Nation-wide epidemiological study of Japanese patients with rare viral myelopathy using novel registration system (HAM-net).
Nation-wide epidemiological study of Japanese patients with rare viral myelopathy using novel registration system (HAM-net).
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DOI：
10.1186/s13023-016-0451-x
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发表时间：
2016-05-25
影响因子：
3.7
通讯作者：
Yamano Y
中科院分区：
文献类型：
--
作者：
Coler-Reilly AL;Yagishita N;Suzuki H;Sato T;Araya N;Inoue E;Takata A;Yamano Y
在日本，至少有100万人感染了人类T淋巴细胞病毒1型(HTLV-1)，其中一小部分人患有HTLV-1相关性脊髓病/热带痉挛麻痹(HAM/TSP)或成人T细胞白血病/淋巴瘤(ATLL)。HAM/TSP患者的脊髓病症状逐渐恶化，如运动障碍和膀胱功能障碍，可能需要坐轮椅，甚至卧床不起。为了更多地了解这种罕见的、使人衰弱的疾病，我们于2012年3月建立了全国注册系统“Ham-net”。我们使用登记表和每年一次的电话采访，不断地从登记的患者那里获得详细的数据。在这项回顾研究中，我们描述了来自日本各地的383名注册患者的人口统计学和临床病史。患者被诊断的年龄中值为53岁，在45岁起病很久之后。大多数人(55.3%)来自最南端的九州和冲绳。主要首发症状为行走困难(81.9%)、排尿功能障碍(38.5%)、下肢感觉障碍(13.9%)。许多患者报告说经常出现腿部麻木和腿部疼痛，绝大多数患者需要对尿路症状和便秘进行医疗干预。从出现运动症状到OSAME运动障碍评分(OMDS)5分(需要单侧支持)的中位数为8年，从OMDS 6分(需要双边支持)到OMDS 9分(无法行走)的中位数为12.5年。健康评估问卷-残疾指数(HAQ-DI)与活动有关的任务，而不是手部运动，对于HAM/TSP患者来说非常困难，并且与OMD有很好的相关性。MOS 36项简明健康调查(SF-36)的得分表明，HAM/TSP患者的身体功能严重受损。有输血史的患者(19.1%)年龄较大，残疾程度较重，HAQ-DI评分较高。有HAM/TSP家族史的患者(8.4%)较年轻，且由于其病程较长，症状相对较轻；许多(15.6%)患者还有ATLL的亲属。Ham-Net国家登记系统是收集散布在日本各地的Ham/TSP患者个人和临床数据的有效工具。我们希望在未来使用Ham-Net进行许多回顾性和前瞻性的流行病学研究。
At least one million people are infected with human T-lymphotropic virus type 1 (HTLV-1) in Japan, a small percentage of whom develop HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) or adult T-cell leukemia/lymphoma (ATLL). Patients with HAM/TSP suffer from progressively worsening myelopathic symptoms, such as motor disability and bladder dysfunction, and may become wheelchair-bound or even bedridden. To learn more about this rare, debilitating disease, we established the national registration system “HAM-net” in March 2012. We continuously obtain detailed data from enrolled patients using the registration forms and an annual telephone interview. In this retrospective study, we describe the demographics and clinical histories of 383 registered patients from all over Japan. Patients were diagnosed at a median of 53 years old, long after disease onset at 45. Most (55.3 %) were originally from the southernmost regions, Kyushu and Okinawa. The main initial symptoms were difficulty walking (81.9 %), urinary dysfunction (38.5 %), and lower limb sensory disturbances (13.9 %). Many patients reported frequent leg numbness and leg pain, and the vast majority required medical intervention for urinary symptoms and constipation. A median of 8 years elapsed from the onset of motor symptoms to Osame Motor Disability Score (OMDS) 5 (requiring unilateral support), 12.5 years to OMDS 6 (requiring bilateral support), and 18 years to OMDS 9 (unable to walk). Health Assessment Questionnaire - Disability Index (HAQ-DI) tasks related to mobility, as opposed to hand motions, were very difficult for HAM/TSP patients and well-correlated with OMDS. Scores on the MOS 36-Item Short-Form Health Survey (SF-36) indicated that physical functioning was severely impaired in HAM/TSP patients. Patients with a history of blood transfusion (19.1 %) were older and suffered from more severe disability as indicated by their high HAQ-DI scores. Patients with a family history of HAM/TSP (8.4 %) were younger and had relatively mild symptoms given their long disease durations; many (15.6 %) also had a relative with ATLL. The HAM-net national registration system has been an effective tool for gathering personal and clinical data from HAM/TSP patients scattered throughout Japan. We expect to conduct many retrospective and prospective epidemiological studies using HAM-net in the future.