Japanese Red Cross Kobe Hospital

Porphyrias are rare metabolic disorders caused by decreased or increased activity of one of the nine enzymes involved in the heme biosynthetic pathway, resulting in the accumulation of porphyrins or their precursors. Although nine subtypes are currently recognized, substantial overlap in clinical manifestations often makes diagnosis challenging. This article presents the English version of the first national clinical practice guidelines for porphyrias in Japan, originally published in 2025 by the Japanese Dermatological Association. The guidelines were developed by the Clinical Practice Guideline Committee, established as part of the Research Project on Intractable Diseases of the Ministry of Health, Labour and Welfare of Japan. Diagnostic and severity assessment criteria were based on those proposed by the Ministry research group. Clinical questions (CQs) were formulated, and recommendations were developed according to the principles of evidence-based medicine, incorporating both domestic and international evidence. The guidelines provide diagnostic criteria, severity classification, and evidence-based recommendations for the management of porphyrias. Although causative genes have been identified, the pathophysiology remains incompletely understood, and no curative therapy is currently available, and liver transplantation may be required in severe cases with liver failure. These guidelines aim to provide practical guidance for clinicians managing porphyrias in Japan. Future revisions will be necessary in response to advances in diagnostic technologies and therapeutic strategies.

Skin thinning over cardiac implantable electronic devices (CIEDs) is frequently observed; however, its clinical implications and temporal progression remain unclear. This study aimed to investigate the clinical significance of skin thickness over CIEDs by examining its association with adverse outcomes, including all-cause mortality and device-related infections, and evaluating its temporal changes and associated clinical factors. This study included 96 patients who underwent ultrasonographic measurement of skin thickness over CIEDs between June 2020 and June 2021. Adverse outcomes were monitored through August 2023. Of those, 34 patients had follow-up ultrasonography to assess temporal changes in skin thickness. Among 34 patients with repeated measurements, skin thickness significantly declined over a median of 42 (IQR: 41-44) months (p = 0.001), and thinning was correlated with changes in hemoglobin levels (r = 0.392, p = 0.024). During a median follow-up of 32 (IQR: 11-34) months, patients with skin thickness ≤ 3.3mm (thin group) had significantly higher all-cause mortality than those with a thickness > 3.3mm (normal group) (log-rank p < 0.001). In multivariable Cox regression analysis, skin thickness ≤ 3.3mm remained an independent predictor of mortality (HR 2.42; 95% CI, 1.01-5.80; p = 0.046). Device-related infections were more frequent in the thin group (8%) than in the normal group (2%), but the difference was not statistically significant. Skin thickness over CIEDs progressively declined over time and was significantly correlated with hemoglobin levels. In addition, skin thickness ≤ 3.3mm was independently associated with increased all-cause mortality.

Self-expanding metal stents (SEMS) are widely used as a bridge to surgery in obstructive colon cancer, offering short-term benefits by reducing postoperative complications and avoiding emergency stoma creation. Additionally, early initiation of systemic chemotherapy may improve oncological outcomes. However, the safety and feasibility of preoperative chemotherapy following SEMS placement remain unclear. This study aims to evaluate the safety and efficacy of neoadjuvant CAPOX chemotherapy after SEMS placement in patients with obstructive colon cancer. This is a prospective, multicenter, single-arm Phase II trial involving patients with clinical stage II or III obstructive colon cancer. Eligible patients undergo successful SEMS placement followed by two cycles of CAPOX chemotherapy prior to elective surgery. The primary endpoint is the incidence of severe perioperative complications, defined as a composite of stent-related adverse events and Clavien - Dindo Grade III or higher postoperative complications. Secondary endpoints include chemotherapy-related adverse events, pathological response, and 2-year relapse-free survival. A total of 75 patients are planned for enrollment across 16hospitals in Japan over 1.5 years. The study is registered in the Japan Registry of Clinical Trials (jRCTs051240077).

Some ALK-positive lung cancers show early resistance to alectinib. This study found brain metastases and elevated NLR associated with early resistance. Spatial transcriptomics and functional analysis suggested involvement of ANXA1. These results reveal clinical and molecular biomarkers that may guide treatment strategies for early resistance to alectinib.

ObjectiveTo predict walking independence in patients with incomplete cervical cord injury (ICCI) using electromyography of the trunk and lower extremity during straight-leg raising (SLR).DesignProspective cohort study. Prediction model using logistic regression.SettingSingle acute-care hospital.ParticipantsForty patients equally split between walking dependent and walking independent groups (mean ages: 70.7±14.4 and 63.7±15.4 years; male-to-female ratios: 18:2 and 16:4; total length of stay: 31.2±20.9 and 25.7±16.5 days).InterventionsNot applicable.Main Outcome MeasuresTrunk and lower extremity acceleration and surface electromyography data measured during nondominant SLR and basic information were used to predict the level of walking independence at discharge or transfer from the acute-care hospital.ResultsThe results showed that the model with the lower extremity motor score (LEMS) and the root mean square (RMS) of the contralateral external oblique (EO) had the lowest Akaike information criterion of 22.24, and both were significant factors in predicting walking independence at discharge or transfer from the acute-care hospital (P=.019 and .034). The predictive accuracy, sensitivity, specificity, and area under the receiver operating characteristic curve were 0.875, 0.850, 0.900, and 0.975 (95% confidence interval: 0.939-1.000), respectively.ConclusionsA higher LEMS and lower RMS of the contralateral EO during nondominant SLR are significant predictors of walking independence at discharge or transfer from an acute-care hospital in patients with ICCI.

There is a paucity of data on safety of calcium channel blockers (CCB) in patients with severe aortic stenosis (AS) and hypertension. Among 2,460 patients with severe AS and hypertension receiving antihypertensive therapy in the CURRENT AS registry-2, we compared the clinical outcomes between patients taking antihypertensive therapy with CCB (CCB group) and without CCB (no CCB group). In the entire study population, CCB was prescribed in 1,763 patients (71.7%), which was the most commonly prescribed antihypertensive agents. The prescription rates of angiotensin converting enzyme inhibitors or angiotensin receptor blockers, beta-blockers, and thiazides were 61.9%, 25.6%, and 7.3% in the CCB group, and 75.8%, 54.4%, and 6.0% in the no CCB group. In the propensity score matched cohort, the cumulative 3-year incidence of all-cause death or hospitalization for heart failure was not different between the CCB and no CCB groups (38.3% vs. 38.7%, log-rank P=0.65; HR, 0.94; 95%CI, 0.77-1.15; P=0.56). The cumulative 3-year incidence of syncope was low regardless of CCB prescription (1.1% vs. 1.0%, P=0.74). Among patients with severe AS and hypertension, CCB was the most commonly prescribed antihypertensive agents, and antihypertensive therapy with CCB was associated with comparable clinical outcomes to antihypertensive therapy without CCB. Syncope was rarely seen in patients with severe AS and hypertension receiving antihypertensive therapy regardless of CCB prescription.

The benefit of early surgery for proximal femoral fractures is widely recognized, but its impact on blood loss remains unclear. Comparisons of blood loss related to early surgery are complicated by the influence of anticoagulants. This retrospective study aimed to I have confirmedinvestigate the effect of early surgery within 48 h on perioperative blood loss in proximal femur fractures, adjusting for factors affecting blood loss using propensity score matching. Early surgery, with or without anticoagulants, reduces blood loss and transfusion requirements compared to delayed surgery. We retrospectively collected data from a cohort of patients aged ≥65 years with isolated proximal femoral fractures who underwent surgery at our hospital from January 2010 to December 2019. Blood loss was assessed from admission to the day after surgery, and blood transfusions were recorded within one week. Patients were divided into two groups: the early surgery group (surgery within 48 h, Group E) and the delayed surgery group (surgery after 48 h, Group W). Propensity score matching was used to adjust for potential confounders, and statistical analyses were performed to compare blood loss and transfusion rates between groups. A total of 829 patients (mean age: 83.7 ± 7.4 years; 653 females, 176 males) were included, with 441 in Group E and 388 in Group W. After matching, 261 patients were analyzed. Blood loss was significantly higher in Group E (553 mL) than in Group W (452 mL, p < 0.005). However, no significant difference was observed in transfusion rates between the groups (Group E: 61.6 %, Group W: 67.8 %; p = 0.186). Early surgery for proximal femur fractures reduces postoperative complications and mortality but should be performed with caution due to increased blood loss.