Epidemiology of distal radius fracture: a regional population-based study in Japan.

BACKGROUND: Distal radius fracture (DRF) is very common worldwide. In particular, aging countries have numerous patients with DRF, resulting in an urgent need for active preventive measures. As few epidemiological studies have investigated DRF in Japan, we aimed to identify the epidemiological characteristics of patients of all ages with DRF in Japan. METHODS: This descriptive epidemiologic study analyzed data obtained from clinical information of patients diagnosed with DRF from January 1, 2011, to December 31, 2020, at a prefectural hospital in Hokkaido, Japan. We calculated the crude and age-adjusted annual incidences of DRF and described the age-specific incidence, injury characteristics (injury location and cause, seasonal differences, and fracture classification), and 1- and 5-year mortality rates. RESULTS: A total of 258 patients with DRF were identified, of which 190 (73.6%) were female and the mean age (standard deviation) was 67.0 (21.5) years. The crude annual incidence of DRF ranged from 158.0 to 272.6 per 100,000 population/year, and the age-adjusted incidence among female patients demonstrated a significant decreasing trend during 2011-2020 (Poisson regression analysis; p = 0.043). The age-specific incidence differed by sex, with peaks at 10-14 years for males and 75-79 years for females. The most common cause of injury was a simple fall in patients > 15 year of age and sports injuries in patients ≤ 15 years of age. DRFs were most frequently sustained outdoors and were more common in the winter season. In patients > 15 years of age, the proportions of AO/OTA fracture types A, B, and C were 78.7% (184/234), 1.7% (4/234), and 19.6% (46/234), respectively, and 29.1% (68/234) of patients received surgical treatment for DRF. The 1- and 5-year mortality rates were 2.8% and 11.9%, respectively. CONCLUSIONS: Our findings were mostly consistent with previous global studies. Although the crude annual incidence of DRF was relatively high because of recent population aging, the age-adjusted annual incidence among female patients showed a significant decreasing trend during this decade.

Postoperative clinical outcomes for kinematically, restricted kinematically, or mechanically aligned total knee arthroplasty: a systematic review and network meta-analysis of randomized controlled trials.

BACKGROUND: Mechanically aligned total knee arthroplasty (MATKA) is a well-established procedure. Kinematically aligned TKA (KATKA) has been proposed to restore and preserve pre-arthritic knee anatomy. However, normal knee anatomy varies widely, and there have been concerns regarding restoring unusual anatomy. Accordingly, restricted KATKA (rKATKA) was introduced to reproduce constitutional knee anatomy within a safe range. This network meta-analysis (NMA) aimed to evaluate the clinical and radiological outcomes of the surgeries. METHODS: We performed a database search on August 20, 2022, which included randomized controlled trials (RCTs) comparing any two of the three surgical TKA techniques for knee osteoarthritis. We conducted a random-effects NMA within the frequentist framework and evaluated confidence in each outcome using the Confidence in Network Meta-Analysis tool. RESULTS: Ten RCTs with 1,008 knees and a median follow-up period of 1.5 years were included. The three methods might result in little to no difference in range of motion (ROM) between methods. In patient-reported outcome measures (PROMs), the KATKA might result in a slight improvement compared with the MATKA (standardized mean difference, 0.47; 95% confidence interval [CI], 0.16-0.78; very low confidence). There was little to no difference in revision risk between MATKA and KATKA. KATKA and rKATKA showed a slight valgus femoral component (mean difference [MD], -1.35; 95% CI, -1.95-[-0.75]; very low confidence; and MD, -1.72; 95% CI, -2.63-[-0.81]; very low confidence, respectively) and a slight varus tibial component (MD, 2.23; 95% CI, 1.22-3.24; very low confidence; and MD, 1.25; 95% CI, 0.01-2.49; very low confidence, respectively) compared with MATKA. Tibial component inclination and hip-knee-ankle angle might result in little to no difference between the three procedures. CONCLUSIONS: KATKA and rKATKA showed similar ROM and PROMs and a slight variation in the coronal component alignment compared with MATKA. KATKA and rKATKA are acceptable methods in short- to mid-term follow-up periods. However, long-term clinical results in patients with severe varus deformity are still lacking. Surgeons should choose surgical procedures carefully. Further trials are warranted to evaluate the efficacy, safety, and subsequent revision risk.

Differences in Sensitivity Between the Japanese and Z Score Criteria for Detecting Coronary Artery Abnormalities Resulting from Kawasaki Disease.

No studies have assessed differences between the Japanese and Z score criteria in the echocardiographic detection sensitivity of coronary artery (CA) abnormalities using large-scale data containing samples from multiple facilities engaged in daily clinical practices of Kawasaki disease (KD). We analyzed data from the 25th Japanese nationwide KD survey, which identified 30,415 patients from 1357 hospitals throughout Japan during 2017-2018. Hospitals were classified according to their use of Z score criteria. We assessed differences in hospital and patient background factors and compared the prevalence of CA abnormalities among groups using the Z score criteria. Multivariable logistic regression analyses were performed to evaluate differences in the detection sensitivity for CA abnormalities. The Z score criteria were more likely to be utilized in larger hospitals with more pediatricians and cardiologists. Even after controlling for potential confounders, detection sensitivities by the Z score criteria were significantly higher than by the Japanese criteria in patients with CA dilatations (adjusted odds ratio (95% confidence interval) 1.77 (1.56-2.01)) and aneurysms (1.62 (1.17-2.24)). No significant difference was found in patients with giant CA aneurysms. Compared with the Japanese criteria, the Z score criteria were significantly more sensitive for detecting patients with CA dilatations regardless of age, and for those with CA aneurysms only in patients aged ≤ 1 year. Our results indicate that differences in the detection sensitivity for CA abnormalities between the Z score and the Japanese criteria were dependent on the CA size and patient age.

AO/OTA 31A3 fractures and postoperative complications in older patients.

BACKGROUND: AO/OTA 31A3 fractures (A3 fractures) have risk for postoperative complications with major impact on morbidity and mortality. For older patients, limited information is available for factors associated with postoperative complications. We aimed to assess factors associated with postoperative complications after surgery using cephalomedullary nails. METHODS: A retrospective cohort study was conducted using the information on patients aged ≥65 years who underwent surgery using cephalomedullary nails for trochanteric fractures due to low-energy trauma in three hospitals. Postoperative complications were diagnosed when patients were identified as nonunion, cutout of lag screw, or nail breakage. First, we compared differences including age, sex, body mass index, American Society of Anesthesiologists physical status classification system, preoperative waking ability, fracture type, nail length, neck shaft angle, reduction method, reduction quality and tip apex distance between patients with and without postoperative complications. Second, multivariable logistic regression analysis was employed to assess factors associated with postoperative complications resulting from A3 fractures. RESULTS: Among 120 patients with A3 fractures, postoperative complications were identified in 12 patients (10.0%). Postoperative complications were significantly more likely to develop among patients with poor reduction quality (adjusted odds ratio [95% confidence interval], 35.0 [4.43-275.9]) and a tip-apex distance ≥25 mm (16.4 [1.92-140.3]). CONCLUSIONS: These findings suggest that surgeons should aim to perform appropriate postoperative reduction and to prevent postoperative complications when using a cephalomedullary nail for A3 fractures among older patients.

Combination of radiographic apparent bone gap and nonunion risk determination score improves accuracy of prediction of tibial shaft delayed union.

BACKGROUND: There is a need for a novel therapeutic strategy for an earlier prediction of long bone union failure as compared to previous methodologies. This study aimed to determine whether a combination of two diagnostic tools would result in a more accurate diagnosis of delayed union. METHODS: The inclusion criteria were as follows: patients with tibial shaft fracture who underwent treatment with intramedullary nailing (IMN) as definitive internal fixation (IF). The study included a total of 114 patients with 116 tibial shaft fractures treated with IMN as definitive IF. Radiographic apparent bone gap (RABG) and nonunion risk determination score (NURDS) can be used to predict nonunion. However, this study aimed to determine whether combination of RABG and NURDS could help deduce a more accurate prediction of delayed union. RESULTS: The union rate was found to be 85% (99 fractures), the delayed union rate was found to be 15% (17 fractures), and the rate of nonunion requiring additional surgical intervention was estimated to be 4% (5 out of the 17 delayed union cases). Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of RABG were found to be 82.3%, 76.0%, 36.8%, and 96.2%, respectively, when an RABG cutoff value of 5.0 mm was applied to our patient cohort. The sensitivity, specificity, PPV, and NPV of NURDS were found to be 47.1%, 82.0%, 30.8%, and 90.1%, respectively, when a NURDS cutoff value of 8.0% was applied to our patient cohort. When RABG and NURDS were above their respective cutoff values, the sensitivity and PPV were estimated to be 90.0% and 56.3%, respectively. When RABG and NURDS were below their respective cutoff values, the specificity and NPV were estimated to be 90.1% and 98.5%, respectively. CONCLUSIONS: The combination of RABG and NURDS evaluation immediately after surgery helps surgeons identify patients who are at a high risk of delayed union, facilitating careful monitoring of these patients and consideration of additional treatments.

Regional Differences in Kawasaki Disease Incidence Reduction Before and After the Onset of the Coronavirus Disease 2019 Pandemic.

OBJECTIVE: To assess regional differences in reduction of the incidence of Kawasaki disease during the mitigation period for the coronavirus disease 2019 pandemic, with a hypothesis that more sparsely populated regions have fewer opportunities for human-to-human contact, resulting in a greater reduction in the incidence of Kawasaki disease. STUDY DESIGN: A retrospective ecological study was conducted using data from patients hospitalized for Kawasaki disease as well as infectious diseases surveillance reports in Shiga Prefecture, Japan, during 2015-2020. We defined the periods before and after the onset of pandemic as January 2015-March 2020 and as April 2020-December 2020, respectively. We compared the reductions in the incidence of Kawasaki disease among 6 administrative regions in the prefecture according to the density of the populations. RESULTS: A total of 1290 patients with Kawasaki disease were identified. The incidence of Kawasaki disease (per 100 000 person-years) was significantly reduced after the coronavirus disease 2019 pandemic onset (period before pandemic onset, 105.6 [95% CI 99.8-111.8]; period after pandemic onset, 68.6 [95% CI 56.7-83.0]). During the period after pandemic onset, the incidence of Kawasaki disease was significantly reduced in May, compared with the corresponding period in previous years. The number of patients aged 2-4 years was significantly reduced after the pandemic onset. Notably, greater reductions in the incidence of Kawasaki disease were found in regions with lower population densities. CONCLUSIONS: Assuming that there were fewer opportunities for human-to-human contact in more sparsely populated regions during the pandemic mitigation period, our findings support the hypothesis that human-to-human contact may be associated with development of Kawasaki disease.

Methionine homozygosity for PRNP polymorphism and susceptibility to human prion diseases.

BACKGROUND: No studies have assessed the independent association of methionine homozygosity at codon 129 with the susceptibility to prion diseases, controlling for the effects of the codon 219 polymorphisms and other potential confounders, using a large-scale population-based dataset. METHODS: We conducted a case-control study using a Japanese nationwide surveillance database for prion diseases. The main exposure was methionine homozygosity at codon 129, and the outcome was development of prion diseases. Multivariable logistic regression models were employed for specific disease subtypes (sporadic Creutzfeldt-Jakob disease (CJD), genetic CJD and Gerstmann-Sträussler-Scheinker disease (GSS)). RESULTS: Of 5461 patients registered in the database, 2440 cases and 796 controls remained for the analysis. The cases comprised 1676 patients with sporadic CJD (69%), 649 with genetic CJD (27%) and 115 with GSS (5%). For patients with methionine homozygosity, potential risk for occurring prion diseases: adjusted OR (95% CI) was 2.21 (1.46 to 3.34) in sporadic CJD, 0.47 (0.32 to 0.68) in genetic CJD and 0.3 (0.17 to 0.55) in GSS. Among patients with specific prion protein abnormalities, the potential risk was 0.27 (0.17 to 0.41) in genetic CJD with 180 Val/Ile, 1.66 (0.65 to 5.58) in genetic CJD with 200 Glu/Lys, 3.97 (1.2 to 24.62) in genetic CJD with 232 Met/Arg and 0.71 (0.34 to 1.67) in GSS with 102 Pro/Leu. CONCLUSIONS: Methionine homozygosity at codon 129 was predisposing to sporadic CJD, but protective against genetic CJD and GSS, after adjustment for codon 219 polymorphism effect. However, the impacts differed completely among patients with specific prion protein abnormalities.

First-line corticosteroids for Kawasaki disease: Pulse versus multiple dose.

BACKGROUND: Kawasaki disease (KD) can result in severe coronary artery abnormalities (CAAs). Corticosteroids added to initial standard intravenous immunoglobulin (IVIG) treatment may decrease the risk for these complications. Different corticosteroid regimens (single-day high dose pulse vs multiple lower doses) may contribute to the discrepant results of prior studies. METHODS: Using data from the 22nd, 23rd , and 24th Japanese nationwide KD surveys (2011-2016), we identified KD patients who did not have CAAs at first presentation and who were treated with either pulse or multiple-dose corticosteroids as part of their initial treatment. Occurrence of subsequent CAAs and treatment failure were compared between the treatment regimens and adjusted odds ratios were calculated controlling for sex, age group, illness day at first treatment, survey, and recurrent KD. RESULTS: There were 782 KD patients who received pulse corticosteroid treatment and 4,817 who received multiple dose treatment. Patients receiving multiple dose treatment were less likely to develop CAAs (5.5% vs 8.3%, OR 0.64; 95% CI: 0.48-0.85) or treatment failure (21.4% vs 41.6%; OR: 0.38; 95% CI: 0.33-0.45). Adjusted analyses showed similar protective effects of multiple-dose treatment against CAAs (OR: 0.67, 95% CI: 0.51-0.90) and treatment failure (OR: 0.39, 95% CI: 0.33-0.46). CONCLUSIONS: Multiple-dose corticosteroid combination treatment resulted in substantially improved outcomes in KD patients compared to pulse treatment. For patients who may be at elevated risk of treatment failure or CAA, use of multiple-dose corticosteroids in conjunction with IVIG is likely to provide considerable clinical benefit.

Follow up of Kawasaki disease based on nationwide survey data in Japan: Mortality among those with a history of Kawasaki disease in Japan: Results of a 30-year follow up: Mortality among those with a history of Kawasaki disease in Japan: Results of a 30-year follow up.

BACKGROUND: The long-term prognosis of those with a history of Kawasaki disease (KD) is still unknown. METHODS: Using a permanent registry system in Japan (koseki), 6,576 persons with a history of KD were followed up. The average follow-up period was 30 years. The endpoint was death. RESULTS: With a 99.5% follow-up rate, 68 deaths (48 males and 20 females) were observed. The overall standardized mortality ratio, of which reference was vital statistics in Japan, was not elevated. However, the observation according to the presence or absence of cardiac sequelae showed that the standardized mortality ratio for those with cardiac sequelae significantly elevated. Nine persons, all of whom were males, died of KD (including those cases where KD was suspected), but all deaths occurred in individuals who were under 30 years of age. CONCLUSIONS: This study revealed the long-term prognosis for KD, but almost all participants were younger than 40 years. Continuing follow up of this cohort is required to clarify whether a history of KD relates to the development of atherosclerosis when participants become middle aged or older.

Kawasaki Disease and Pediatric Infectious Diseases During the Coronavirus Disease 2019 Pandemic.

OBJECTIVE: To assess the epidemiologic association between Kawasaki disease and common pediatric infectious diseases (PIDs) identified during the coronavirus disease 2019 (COVID-19) pandemic period to confirm whether the infection-triggered theory is a plausible hypothesis for the pathogenesis of Kawasaki disease. STUDY DESIGN: A retrospective epidemiologic study was conducted using datasets obtained from Web-based surveillance of Kawasaki disease and PIDs in Japan. We compared weekly numbers of patients who developed Kawasaki disease and specific PIDs between 2020 and 2017-2019 and evaluated the association between the percent reduction in the number of patients with these diseases. RESULTS: A total of 868 patients developed Kawasaki disease in 2020. During the social distancing period in 2020, the number of patients with Kawasaki disease was approximately 35% lower than in 2017-2019. Time from the onset of Kawasaki disease until the first hospital visit did not differ significantly among the examined years. The proportion of older children with Kawasaki disease decreased more than that of infants with Kawasaki disease (age <1 year), resulting in a significant difference in the proportion of infant patients between 2020 and 2017-2019 (24% vs 19%; P < .01). The number of patients with incomplete Kawasaki disease was unchanged from that of previous years. The weekly percent reduction in patient numbers differed between Kawasaki disease and PIDs during 2020, with no strong correlation between the 2 diseases. CONCLUSIONS: Our data indicate that parents of patients with Kawasaki disease did not avoid hospital visits during the COVID-19 pandemic period. The findings indicate the possibility that triggering Kawasaki disease might be associated with presently unidentified respiratory pathogen(s) that potentially might be acquired from both within and outside the household.

Prevention paradox between stroke and multiple potential risk factors using data from a population-based cohort study.

Previous studies have found the prevention paradox in the association between stroke events and a single specific risk factor, indicating that a population-based strategy may be more effective than a high-risk-based strategy for prevention. We tested the hypothesis that the prevention paradox does not apply when focusing on multiple potential risk factors simultaneously. The study cohort included 9051 individuals from Japan aged 40-89 years. The time-dependent Cox proportional-hazards models were used to identify the primary risk factor associated with stroke onset. We classified participants based on risk factors in two distinct ways: 1) classifying the high-risk group participants according to a single specific risk factor that had a large association with stroke in both sexes and all ages and 2) classifying the high-risk group participants according to 1-3 risk factor(s) including hypertension, hyperglycemia, and/or dyslipidemia. Then, we compared the proportions of the total number of participants who developed stroke in both groups to assess the prevention paradox. We found that hypertension was a primary risk factor for stroke incidence, regardless of sex and age. The percentage of patients with a single specific risk of and developed stroke was 46%-63%, while the percentage of patients with 1-3 risk factor(s) was 71-83%. This finding leads to the conclusion that the prevention paradox does not hold when multiple stroke risk factors were associated, suggesting that a high-risk-based strategy that focuses on patients with multiple risk factors may be more effective in preventing strokes.

Improvement of hand hygiene adherence among staff in long-term care facilities for elderly in Japan.

INTRODUCTION: Hand hygiene is crucial for infection control in long-term care facilities for elderly (LTCFEs), because it can be easily implemented in the low-resource settings of LTCFEs. This study investigated the actual status of hand hygiene adherence in LTCFEs, identified the factors inhibiting its appropriate implementation, and evaluated the effectiveness of a hand hygiene promotion program. METHODS: In this before-and-after study, participants were staff members (n = 142) at two LTCFEs in Gunma Prefecture, Japan. We modified the World Health Organization's "five moments for hand hygiene" and assessed participants' hand hygiene adherence rates in four situations: (1) Before touching around a resident's mucous membrane area; (2) Before medical practice or clean/aseptic procedures; (3) After body fluid exposure/risk or after touching around a resident's mucous membrane area; and (4) After touching a resident's contaminated environments. The study was divided into four phases. In Phase 1, participants self-assessed their hand hygiene adherence using a questionnaire. In Phase 2, we objectively assessed participants' pre-intervention adherence rates. In Phase 3, an intervention comprising various hand hygiene promotion measures, such as education and hands-on training on hand hygiene practices and timings, was implemented. In Phase 4, participants' post-intervention adherence rates were objectively measured. RESULTS: Although most participants reported high hand hygiene adherence rate in the self-assessment (93.1%), the pre-intervention evaluation revealed otherwise (16.8%). Participants' post-intervention adherence rates increased for all four situations (77.3%). CONCLUSION: The intervention program helped increase participants' hand hygiene adherence rates, indicating its effectiveness. Similar interventions in other LTCFEs may also improve adherence rates.

Epidemiology and Risk Factors for Giant Coronary Artery Aneurysms Identified After Acute Kawasaki Disease.

A giant coronary artery (CA) aneurysm is a potentially fatal cardiac complication resulting from Kawasaki disease (KD). We aimed to identify epidemiologic characteristics and potential risk factors associated with giant CA aneurysms identified after acute KD. We analyzed 90,252 patients diagnosed with KD from 2011 to 2018, using data obtained in nationwide KD surveys conducted in Japan. Multivariable logistic regression analyses were performed to evaluate potential risk factors associated with subsequent giant CA aneurysm complications (defined as lumen size ≥ 8 mm), adjusting for all potential factors. Giant CA aneurysms were identified in 144 patients (0.16%) after acute KD. The annual prevalence ranged from 0.07 to 0.20% during the study period. In the multivariate analyses, male sex (adjusted odds ratio 2.09 [95% confidence interval 1.41-3.11], recurrent KD (1.90 [1.09-3.33]), IVIG administration at 1-4 days of illness (1.49 [1.04-2.15]) and ≥ 8 days after KD onset (2.52 [1.38-4.60]; reference, 5-7 days), detection of CA dilatations and aneurysms at initial echocardiography (4.17 [1.85-5.41] and 46.5 [28.8-74.8], respectively), and resistance to IVIG treatment (6.09 [4.23-8.75]) were significantly associated with giant CA aneurysm complications identified after acute KD. The annual prevalence of giant CA aneurysms identified after acute KD did not increase during the study period. Patients with larger CA abnormalities detected at initial echocardiography were independently associated with progression to giant CA aneurysm complications after acute KD regardless of the number of days from onset at treatment initiation.

Characterization of Sporadic Creutzfeldt-Jakob Disease and History of Neurosurgery to Identify Potential Iatrogenic Cases.

We previously reported a phenotype of Creutzfeldt-Jakob disease (CJD), CJD-MMiK, that could help identify iatrogenic CJD. To find cases mimicking CJD-MMiK, we investigated clinical features and pathology of 1,155 patients with diagnosed sporadic CJD or unclassified CJD with and without history of neurosurgery. Patients with history of neurosurgery more frequently had an absence of periodic sharp-wave complexes on electroencephalogram than patients without a history of neurosurgery. Among 27 patients with history of neurosurgery, 5 had no periodic sharp-wave complexes on electroencephalogram. We confirmed 1 case of CJD-MMiK and suspected another. Both had methionine homozygosity at codon 129 of the prion protein gene and hyperintensity lesions in the thalamus on magnetic resonance images of the brain, which might be a clinical marker of CJD-MMiK. A subgroup with a history of neurosurgery and clinical features mimicking dura mater graft-associated CJD might have been infected during neurosurgery and had symptoms develop after many years.

Outcomes in Kawasaki disease patients with coronary artery abnormalities at admission.

BACKGROUND: Previous studies demonstrated that coronary artery lesions (CALs) resulting from Kawasaki disease (KD) can improve over time. However, limited information is available on sub-acute outcomes of CALs detected at admission during KD illness. METHODS: The nationwide Japanese KD survey contained substantial information on KD patients with CALs detected at admission and who received standard IVIG treatment within 10 days of disease onset. Coronary outcomes were evaluated by changes in CALs from admission to the first assessment at 30 days from disease onset in three categories: improved, unchanged, and progressed. Ordinal logistic regression analysis was performed to evaluate factors associated with the outcomes. RESULTS: Of 2024 patients with CALs detected at admission, improved, unchanged, and progressed outcomes were found in 1548 (76.5%), 390 (19.3%), and 86 (4.2%), respectively. Over 80% of patients with coronary artery (CA) dilatations had improved outcome. Independent factors associated with worse outcomes were larger-size CALs (adjusted ORs [95% CIs]: CA aneurysm = 5.13 [3.65-7.22] and giant CA aneurysms = 7.49 [3.56-15.72] compared with CA dilatation, respectively), age ≥ 60 months (1.45 [1.08-1.94] compared with 12-59 months), recurrent KD (1.57 [1.07-2.29]), parental history of KD (2.23 [1.02-4.85]), and delayed admission (8-10 days from disease onset: 1.76 [1.21-2.57] compared with 1-4 days). CONCLUSIONS: KD patients with larger CALs, ≥60 months old, and with recurrent status or parental history may require more rigorous treatment. In addition, delayed admission may result in worse coronary outcome, indicating that prompt diagnosis and treatment are required.

Epidemiology, Treatments, and Cardiac Complications in Patients with Kawasaki Disease: The Nationwide Survey in Japan, 2017-2018.

OBJECTIVE: To report the epidemiologic characteristics, treatments, and cardiac complications of Kawasaki disease, using data from the nationwide survey in Japan. STUDY DESIGN: The nationwide Kawasaki disease survey in Japan has been conducted biennially since 1970. The most recent survey was completed in 2019, obtaining information for patients who developed Kawasaki disease during 2017-2018. Survey respondents were hospitals specializing in pediatrics and those with ≥100 beds and a pediatric department throughout Japan, where patients with Kawasaki disease were eventually hospitalized. RESULTS: The survey identified 32 528 patients with Kawasaki disease, which consisted of 15 164 (46.6%) in 2017 and 17 364 (53.4%) in 2018. The highest annual incidence rate was recorded in 2018 (359 per 100 000 children aged 0-4 years). After 1982, patients with ≤4 principal Kawasaki disease signs gradually increased, resulting in 6847 (21.1%) patients diagnosed during 2017-2018. Among the 30 784 patients receiving initial intravenous immunoglobulin administration, 6061 (19.7%) did not respond. Within 30 days of Kawasaki disease onset, 9.0% of patients were diagnosed with cardiac complications, and 2.6% of patients developed cardiac sequelae after the acute illness. CONCLUSIONS: The annual number of patients developing Kawasaki disease in Japan increased from 1970 through 2018, whereas the proportion of patients with Kawasaki disease with cardiac complications decreased in the most recent 2 decades. Early diagnosis of Kawasaki disease as well as advances in initial treatments could explain these findings.

MM2-type sporadic Creutzfeldt-Jakob disease: new diagnostic criteria for MM2-cortical type.

OBJECTIVE: To clinically diagnose MM2-cortical (MM2C) and MM2-thalamic (MM2T)-type sporadic Creutzfeldt-Jakob disease (sCJD) at early stage with high sensitivity and specificity. METHODS: We reviewed the results of Creutzfeldt-Jakob disease Surveillance Study in Japan between April 1999 and September 2019, which included 254 patients with pathologically confirmed prion diseases, including 9 with MM2C-type sCJD (MM2C-sCJD) and 10 with MM2T-type sCJD (MM2T-sCJD), and 607 with non-prion diseases. RESULTS: According to the conventional criteria of sCJD, 4 of 9 patients with MM2C- and 7 of 10 patients with MM2T-sCJD could not be diagnosed with probable sCJD until their death. Compared with other types of sCJD, patients with MM2C-sCJD showed slower progression of the disease and cortical distribution of hyperintensity lesions on diffusion-weighted images of brain MRI. Patients with MM2T-sCJD also showed relatively slow progression and negative results for most of currently established investigations for diagnosis of sCJD. To clinically diagnose MM2C-sCJD, we propose the new criteria; diagnostic sensitivity and specificity to distinguish 'probable' MM2C-sCJD from other subtypes of sCJD, genetic or acquired prion diseases and non-prion disease controls were 77.8% and 98.5%, respectively. As for MM2T-sCJD, clinical and laboratory features are not characterised enough to develop its diagnostic criteria. CONCLUSIONS: MM2C-sCJD can be diagnosed at earlier stage using the new criteria with high sensitivity and specificity, although it is still difficult to diagnose MM2T-sCJD clinically.

Association between length of residence and prevalence of MRSA colonization among residents in geriatric long-term care facilities.

BACKGROUND: A high prevalence of methicillin-resistant Staphylococcus aureus (MRSA) colonization has been reported among residents in geriatric long-term care facilities (LTCFs). Some studies indicate that MRSA might be imported from hospitals into LTCFs via resident transfer; however, other studies report that high MRSA prevalence might be caused by cross-transmission inside LTCFs. We aimed to assess which factors have a large impact on the high MRSA prevalence among residents of geriatric LTCFs. METHODS: We conducted a cohort study among 260 residents of four geriatric LTCFs in Japan. Dividing participants into two cohorts, we separately analyzed (1) the association between prevalence of MRSA carriage and length of LTCF residence (Cohort 1: n = 204), and (2) proportion of residents identified as MRSA negative who were initially tested at admission but subsequently identified as positive in secondary testing performed at ≥2 months after their initial test (Cohort 2: n = 79). RESULTS: Among 204 residents in Cohort 1, 20 (9.8%) were identified as positive for MRSA. Compared with residents identified as MRSA negative, a larger proportion of MRSA-positive residents had shorter periods of residence from the initial admission (median length of residence: 5.5 vs. 2.8 months), although this difference was not statistically significant (p = 0.084). Among 79 residents in Cohort 2, 60 (75.9%) were identified as MRSA negative at the initial testing. Of these 60 residents, only one (1.7%) had subsequent positive conversion in secondary MRSA testing. In contrast, among 19 residents identified as MRSA positive in the initial testing, 10 (52.6%) were negative in secondary testing. CONCLUSIONS: The prevalence of MRSA was lower among residents with longer periods of LTCF residence than among those with shorter periods. Furthermore, few residents were found to become MRSA carrier after their initial admission. These findings highlight that MRSA in LTCFs might be associated with resident transfer rather than spread via cross-transmission inside LTCFs.

Cumulative incidence of Kawasaki disease with cardiac sequelae in Japan.

BACKGROUND: Some patients with Kawasaki disease (KD) develop cardiac sequelae, which increase the risk of subsequent ischemic heart events. Knowing the cumulative incidence (CI) of KD with cardiac sequelae may contribute to developing health policies to prevent subsequent ischemic events in these patients. METHODS: Study participants consisted of 254 984 patients aged 0-9 years with KD who were registered in nationwide surveys in Japan from 1991-2016. We calculated the incidence probabilities by dividing the number of patients with KD aged 0-9 years by the population used in vital statistics of each calendar year. We calculated the cumulative proportion of those not affected by KD, by multiplying each probability in patients aged from age 0-9 years. The CI of KD was obtained by subtracting this value from 1. We also calculated the number of patients in each birth cohort (BC). The same was done to calculate the CI of KD-related cardiac sequelae. RESULTS: The CIBC steadily increased from 0.005067 in males and 0.003668 in females in 1991 to 0.011431 in males and 0.0088253 in females in 2007. The CIBC of KD with cardiac sequelae decreased from 0.000478 in males and 0.000213 in females in 1997 to 0.000339 in males and 0.000169 in females in 2007. CONCLUSION: The increasing CIBC of KD indicates an increased susceptibility to KD in accordance with birth year. The decreasing CIBC of cardiac sequelae suggests the efficacy of KD treatment.

Nationwide epidemiologic survey of Kawasaki disease in Japan, 2015-2016.

BACKGROUND: Approximately 50 years have passed since Kawasaki disease (KD) was first reported. The KD nationwide survey began in 1970. Although >360 000 cases have already been reported in Japan, the cause is still unknown. In Japan, the number of patients and incidence rate of KD has continued to increase. It is necessary to examine the trend of the occurrence in the surveillance of KD. METHODS: The nationwide survey of patient incidence in 2015 and 2016 was conducted in 2017, as the 24th nationwide survey of KD. A questionnaire was sent to pediatric departments in hospitals with >100 beds and specialized pediatric hospitals, and was responded to by the attending pediatricians. RESULTS: The total number of patients in 2 years was 31 595, and the sex ratio (male/female) was 1.34. The incidence rate (/100 000 children aged 0-4 years/year) was 330.2 (371.2 in boys, 287.3 in girls) in 2015, and 309.0 (343.2 in boys, 273.2 in girls) in 2016. The number of patients by month peaked in January. The age-specific incidence rate according to sex was highest in children between 9 and 11 months of age, after which the incidence rate gradually decreased with advancing age. CONCLUSIONS: We summarize the most recent nationwide survey of KD and consider the change in the epidemiologic picture.