Objectives Dermatomyositis (DM) is often associated with fatal anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive rapidly progressive interstitial lung disease (RP-ILD). RP-ILD often fails to respond to intensive treatment and has a poor prognosis. We examined the effectiveness of early plasma exchange therapy plus intensive treatment with high-dose corticosteroids and multiple immunosuppressants. Methods Autoantibodies were identified by an immunoprecipitation assay and enzyme-linked immunosorbent assay. All clinical and immunological data were collected retrospectively from medical charts. We divided patients into two groups based on treatment regimen: intensive immunosuppressive therapy alone as initial treatment (IS group) and early initiation of plasma exchange (PE) plus intensive immunosuppressive therapy (ePE group). Early PE therapy was designated if PE therapy was initiated within two weeks of starting treatment. Comparisons of the treatment response and prognosis between groups were performed. Patients Anti-MDA5-positive DM with RP-ILD was screened. Results Forty-four RP-ILD and DM patients had anti-MDA5 antibodies. Four patients were excluded because they died before receiving sufficient combined immunosuppressive therapy or before the evaluation of the immunosuppressive treatment effectiveness (IS, n=31; ePE, n=9). All 9 patients in the ePE group had improved respiratory symptoms and were alive, whereas 12 of 31 patients in the IS group died (100 vs. 61%, p=0.037). Of the 8 patients who had 2 values for a poor prognosis, indicating the highest risk for death using the MCK model, 3 of 3 patients in the ePE group and 2 of 5 in the IS group were alive (100 vs. 40%, p=0.20). Conclusion The early initiation of ePE therapy plus intensive immunosuppressive therapy was effective for patients with DM and refractory RP-ILD.

Background: Recently, metabolic dysfunction-associated fatty liver disease (MAFLD) has been proposed. It is uncertain how indices that predict fatty liver are associated with MAFLD in Japanese. Methods: Among subjects who underwent a health examination at our hospital, 1257 (men: 787, women: 474) subjects participated in fatty liver evaluation of the fatty liver index (FLI) and fatty liver predicting index (FLPI) were included in this cross-sectional study. The discriminatory ability of each index for MAFLD was tested using receiver operating characteristic curve analysis. The association between FLI, FLPI, and MAFLD was investigated using multiple logistic regression analysis. Results: FLI and FLPI had good discriminatory ability for identifying MAFLD in both men and women, with specific cutoff values. Both FLI and FLPI were significantly higher in subjects with MAFLD, and the odds of MAFLD were higher among those in the highest tertile relative to the lowest tertile in both men and women. FLI and FLPI were higher in subjects who met the criteria for both MAFLD and metabolic syndrome (MetS) compared to those who had MAFLD or MetS alone, and most of the examined parameters in subjects with both conditions indicated a high metabolic risk profile. Conclusions: The study suggests that FLI and FLPI are valuable tools for predicting MAFLD and are similarly correlated with the disease. Furthermore, the highest values of these indices were observed in subjects who met the criteria for both MAFLD and MetS, emphasizing the importance of considering both conditions when assessing metabolic risk.

Recently, a once-daily dose of edoxaban (15-mg) has been approved for stroke prevention in non-valvular atrial fibrillation (NVAF) patients aged ≥ 80 years, in whom standard oral anticoagulants are not recommended because of high bleeding risk (HBR), based on the ELDERCARE-AF trial. However, information regarding the characteristics and clinical outcomes among such patients is limited. Thus, this study aimed to clarify the characteristics and event rates in elderly patients with NVAF and HBR defined by the ELDERCARE-AF criteria. Of the 7406 NVAF outpatients included in the J-RHYTHM Registry, 60 patients with creatinine clearance (CrCl) < 15 mL/min were excluded. The remaining 7346 patients (age, 69.7 ± 9.9 years; men, 70.9%; warfarin use, 78.7%) were divided into three groups: Group 1, aged < 80 years (n = 6165); Group 2, aged ≥ 80 years without HBR (n = 584); and Group 3, aged ≥ 80 years with HBR (at least one of the followings; CrCl, 15–30 mL/min, history of bleeding, body weight ≤ 45 kg, and antiplatelet use) (n = 597, eligible for 15-mg edoxaban). Patients in Group 3 had a higher prevalence of comorbidities, and therefore, both higher thromboembolic and bleeding risk scores than in the other groups. During the 2-year follow-up period, the incidence rates (per 100 person-years) of thromboembolism in Groups 1, 2, and 3 were 0.7, 1.5, and 2.1 (P < 0.001), major hemorrhage, 0.8, 1.2, and 2.0 (P < 0.001), and all-cause death, 0.8, 2.6, and 4.6 (P < 0.001), respectively. Adjusted hazard ratios of Group 3 were 1.64 (95% confidence interval 0.89–3.04, P = 0.116) for thromboembolism, 1.53 (0.85–2.72, P = 0.154) for major hemorrhage, and 1.84 (1.19–2.85, P = 0.006) for all-cause death compared with Group 1. The NVAF Patients aged ≥ 80 years with HBR defined by the ELDERCARE-AF criteria were certainly at a higher adverse event risk, especially for all-cause death. Clinical trial registration: The J-RHYTHM Registry is registered in the University Hospital Medicine Information Network (UMIN) Clinical Trials Registry (unique identifier: UMIN000001569) http://www.umin.ac.jp/ctr/.

BackgroundIn young healthy children, assessing airflow limitation may be difficult because of the narrowing of the airways, which is a pathology of asthma, and responsiveness to bronchodilators. ObjectiveWe investigated whether lung sound analysis could predict the development of recurrent wheezing (RW), one of the signs of asthma. MethodsIn healthy children aged 3 to 24 months, we recorded and analyzed lung sounds before and after inhalation of bronchodilators and conducted a questionnaire survey. The children were followed up and assessed for the development of RW at 3 years of age. ResultsOf the 62 patients analyzed, 19 (30.6%) developed RW. The parameters RPF50 and RPF75, calculated by lung sound analysis, were lower in the RW group, with odds ratios (95% CI) of 0.77 (0.61–0.98) and 0.81 (0.66–0.99), respectively. The change rate of lung sound analysis parameters after bronchodilator inhalation was not different among all participants; however, in the subgroup of children with a history of atopic dermatitis, B4/AT and ΔRPF50 were elevated in the RW group (P = .015 and P = .041, respectively). In the subgroup of children with total IgE >20 kUA/L, the sensitivity and specificity (95% CI) for predicting the development of RW were 85.7% (48.7-99.3) and 68.8% (44.4-85.8), respectively, when the cutoff value of ΔRPF50 was set at 10.5%. ConclusionThe lung sound analysis method allows noninvasive assessment of the airway, including airway hypersensitivity, in young children and may predict the risk of developing RW.

The American Joint Committee on Cancer (AJCC) 8th edition T-staging system for distal cholangiocarcinoma (DCC) proposes classification according to the depth of invasion (DOI); nevertheless, DOI measurement is complex and irreproducible. This study focused on the fibromuscular layer and evaluated whether the presence or absence of penetrating fibromuscular invasion of DCC contributes to recurrence and prognosis. In total, 55 patients pathologically diagnosed with DCC who underwent surgical resection from 2002 to 2022 were clinicopathologically examined. Subserosal layer and/or pancreatic (SS/Panc) invasion, defined as penetration of the fibromuscular layer and invasion of the subserosal layer or pancreas by the cancer, was assessed with other clinicopathological prognostic factors to investigate recurrence and prognostic factors. According to the AJCC 8th edition, there were 11 T1, 28 T2, and 16 T3 cases, with 44 (80%) cases of SS/Panc invasion. The DOI was not significantly different for both recurrence and prognostic factors. In the multivariate analysis, only SS/Panc was identified as an independent factor for prognosis (hazard ratio: 16.1; 95% confidence interval: 2.1-118.8, p = 0.006). In conclusion, while the determination of DOI in DCC does not accurately reflect recurrence and prognosis, the presence of SS/Panc invasion may contribute to the T-staging system.

Abstract Background In current practice, the ablation target of atypical atrioventricular nodal tachycardia (AVNRT) is the earliest atrial activation site in the coronary sinus (CS) or conventional slow pathway (SP) region. Objectives The purpose of this study was to map the site of earliest retrograde atrial activation using electroanatomic 3D mapping during atypical AVNRT and to evaluate successful ablation sites. Methods Forty-two patients with a total of 49 AVNRTs (slow/fast 30; fast/slow 15; slow/slow 4) underwent EP study and ablation. Among them there were 14 patients (10 women, 60±19 y/o) in whom 19 atypical AVNRT (fast/slow 15; slow/slow 4) were induced. Results The intracardiac electrocardiograms or 3D mapping of the exit site during tachycardia revealed that 7 patients had exit sites solely inside the CS (left inferior extension=LIE), 3 solely in the right postero-septal tricuspid annulus (TA) (right inferior extension=RIE), and 4 had both LIE and RIE exits. The distance from the CS ostium to LIE exits was 14±6 mm. RIE exits were located on the TA posterior to the CS ostium (between 5 and 6 o’clock in the LAO projection). Ablation targeting these exits or traditional SP succeeded in long term elimination of AVNRT in 13 of the 14 patients (93%). There were no complications. Conclusions Catheter ablation targeting the exit sites of LIE or RIE mapped at the CS or TA holds promise as an effective and safe alternative approach to the current targets of ablation for atypical AVNRT cases.Distribution of exit sites during atypicthe circuit of atypical AVNRT：the exit s

BackgroundRuptured aneurysm is a serious complication of distal pancreatectomy (DP) or pancreatoduodenectomy (PD) that can be life-threatening if not treated promptly. This study aimed to examine the efficacy of a Viabahn stent graft for stopping bleeding after pancreatectomy.MethodsBetween April 2016 and June 2022, we performed 245 pancreatectomies in our institution. Six patients experienced postoperative bleeding and underwent endovascular treatment.ResultsAll six cases of bleeding occurred post-PD (3.7%). The bleeding was from gastroduodenal artery (GDA) pseudoaneurysms in three patients, and Viabahn stent grafts were inserted. All three patients did not show liver function abnormalities or hepatic blood flow disorders. One patient with a Viabahn stent graft experienced rebleeding, which required further management to obtain hemostasis. Of the six cases in which there was hemorrhage, one case of bleeding from the native hepatic artery could not be managed.ConclusionsUsing the Viabahn stent graft is an effective treatment option for postoperative bleeding from GDA pseudoaneurysms following PD. In most cases, using this device resulted in successful hemostasis, without observed abnormalities in hepatic function or blood flow.

Imaging modality-based evidence is limited that compares the extent of coronary arterial repair after percutaneous coronary intervention between patients with stable angina pectoris (SAP) and those with acute coronary syndrome (ACS). Between December 2018 and November 2021, a single-center, nonrandomized, observational study was conducted in 92 patients with SAP (n = 42) or ACS (n = 50), who were implanted with Orsiro sirolimus-eluting stent (O-SES) providing a hybrid (active and passive) coating and underwent 1-year follow-up by coronary angioscopy (CAS) after implantation. CAS assessed neointimal coverage (NIC), maximum yellow plaque (YP), and mural thrombus (MT). Baseline clinical characteristics were comparable between the SAP and ACS groups. The follow-up periods were comparable between the two groups (390.1 ± 69.9 vs. 390.6 ± 65.7 days, p = 0.99). The incidences of MT at 1 year after implantation were comparable between the two groups (11.4% vs. 11.1%, p = 0.92). The proportions of "Grade 1" in dominant NIC grades were highest in both groups, and the proportions of maximum YP grades and MT were comparable between the two groups. O-SES-induced coronary arterial repair at the site of stent implantation, irrespective of the types of coronary artery disease.

BackgroundWe report two cases of biceps brachii and brachialis paralysis due to musculocutaneous nerve injury in which elbow joint flexion was reconstructed using rotational transfer of the latissimus dorsi muscle with sutures to the radial and ulnar tuberosities, thereby enabling flexion by simultaneous activation of the humeroradial and humeroulnar joints. In cases of associated brachialis paralysis, weaker flexion strength can be expected when the forearm is in a pronated position than when it is in a supinated state. To the best of our knowledge, no previous study has reported the rotational position of the forearm during elbow joint flexion reconstruction.Case presentationCase 1 involved a 30-year-old Asian male who presented with a rupture of the musculocutaneous, median, radial, and ulnar nerves. Reconstruction was performed by rotational transfer of the latissimus dorsi muscle. In this case, the supination and pronation flexion forces were equal. Case 2 involved a 50-year-old Asian man who presented with partial loss of the musculocutaneous nerve, biceps brachii, and pectoralis major due to debridement. Reconstruction was performed by rotational transfer of the latissimus dorsi muscle. In this case, supination and pronation flexion strengths were demonstrated to be equal. Our reconstruction method used the rotational transfer of the latissimus dorsi muscle; the distal muscle flap was divided into radial and ulnar sides to allow elbow joint flexion by simultaneously activating the humeroradial and humeroulnar joints. These sides were then fixed to the anchors at the radial and ulnar tuberosities. Finally, they were wrapped around the myotendinous junction of the biceps brachii or brachialis and secured using sutures.ConclusionsAlthough larger studies are required to verify these methods, this case study successfully demonstrates the following: (1) the flexion strength in the supinated position was equal to that in the pronated position; (2) the stability of the humeroradial and humeroulnar joints was unaffected by the forearm's rotational position; and (3) a satisfactory range of motion of the elbow joint was obtained, with no complications.

Backgroundand objective: This study examined the validity of sniff nasal inspiratory (SNIP) and reverse-sniff nasal expiratory pressures (RSNEP) for estimating respiratory muscle strength and for predicting poor life expectancy following exacerbation in patients with chronic obstructive pulmonary disease (COPD). MethodsThis prospective study included patients who were admitted for COPD exacerbation and underwent rehabilitation. At hospital discharge, SNIP, RSNEP, and maximum mouth inspiratory (MIP) and expiratory pressures (MEP) were measured, and the body mass index, degree of airflow obstruction, dyspnea, and exercise capacity (BODE) index was calculated by evaluating body mass index, forced expiratory volume in 1 s (FEV1), the Modified Medical Research Council Dyspnea Scale, and 6-min walk distance. ResultsData from 43 patients (mean age 76.8 years, FEV1 42.8 % predicted) were analyzed. SNIP and RSNEP were moderately correlated with MIP and MEP, respectively. Bland-Altman plot means of SNIP (48.3 ± 17.5) and RSNEP (44.7 ± 23.8 cmH2O) were lower than those of MIP (54.8 ± 19.9) and MEP (76.4 ± 31.2 cmH2O), respectively, and the SNIP–MIP and RSNEP–MEP 95 % limits of agreement were wide. Logistic regression showed that SNIP and RSNEP were significantly associated with BODE score ≥7 (poor life expectancy), and predictive accuracy was 81.4 % when combining SNIP ≤49 and RSNEP ≤42 cmH2O. ConclusionAfter exacerbation in patients with COPD, SNIP and RSNEP are useful indicators that complement MIP and MEP. Furthermore, a combined SNIP and RSNEP test may be beneficial in predicting poor life expectancy.