Nagano Red Cross Hospital

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) may trigger secondary autoimmune diseases, with autoimmune arthritis representing a rare yet challenging complication.Here, we report a six-year-old male who developed polyarticular juvenile idiopathic arthritis (JIA) three years after allo-HSCT for lymphoma. Despite resistance to multiple biologic disease-modifying anti-rheumatic drugs (bDMARDs), including adalimumab and tocilizumab, treatment with the Janus kinase 1/2 inhibitor ruxolitinib achieved clinical remission sustained for over two years without significant adverse events or lymphoma relapse.Ruxolitinib may represent a viable therapeutic option for refractory secondary JIA post-allo-HSCT, particularly when conventional bDMARDs fail.

Introduction and Purpose/Objectives: With the frequent occurrence of natural disasters, we have found the need to train community-based disaster mitigation nurses in silent phase, not just to train nurses to be dispatched after disasters occur. In Nagano Prefecture, we held a kickoff seminar and a three-part series of disaster mitigation seminars in 2022.

Testosterone affects several organs in the body and is very important for male well-being. Aging men with late-onset hypogonadism (LOH) experience physiologic, psychiatric, and sexual symptoms related to a decline in the serum concentration of testosterone with age. However, it is well-known that the extent of the decline in testosterone concentration does not correlate with the severity of LOH-related symptoms. Therefore, it is difficult to diagnose and treat patients with LOH. In addition, the symptoms, response to testosterone replacement therapy (TRT), and medical insurance coverage differ among ethnicities and countries. The evaluation of testosterone is essential for the diagnosis and treatment of LOH. The effects of testosterone are determined not only by the serum testosterone concentration but also by the androgen receptor sensitivity. A low number of glutamine repeats is indicative of high androgenic activity, and the number shows ethnicity-related differences (fewer in African American than in Caucasian people and more in East Asian people). The diagnosis of LOH is typically made using subjective symptoms and the serum testosterone concentration. The Aging Male Symptoms scale is widely used to evaluate the symptoms. The normal range of total testosterone concentration varies around the world; therefore, clinicians should follow the guidelines of their regional academic society. The principal treatment for LOH is TRT. There are many types of TRT and other treatment strategies are also available. Thus, physicians should treat LOH according to each patient’s situation, considering related disorders, such as diabetes, osteoporosis, metabolic syndrome, and depression.

The present study is the first large-scale, multicenter survey on modified electroconvulsive therapy (ECT) in Japan. We aimed to comprehend the current implementation status of ECT based on the annual reports of 2016 from 21 facilities that were certified by the Japanese Society of General Hospital Psychiatry as ECT certified facilities and participated in this multicenter observational study. We investigated the distributions of diagnosis, gender, and age of patients receiving acute-phase ECT, and the efficacy, safety, and adverse events. The number of patients receiving acute-phase ECT was 524. According to International Classification of Diseases, 10th Revision, 344 patients (65.6%) were diagnosed with mood disorders (F3), 156 patients (29.8%) were diagnosed with schizophrenia and with schizotypal and delusional disorders (F2), and 151 subjects were male and 334 subjects were female. The mean age of patients was 60.4 years (SD 15.9), and patients 60 years or older accounted for 57.9%. Efficacy did not significantly differ between diagnoses, nor between genders. However, the efficacy rate was significantly higher in elderly patients. In acute-phase ECT, 4 severe adverse events occurred. Our multicenter study confirmed that F3 (mood disorders) was the most common indication for ECT at 66%, followed by F2 (schizophrenia, schizotypal, and delusional disorders) at 30%, with no difference in efficacy, indicating that ECT is still performed as 1 of the treatment options for schizophrenia in Japan. The present results suggested that accumulation of annual data from multiple centers can be useful for more effective and safer ECT practices.

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Alice in Wonderland syndrome (AIWS) is extremely rare, occurring more often in young individuals than in older adults. Symptoms of this syndrome typically include an altered body image, size perception, and time perception. However, the pathophysiology and lesions responsible for this syndrome remain unclear. In most cases, specific lesions cannot be identified using computed tomography or magnetic resonance imaging. Two patients with isolated cortical venous thrombosis in the right occipital area experienced transient visual symptoms of AIWS. Furthermore, a literature search indicated that AIWS with visual distortions is associated with right occipital lobe lesions, supporting the findings of our study.

This prospective multicenter study aimed to determine the effects of human herpesvirus-6B (HHV-6B) reactivation on central nervous system (CNS) function in cord blood transplant (CBT) recipients. Our focus was to track HHV-6B reactivation and evaluate its association with delirium and cognitive function, specifically in the domains of verbal memory, attention/processing speed, and quality of life (QOL). A cohort of 38 patients participated in this study. Of the 37 patients evaluated, seven (18.9%) developed delirium, with six of these cases emerging after HHV-6B reactivation (median lag, 7 days). Evaluation of verbal memory showed that the final trial score for unrelated words at 70 days after transplantation was significantly lower than that before preconditioning (P = 0.004) among patients (n = 15) who experienced higher-level HHV-6B reactivation (median or higher maximum plasma HHV-6 DNA load for participating patients). Patients without higher-level reactivation did not show significant declines in verbal memory scores. QOL was assessed using the 36-item Short-Form Health Survey, and the social functioning score 1 year post-transplantation was significantly lower in patients who experienced higher-level HHV-6B reactivation than in those who did not. Our findings suggest that higher-level HHV-6B reactivation can detrimentally affect certain cognitive functions in CBT recipients.Supplementary InformationThe online version contains supplementary material available at 10.1007/s12185-024-03714-2.

Abstract Chronic myelomonocytic leukaemia (CMML) is a haematological malignancy with a poor prognosis. Allogeneic haematopoietic stem cell transplantation remains the only curative approach. Without human leukocyte antigen-matched related sibling donors, the optimal alternative donor has yet to be established. Although unrelated bone marrow transplantation (UBMT) has been extensively studied, cord blood transplantation (CBT) for CMML remains largely unexplored. This nationwide retrospective study compared the outcomes of UBMT and single-unit umbilical CBT in patients with CMML. This study included 118 patients who underwent their first allo-HSCT during 2013–2021. Of these, 50 received BMT (UBMT group), while 68 underwent CBT (CBT group). The primary endpoint was the 3-year overall survival (OS). There were comparable 3-year OS rates between the UBMT (51.0%, 95% confidence interval [CI]: 34.1–65.5%) and CBT (46.2%, 95% CI: 33.2–58.1%; P = 0.60) groups. In the inverse probability of treatment weighting analysis, CBT did not show significantly improved outcomes compared with UBMT regarding the 3-year OS rate (hazard ratio 0.97 [95% CI: 0.57–1.66], P = 0.91). Thus, CBT may serve as a promising alternative to UBMT for patients with CMML. Further research is necessary to optimise transplantation strategies and enhance outcomes in patients with CMML undergoing CBT.