Abstract：
OBJECTIVE: The aim of this study was to estimate the number of patients in Japan who had visited an ophthalmologist for macular dystrophy of various types, including Best vitelliform macular dystrophy (BVMD), Stargardt disease, occult macular dystrophy (OMD), cone (-rod) dystrophy, X-linked retinoschisis (XLRS), and central areolar choroid dystrophy (CACD).
METHODS: Nationwide epidemiologic survey METHODS: Questionnaires were distributed to 965 major facilities, including all the university hospitals in Japan. The aim of the questionnaire was to determine the number of patients with each type of macular dystrophy who had visited an outpatient clinic during the past 5 years (January 2015 to December 2019).
RESULTS: Over 70% of the patients were diagnosed and followed up at university hospitals. The estimated annual number of newly diagnosed cases was as follows: 55.3 for BVMD, 36.7 for Stargardt disease, 35.8 for OMD, 160.6 for cone (-rod) dystrophy, 31.0 for XLRS, 29.8 for CACD, and 174.1 for other types of macular dystrophy. The total number of patients with macular dystrophy diagnosed and followed at major institutions was estimated to be 6651.
CONCLUSIONS: This was the first nationwide survey of macular dystrophy in Japan and provided an approximate number of affected patients. The diagnosis of macular dystrophy is primarily carried out at facilities with affiliated specialists, such as university hospitals. By examining the incidence of multiple diseases simultaneously, we were able to compare the incidence of each type of macular dystrophy.
METHODS: Nationwide epidemiologic survey METHODS: Questionnaires were distributed to 965 major facilities, including all the university hospitals in Japan. The aim of the questionnaire was to determine the number of patients with each type of macular dystrophy who had visited an outpatient clinic during the past 5 years (January 2015 to December 2019).
RESULTS: Over 70% of the patients were diagnosed and followed up at university hospitals. The estimated annual number of newly diagnosed cases was as follows: 55.3 for BVMD, 36.7 for Stargardt disease, 35.8 for OMD, 160.6 for cone (-rod) dystrophy, 31.0 for XLRS, 29.8 for CACD, and 174.1 for other types of macular dystrophy. The total number of patients with macular dystrophy diagnosed and followed at major institutions was estimated to be 6651.
CONCLUSIONS: This was the first nationwide survey of macular dystrophy in Japan and provided an approximate number of affected patients. The diagnosis of macular dystrophy is primarily carried out at facilities with affiliated specialists, such as university hospitals. By examining the incidence of multiple diseases simultaneously, we were able to compare the incidence of each type of macular dystrophy.
摘要：
目的：这项研究的目的是估计日本因各种黄斑营养不良而就诊眼科医生的患者人数，包括最佳卵黄样黄斑营养不良（BVMD），Stargardt病,隐匿性黄斑营养不良（OMD），锥（-杆）营养不良，X-连锁视网膜裂（XLRS），和中央乳晕脉络膜营养不良（CACD）。
方法：全国流行病学调查方法：向965个主要机构分发问卷，包括日本所有的大学医院.问卷的目的是确定在过去5年中（2015年1月至2019年12月）就诊于门诊的每种类型的黄斑营养不良患者的数量。
结果：超过70%的患者在大学医院得到诊断和随访。估计每年新诊断病例数如下:BVMD为55.3例,Stargardt病的36.7，35.8对于OMD，160.6用于圆锥（-杆）营养不良，XLRS为31.0，29.8对于CACD，和174.1其他类型的黄斑营养不良。在主要机构诊断和随访的黄斑营养不良患者总数估计为6651。
结论：这是日本首次在全国范围内对黄斑营养不良进行的调查，并提供了大约数量的受影响患者。黄斑营养不良的诊断主要在附属专家的机构进行，比如大学医院。通过同时检查多种疾病的发病率，我们能够比较每种类型的黄斑营养不良的发生率。
方法：全国流行病学调查方法：向965个主要机构分发问卷，包括日本所有的大学医院.问卷的目的是确定在过去5年中（2015年1月至2019年12月）就诊于门诊的每种类型的黄斑营养不良患者的数量。
结果：超过70%的患者在大学医院得到诊断和随访。估计每年新诊断病例数如下:BVMD为55.3例,Stargardt病的36.7，35.8对于OMD，160.6用于圆锥（-杆）营养不良，XLRS为31.0，29.8对于CACD，和174.1其他类型的黄斑营养不良。在主要机构诊断和随访的黄斑营养不良患者总数估计为6651。
结论：这是日本首次在全国范围内对黄斑营养不良进行的调查，并提供了大约数量的受影响患者。黄斑营养不良的诊断主要在附属专家的机构进行，比如大学医院。通过同时检查多种疾病的发病率，我们能够比较每种类型的黄斑营养不良的发生率。
