Izumi fever (IF), also known as Far East scarlet-like fever (FESLF), is caused by Yersinia pseudotuberculosis and it has clinical features resembling those of Kawasaki disease (KD). As both diseases ...are rare in adolescents and young adults, it is challenging to recognize them, thus often leading to a delayed diagnosis. We herein present two cases of IF or FESLF (IF/FESLF). The first case was misdiagnosed as KD, which led to a diagnostic delay. The second case was recognized earlier owing to our experience with the first case. Although cultures were negative in both cases, presumably due to the prior use of antimicrobial agents, our clinical suspicion and a paired serological assay for anti-Yersinia pseudotuberculosis antibodies finally led to a successful diagnosis.
Izumi fever (IF), also known as Far East scarlet-like fever (FESLF), is caused by Yersinia pseudotuberculosis and it has clinical features resembling those of Kawasaki disease (KD). As both diseases ...are rare in adolescents and young adults, it is challenging to recognize them, thus often leading to a delayed diagnosis. We herein present two cases of IF or FESLF (IF/FESLF). The first case was misdiagnosed as KD, which led to a diagnostic delay. The second case was recognized earlier owing to our experience with the first case. Although cultures were negative in both cases, presumably due to the prior use of antimicrobial agents, our clinical suspicion and a paired serological assay for anti-Y. pseudotuberculosis antibodies finally led to a successful diagnosis.
Background:Guidelines for peripheral arterial disease (PAD) recommend long-term antiplatelet therapy in symptomatic patients to reduce cardiovascular morbidity and mortality risk. Although diabetes ...is a known risk factor for PAD, PAD has been undertreated in these patients. This study aimed to evaluate risk factors for major amputation in patients with diabetes undergoing antiplatelet therapy for PAD.Methods and Results:This retrospective analysis of a 2-year observational cohort study (1,745 clinics in Japan, September 2009–2013) evaluated predictors of amputation in patients with diabetes undergoing antiplatelet therapy for PAD. Among 4,016 eligible patients, 52 had an amputation during follow-up. Amputation risk (Cox regression analysis) was predicted at baseline by history of lower extremity revascularization/amputation (hazard ratio HR: 2.92; 95% confidence interval CI: 1.39, 6.14), chronic kidney disease (HR: 4.19; 95% CI: 1.95, 8.97), and comorbid cerebrovascular and heart disease (HR: 3.32; 95% CI: 1.19, 9.30), and was unaffected by choice of oral antiplatelet therapy. In patients with PAD and diabetes, amputation event rate was highest for those with ankle-brachial pressure index (ABI) <0.40 and progressively decreased at higher ABI cut-offs.Conclusions:These findings inform real-world understanding of PAD in diabetic patients receiving antiplatelet therapy in Japan, and showed that ABI <0.4 was the strongest risk factor for amputation.
From late March through April 2021, we experienced a cluster of patients with COVID-19, named “Cluster K”, with rapid severe illness compared with those who were infected before.
Patients with ...COVID-19 who were enrolled in this study were divided into two groups: 66 patients from November 2020 to March 2021 (group A) and 37 patients whose infection links were traced from Cluster K (group B). The primary outcome was mortality rate, and the secondary outcome was maximal oxygen flow rate as the severity of the disease. Viral genome sequences were compared between the two groups.
Mortality rates were 6.1% in group A and 16.2% in group B (odds ratio: 2.97, 95% confidence interval: 0.65–15.38). The patients in group B required high oxygen flow rate (O2 ≥10 l/min) in the earlier clinical course (P = 0.029). Viral genome sequences revealed five amino acid mutations; of these, four were found on three nonstructural proteins (NSPs): one in nsp3 and nsp15, two in nsp6 (one of them is near the potential sites under positive selective pressure). Another one was on the S protein.
This study suggests that mutations in NSPs, especially nsp6, are associated with adverse clinical outcome in patients with COVID-19.
Background:Despite mounting evidence of increased cardiovascular events in patients with peripheral arterial disease (PAD), the overall incidence of cardiovascular events in PAD patients has not been ...fully clarified in Japan. The prospective Surveillance of cardiovascular Events in Antiplatelet-treated arterioSclerosis Obliterans patients in JapaN (SEASON) is a prospective observational multicenter study and here we report the baseline clinical characteristics, including atherosclerosis risk factor prevalence, in PAD patients treated with antiplatelet agents.Methods and Results:The SEASON registry enrolled 11,375 patients in 1,745 institutions and the data for 10,322 patients were analyzed. At baseline, the average age was 73.8±9.9 years, 60.0% were male and 83.9% were in Fontaine stage I or II. They had arteriosclerosis risk factors, such as current smoking (16.2%), hypertension (61.5%), diabetes mellitus (38.3%) and dyslipidemia (38.8%). There were complications including heart disease (29.7%), cerebrovascular disease (17.1%) and chronic kidney disease (14.3%). A subpopulation analysis revealed that the proportions of patients with risk factors were high in patients with lower ankle-brachial pressure index value.Conclusions:The baseline characteristics of the SEASON population demonstrate that real-world PAD patients have cardiovascular risk factors and comorbidities next to definite PAD patients. Further analysis of this database will contribute to understanding the real-world situation of PAD patients receiving antiplatelet therapy in Japan. (Circ J 2016; 80: 712–721)
The present analysis was intended to evaluate the real-world management of peripheral arterial disease (PAD) in Asia, and to explore cardiovascular events in patients with PAD undergoing antiplatelet ...therapy over 2 years of follow-up. The Surveillance of cardiovascular Events in Antiplatelet-treated arteriosclerosis Obliterans patients in JapaN (SEASON) registry is a prospective observational multicenter study of cardiovascular events in antiplatelet-treated patients with PAD in Japan. The SEASON registry included 11,375 patients who were scheduled to receive treatment for PAD. Two analysis populations were defined: a real-world population (RWP; n = 10,322) and a definite PAD population (DPP; n = 3992) who had ankle-brachial pressure index (ABPI) <0.9 and intermittent claudication, or a history of lower limb revascularization. The primary outcome measure was the rate of the composite of cerebrovascular, cardiovascular, and peripheral vascular events. The composite event rates (95% confidence interval) were 3.28 (3.00-3.57) and 5.71 (5.13-6.34) events per 100 patient-years in the RWP and DPP groups, respectively. Fontaine IV classification and ABPI <0.4 at baseline were both identified as strong risk factors for vascular events. These findings contribute to understanding the situation for real-world patients with PAD receiving antiplatelet therapy.
Background
Stem cell transplantation (SCT) outcomes have improved over the last three decades, with many patients being rescued with this treatment. However, improved outcomes have led to issues with ...long‐term sequelae. One of these sequelae in children is renal dysfunction, an index of which is estimated using glomerular filtration rate (eGFR).
Procedure
We retrospectively analyzed eGFR in 83 pediatric patients who received SCT. Data from all patients extended up to 12 months or more post SCT. The median follow‐up time was 127.7 months (range 12.0‐268.8 months).
Results
Eighteen patients (21.7%) had low eGFR (<90 ml/min/1.73 m2) post SCT. Cumulative incidence of low eGFR was 25.8 ± 2.0%. Nine (10.6%) patients had a low eGFR pre‐SCT. However, pre‐ and post‐SCT incidence of low eGFR were not correlated. Meanwhile, only two patients (2.4%) exhibited severe renal dysfunction, with eGFRs < 60 ml/min/1.73 m2. Independent risk factors for low eGFR were solid tumor and use of fludarabine. Moreover, age at SCT ≥ 7 years was also a long‐term post‐SCT risk factor for low eGFR in all patients.
Conclusion
Independent post‐SCT long‐term risk factors for low eGFR in children were solid tumor and use of fludarabine. Moreover, age at SCT ≥ 7 years was a post‐SCT long‐term risk factor for low eGFR across all patients.
Background: Randomized phase III clinical trials suggest that the antibody cocktail containing casirivimab and imdevimab reduces the risk of hospitalization/death in high-risk COVID-19 patients. ...However, the efficacy of the cocktail in daily clinical practice remains unknown.Methods: We administered the antibody cocktail to consecutive febrile high-risk COVID-19 patients hospitalized at our institute from late July 2021 through early September 2021. During the time, the delta variant was the dominant variant in our community. Primary efficacy was defined as reduction of fever to below 37°C and absence of need for additional medical interventions after the cocktail therapy. In addition, several laboratory data were examined to detect the cutoff values for favorable clinical outcomes by ROC curve analysis.Results: The cocktail was given to 55 febrile high-risk COVID-19 patients, 38 (69.1%) out of which were male. The median values (and ranges) of the age, SpO2, and number of comorbidities were 51 (20-94) years; 97% (93%-99%), and 2 (1-5), respectively. 43.6% of the patients showed evidence of pneumonia. The cocktail therapy showed 74.5% efficacy in the 55 patients who received the therapy. The median value and range of the duration (day) of fever after the cocktail therapy was 1.5 (1-4), respectively. The cocktail infusion was administered a median of 4 days from disease onset, and the median duration of fever from disease onset was 5.4 days. The median observation period after the cocktail infusion was 6 days. The cutoff values of the age, SpO2, serum CRP, and serum LDH were ≤72 years old, ≥96%, <2.64mg/dL, and <220U/L, respectively (odds ratios=12.2, 27.8, 11.6, and 17.2, respectively).Conclusions: The antibody cocktail therapy was associated with a speedy recovery from COVID-19-related fever, and the efficacy is likely affected by the age, SpO2, serum CRP, and serum LDH values.
Almost 90% of nephrogenic diabetes insipidus (NDI) is caused by mutations in the arginine vasopressin receptor 2 gene (AVPR2) on the X chromosome. Herein, we reported clinical and biochemical ...parameters in four cases of three unrelated Japanese families and analyzed the status of the AVPR2. Two of the four patients had poor weight gain. However, in the male and female sibling cases, neither had poor weight gain while toddlers, but in the male sibling, episodes of recurrent fever, polyuria, and polydipsia led to the diagnosis of NDI at 4 years of age. Analysis of AVPR2 identified two nonsense mutations (c.299_300insA; p.K100KfsX91 and c.296G > A; p.W99X) and one missense mutation (c.316C > T; p.R106C). These mutations were previously reported. The patient with c.316C > T; p.R106C had milder symptoms consistent with previous reports. Of the familial cases, the sister was diagnosed as having NDI, but a skewed X-inactivation pattern in her peripheral blood lymphocytes was not identified. In conclusion, our study expands the spectrum of phenotypes and characterized mutations in AVPR2 in NDI.
Kawasaki disease is an acute febrile disease predominantly seen in young children. We report a case of Kawasaki disease in a 32-year-old pregnant woman. She developed a generalized erythematous skin ...rash accompanied by high fever. Bilateral conjunctival congestion, tender cervical lymphadenopathy, an edematous lower lip and peripheral edema followed by desquamation were observed. She was successfully treated with aspirin and intravenous gammaglobulin (1 g/kg/day). Her course was not complicated by coronary artery aneurysm and she delivered a healthy baby. To the best of our knowledge, this is the first case of Kawasaki disease in a pregnant woman. We suggest that Kawasaki disease should be included in the differential diagnosis of a generalized, erythematous skin rash accompanied by high fever in adults.
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