统检查,随后进行NCS。将正常范围外2.5个标准差的年龄相关临界值视为异常。

结果

尽管77%的儿童NCS存在异常,但无一例有周围神经病变的临床特征。其中,33%有单纯运动神经改变,7%有单纯感觉神经改变,1%有异常F波反应,26%有混合神经改变。这些改变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

然而,在未表现出任何神经体征或症状的输血依赖型地中海贫血患儿中,平均血红蛋白(Hb)水平低于9.5 g/dl时出现异常NCS并不罕见。需要进一步开展全面的病例对照研究,以确定9.5至10.5 g/dl这一更具体的Hb目标范围是否合适,并研究螯合疗法对这些改变的潜在影响。

相似文献

本文引用的文献

3
Polyneuropathy and myopathy in beta-thalassemia major patients.重型β地中海贫血患者的多发性神经病和肌病。
Ann Hematol. 2018 May;97(5):899-904. doi: 10.1007/s00277-018-3251-7. Epub 2018 Feb 9.
5
Beta-thalassemia mutations in western India.印度西部的β地中海贫血突变
Indian J Pediatr. 2008 Jun;75(6):567-70. doi: 10.1007/s12098-008-0109-3. Epub 2008 Aug 31.
6
Peripheral neuropathy in thalassaemia.地中海贫血中的周围神经病变
Ann Saudi Med. 2006 Sep-Oct;26(5):358-63. doi: 10.5144/0256-4947.2006.358.
8
Long term audiological evaluation of beta-thalassemic patients.
Acta Otorhinolaryngol Belg. 2004;58(2):113-7.
9
Axonal sensorimotor neuropathy in patients with beta-thalassaemia.β地中海贫血患者的轴索性感觉运动神经病
J Neurol Neurosurg Psychiatry. 2004 Oct;75(10):1483-6. doi: 10.1136/jnnp.2003.024794.
10

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验