Characteristics of Moyamoya Disease Based on National Registry Data in Japan.

Background and Purpose- A public registration system for intractable diseases was started in Japan in 1972 to investigate the etiology and pathogenesis of intractable diseases while reducing out-of-pocket medical expenses on patients. The goal of this study was to investigate the epidemiology and clinical characteristics of Moyamoya disease using data from applications submitted to this system between 2004 and 2008. Methods- In addition to demographic factors such as onset age and family history, we evaluated clinical presentation type, imaging findings, clinical symptoms, and functioning in activities of daily living (ADL). Results- Of 3859 cases for which applications were submitted, 2545 were confirmed to meet the diagnostic criteria after data cleansing. Onset age showed a bimodal distribution, and Moyamoya disease had a higher incidence in women than in men. The presence of occlusion and infarction in the proximal region of the anterior cerebral artery was more frequent in pediatric cases than adult cases. Our findings also indicated that 23% of patients required assistance with ADL. Cerebral infarction (odds ratio [OR], 12.5; 95% CI, 3.55-44.66), seizure (OR, 7.44; 95% CI, 1.29-42.96), and sensory disorders (OR, 5.23; 95% CI, 1.15-23.75) were identified as significant predictors of impaired ADL in pediatric cases 3 years after the initial application. Moderate ADL function (OR, 11.59; 95% CI, 5.29-25.39) and intellectual disabilities (OR, 4.38; 95% CI, 1.58-12.17) at the time of the application were identified as significant prognostic factors in adults. Conclusions- The results of this study indicated that characteristics of Moyamoya disease such as onset type, symptoms, and imaging abnormalities differ with onset age. Prognostic analyses suggested that pediatric cases with good ADL but with infarct type onset, seizure, or sensory disorders might have a subsequent decline in ADL.

Disaster Vulnerability of Hospitals: A Nationwide Surveillance in Japan.

OBJECTIVE: Hospital preparedness against disasters is key to achieving disaster mitigation for health. To gain a holistic view of hospitals in Japan, one of the most disaster-prone countries, a nationwide surveillance of hospital preparedness was conducted. METHODS: A cross-sectional, paper-based interview was conducted that targeted all of the 8701 registered hospitals in Japan. Preparedness was assessed with regard to local hazards, compliance to building code, and preparation of resources such as electricity, water, communication tools, and transportation tools. RESULTS: Answers were obtained from 6122 hospitals (response rate: 70.3%), among which 20.5% were public (national or city-run) hospitals and others were private. Eight percent were the hospitals assigned as disaster-base hospitals and the others were non-disaster-base hospitals. Overall compliance to building code, power generators, water tanks, emergency communication tools, and helicopter platforms was 90%, 84%, 95%, 43%, and 22%, respectively. CONCLUSION: Major vulnerabilities in logistics in mega-cities and stockpiles required for chronic care emerged from the results of this nationwide surveillance of hospitals in Japan. To conduct further intensive surveillance to meet community health needs, appropriate sampling methods should be established on the basis of this preliminary study. Holistic vulnerability analysis of community hospitals will lead to more robust disaster mitigation at the local level.