Search

Spinal cord sarcoidosis (SCS) is rare, and its diagnosis is challenging. We examined clinical, laboratory, and imaging features in patients with SCS to obtain useful clues for diagnosis and prognosis. Eleven consecutive patients (four males, seven females) at a single Japanese institution were investigated. Median age at onset was 66 years old. The most frequent site affected, other than the nervous system, was the respiratory system. While histological confirmation of non-caseating granulomas was often found there, no patient had respiratory symptoms. Peripheral nerve involvement was detected in 64% of patients. Soluble IL-2 receptor (sIL-2R) levels in serum and cerebrospinal fluid (CSF) were elevated in 64% and 45% of patients, respectively, and this finding was more common than elevation of angiotensin-converting enzyme (ACE). 18F-fluorodeoxyglucose (FDG) positron emission tomography showed abnormally high uptake in spinal lesions of all examined patients. Although corticosteroids were administrated to all patients, and immuno-suppressants were prescribed to six (55%), the modified Rankin Scale was unchanged or worsened in four (36%) patients during the follow-up period. Neurological exacerbation of myelopathy was seen in four (36%) patients. Complete response rate was only seen in 9%. High levels of cell count, protein, ACE, and sIL-2R in CSF were significantly more frequent in patients with a marked improvement after immunotherapy than in the other patients. These results suggest that high serum and CSF sIL-2R, high uptake of FDG, and peripheral nerve involvement are indicative of SCS. Given that SCS is commonly intractable, CSF abnormalities may predict efficacy of immunotherapies. [ABSTRACT FROM AUTHOR]

While prognoses in relation to myocardial infarction (MI) type have been elucidated in past reports, the results were not consistent, perhaps due to occurrence of Type 2 MI with CVS and its mortality. The Japanese registry of acute Myocardial Infarction diagnosed by Universal Definition (J-MINUET) is a prospective multicenter registry in Japan. In contrast to thromboembolic event-related Type 1 myocardial infarction (MI), clinical features of Type 2 MI, including coronary vasospasm (CVS), are varied due to the heterogeneous nature of its development. To elucidate the MI type-related all-cause mortality, 2989 consecutive patients with AMI were stratified as Type 1 MI, Type 2 MI with CVS, and Type 2 MI with non-CVS. Most patients (n = 2834; 94.8%) were classified as Type 1 MI and 155 patients (5.2%) were classified as Type 2 MI. Of the Type 2 MI patients, 87 (56% of Type 2 MI) were diagnosed as MI with CVS. Although the 3-year mortality was comparable between Type 1 and Type 2 MI patients, significant differences were observed between Type 2 MI with CVS and with non-CVS (3.4% and 22.1%, p < 0.001). Among Japanese patients with AMI, mortality rates between Type 1 MI and Type 2 MI are comparable, but further stratification of Type 2 MI (with or without CVS) may be useful in predicting the prognosis of patients with Type 2 MI. [ABSTRACT FROM AUTHOR]

Azimuthal asymmetry of atmospheric delay, coming from hydrostatic delay (dry air) and wet delay (water vapor), makes atmospheric delay gradients (MacMillan, 1995). Those gradients, unless properly treated, may cause GNSS positioning errors of up to a few centimeters, and its spatial and temporal changes help us study dynamics of tropospheric water vapor. We studied average atmospheric delay gradients in winter (January) and summer (July) at ~1300 continuous GNSS stations in Japan with time resolution of 4 hours using the GAMIT software. Gradients at the coastal stations in summer are dominated by changes in atmospheric water vapor and are made of diurnal landward components and stationary southward components. Those in winter, on the other hand, showed stationary southward components throughout the day. Diurnal landward gradients in summer show maximum in daytime (~0.5 mm). They correlate well with the strength of sea breeze (2-4 m/s) and would originate from the landward thickening of the atmospheric boundary layer due to landward winds and topographic slopes. We also found those at inland stations often show strong diurnal changes in gradient. They may come from mountain-valley breeze, but their pattern is not so clear as coastal stations. Surface pressure distribution and spatial changes in zenith total delays suggest that the stationary southward components in winter (~0.26 mm) come ~70 % from hydrostatic delay and ~30 % from wet delay. This reverses for stationary southward components in summer (~0.30 mm), i.e. they come ~30 % from hydrostatic delay and ~70 % from wet delay. [ABSTRACT FROM AUTHOR]

Background: Sex differences in clinical characteristics of ischemic heart disease patients have been widely examined, differences in the type of coronary artery disease (CAD) and their impacts on prognosis have not been well elucidated in Japanese. Methods: The Kumamoto Intervention Conference Study (KICS) is a multicenter registry enrolling consecutive patients undergoing percutaneous coronary intervention (PCI) in 16 centers in Japan. Between August 2008 and March 2014, 11,095 consecutive patients were enrolled in this registry. To clarify the sex differences, depending on the type of coronary artery disease, 10,822 successful PCI subjects with 1-year follow-up were enrolled in this study. Results: In this patient population, 2,983 patients (28.3%) were female and the remaining 7,839 patients (71.7%) were male. Age and comorbidity of chronic kidney disease(CKD) were significantly higher in female, and other atherosclerotic factors were significantly higher in male. The 1-year MACE was significantly higher in female patients (2.6% vs. 2.0%, p=0.084). Sub-analysis in patients with stable coronary artery disease (sCAD) or unstable angina (UA), sex difference was not observed in any 1-year MACE, procedural factors, or medication treatments. Multivariate analysis revealed CKD and peripheral artery disease (PAD) in female, and PAD in males as independent predictors for 1-year MACE. As regards patients with acute myocardial infarction (AMI), 1-year MACE was significantly higher in female (2.5% vs. 1.5%, p=0.023). Proportion of Killip3/4 (11.5% vs. 9.2%, p=0.034) and slow flow/no reflow rate during PCI procedures (11.5% vs. 8.9%, p=0.015) were significantly higher in female than in male. Post dilatation after stent deployments was significantly fewer in female than in male (44.0% vs. 50.7%, p=0.001). Regarding with medication treatments, usage of beta-blocker was significantly lower in female than in male (48.6% vs. 53.4%, p=0.009). Predictive factors for 1-year MACE in AMI patients were high age and left main trunk (LMT) lesions in female, CKD and LMT lesion in male, respectively. Conclusion: Sex differences in the clinical events after PCI were observed only in AMI patients, furthermore, the predictors for the events, depending on the sex, were different among the different type of coronary artery disease. [ABSTRACT FROM AUTHOR]

Background: Recessive mutations in SLC12A6 have been linked to hereditary motor sensory neuropathy with agenesis of the corpus callosum. Patients with early‐onset peripheral neuropathy associated with SLC12A6 heterozygous variants were reported in 2016. Only five families and three variants have been reported to date, and the spectrum is unclear. Here, we aim to describe the clinical and mutation spectra of SLC12A6‐related Charcot–Marie–Tooth (CMT) disease in Japanese patients. Methods: We extracted SLC12A6 variants from our DNA microarray and targeted resequencing data obtained from 2598 patients with clinically suspected CMT who were referred to our genetic laboratory by neurological or neuropediatric departments across Japan. And we summarized the clinical and genetic features of these patients. Results: In seven unrelated families, we identified one previously reported and three novel likely pathogenic SLC12A6 heterozygous variants, as well as two variants of uncertain significance. The mean age of onset for these patients was 17.5 ± 16.1 years. Regarding electrophysiology, the median motor nerve conduction velocity was 39.6 ± 9.5 m/sec. For the first time, we observed intellectual disability in three patients. One patient developed epilepsy, and her brain MRI revealed frontal and temporal lobe atrophy without changes in white matter and corpus callosum. Conclusions: Screening for the SLC12A6 gene should be considered in patients with CMT, particularly those with central nervous system lesions, such as cognitive impairment and epilepsy, regardless of the CMT subtype. [ABSTRACT FROM AUTHOR]

Cardiovascular and cerebrovascular diseases are considered the principal cause of morbidity and mortality worldwide; the effect of stroke-induced cardiac manifestations is well recognized; however, not enough clinical data have been found about the impact of stroke with underlying cardiac disease. This study's objective is to assess the impact of stroke on the prognosis of patients with underlying IHD, who underwent PCI treatment. This was a multicenter, 1-year observational study in patients undergoing PCI in one of the 17 participating centers across Japan. 18,495 patients were registered on the PCI list; 2481 patients had a prior stroke experience, whereas 15,979 were stroke-free. Our study revealed that stroke patients were significantly older (mean age 73.5 ± 9.6, 69.7(± 11.5), respectively), and suffered from more comorbidities (diabetes, hypertension, and chronic kidney disease, p < 0.0001). During the 1-year period, subjects with stroke showed higher incidence of clinical events compared to those without stroke; to illustrate, all-cause death accounted for 6.2% in patients with stroke, in contrast to only 2.8% in stroke-free patients (p < 0.0001), cardiac death amounted for 2.2 and 1.2%, respectively (p < 0.0001), recurrent stroke for 3.1% and 1.2% (p < 0.0001), non-cardiac death for 3.6 and 1.54% (p < 0.0001), and finally, hemorrhagic complications with 2.6 and 1.3% (p < 0.0001). Kaplan–Meier analysis revealed that stroke patients had a higher probability of all-cause mortality, cardiac death, and recurrent stroke (log-rank p < 0.0001). Cox hazard analysis also showed that the presence of stroke is a significant indicator in determining the outcome of cardiac death (HR = 1.457, 95% CI 1.036–2.051, p = 0.031); hence, proving it to be a crucial predictor on cardiac prognosis. History of prior stroke was common in PCI patients, and independently associated with a higher rate of subsequent cardiovascular and cerebrovascular events recurrence. Thus, highlighting an urgent need for comprehensive prevention of cardiac and cerebrovascular diseases. [ABSTRACT FROM AUTHOR]

The epidemic of coronavirus disease‐2019 (COVID‐19) is the major public health issue in the world. COVID‐19 vaccines are one of the most effective strategies against COVID‐19. Here we report a 36‐year‐old female patient who had thirst, polydipsia, polyuria, palpitations, loss of appetite, and fatigue 3 days after the first dose of COVID‐19 RNA‐based vaccines without a prior history of diabetes. Ten days after vaccination, she visited our hospital with diabetic ketoacidosis and was diagnosed with type 1 diabetes. Hyperglycemia (501 mg/dL), anion gap metabolic acidosis and ketonuria were observed. The glycated hemoglobin level was 7.0%. Islet‐related autoantibodies were all negative. The glucagon tolerance test revealed attenuated secretion of insulin. Human leukocyte antigen was haplotype DRB1*0405‐DQB1*0401, which was associated with type 1 diabetes in Japan. The present case suggests that COVID‐19 RNA‐based vaccines might trigger the onset of type 1 diabetes, even in subjects without prior histories of diabetes. [ABSTRACT FROM AUTHOR]

Influenza D virus (IDV) can potentially cause respiratory diseases in livestock. We isolated a new IDV strain from diseased cattle in Japan; this strain is phylogenetically and antigenically distinguished from the previously described IDVs. [ABSTRACT FROM AUTHOR]

Introduction: Under the awareness of appropriate use criteria, the significance of PCI for the patients with silent myocardial ischemia (SMI) decreases. On the other hand, the prognoses of SMI patients have been reported to be equivalent to those of symptomatic effort angina pectoris (EAP) patients. We aimed to clarify the prognosis of SMI after PCI and elucidate the factors influencing the mortality. Methods: The Kumamoto Intervention Conference Study is multicenter registry enrolling consecutive patients undergoing PCI in 16 centers in Japan. Overall, 11,093 consecutive patients were enrolled during April 2008 to March 2014. To compare the clinical events between SMI and EAP, 5,039 subjects, excluding acute coronary syndrome, post successful PCI were enrolled with 1-year follow-up data. Results: In this study, 1,124 patients were classified as SMI and 3,915 patients were EAP. Male, diabetes, smoking habits, prior myocardial infarction (MI), and composite of PAD were all significantly higher in the SMI group. Although nonfatal MI and stent thrombosis were comparable between the 2 groups, all cause mortality and cardiac death during 12-months were significantly higher in the SMI group than in the EAP group (3.7% vs 1.6%, 1.4% vs 0.4%, respectively). Kaplan-Meier analysis showed a significant difference between the groups (Figure). In addition to lower BMI, composite of PAD, and LMT lesion, SMI was an independent predictor for cardiac death. Even after adjustment by propensity-score matching, SMI showed a higher cardiac death rate compared with EAP (1.4% vs 0.2%, p=0.001), and it remained as a significant predictor. In the sub-analysis for SMI patients, multivariate analysis revealed lower BMI and LMT lesion as predictors for cardiac death. Conclusion: In this study, SMI itself was associated with higher mortality after PCI, especially in patients with lower BMI or LMT lesion. Strict follow-up and assessment of residual ischemia should be necessary for SMI patients. [ABSTRACT FROM AUTHOR]