Pulmonary Arterial Hypertension in Neurofibromatosis Type 1: A Case with a Novel NF1 Gene Mutation.

Neurofibromatosis type 1 (NF1) is an autosomal dominant multi-organ disease. The clinical manifestations include not only skin lesions and malignant tumors but also lung complications, including pulmonary arterial hypertension (PAH). However, the association between gene mutations in NF1 and the occurrence of PAH has not yet been elucidated.

Regression of left ventricular hypertrophy after tafamidis therapy in a patient with transthyretin amyloidosis variant.

Transthyretin amyloidosis (ATTR) variant is a life-threatening hereditary disease predominantly affecting the peripheral nervous system and heart. Tafamidis, which prevents the deposition of amyloid by stabilizing transthyretin, is available for the treatment of neuropathy and cardiomyopathy of ATTR. However, whether tafamidis could eliminate established amyloid deposits and improve cardiac function remains unknown.

Safety and Effectiveness of Letermovir in Allogenic Hematopoietic Stem Cell Transplantation Recipients: Interim Report of Post-marketing Surveillance in Japan.

Objective: Since May 2018, a 6-year post‑marketing surveillance (PMS) has been underway to evaluate the safety and effectiveness of letermovir for cytomegalovirus (CMV) prophylaxis in Japanese patients with allogenic hematopoietic stem-cell transplantation (allo-HSCT). The interim PMS data for 461 patients collected as of March 2021 are reported in this publication.

Methods: The case report forms (CRFs) were drafted in part by the Japanese Data Center for Hematopoietic Cell Transplantation (JDCHCT) using data elements in the Transplant Registry Unified Management Program (TRUMP) and sent to individual HSCT centers to decrease burden of reporting.

Healthcare resource utilization and economic burden of antifungal management in patients with hematologic malignancy in Japan: a retrospective database study.

Objective: To examine treatment patterns of real-world antifungal management of patients at high risk of invasive fungal infections (IFI) and evaluate healthcare resource utilization and costs associated with antifungal management of IFIs in Japan.

Methods: This retrospective, observational study extracted data from a hospital-based claims database for patients in Japan who either (a) underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT), or (b) were hospitalized with acute myeloid leukemia (AML) or myelodysplastic syndromes (MDS) and received chemotherapy during the study period of January 2010 to January 2019.

Results: 863 patients were included in the allo-HSCT cohort and 4498 patients were included in the AML/MDS cohort.

Relationship between Pain Scores and EORTC QLQ-C15-PAL Scores in Outpatients with Cancer Pain Receiving Opioid Therapy.

Cancer pain is one of the most frequent and distressing symptoms associated with cancer and has a serious impact on the QOL of patients. However, inadequate pain treatment has also been reported in outpatients with cancer pain. The aims of this study were (1) to evaluate the relationship between pain intensity using the Numerical Rating Scale (NRS) and QOL scores using the Japanese version of the European Organization for Research and Treatment of Cancer (QOL Questionnaire Core 15 for Palliative Care (QLQ-C15-PAL)), and (2) to investigate their association with various pain patterns, especially with baseline and breakthrough pain.

The disease burden of mucormycosis in Japan: results from a systematic literature review and retrospective database study.

Objective: To evaluate outcomes, healthcare resource utilization, and costs associated with mucormycosis in inpatient settings in Japan.

Methods: We performed a systematic literature review, followed by a retrospective database study using hospital health claims for patients in Japan hospitalized with a diagnosis of mucormycosis between January 2010 and January 2019. Outcomes assessed included duration of index hospitalization; index stay mortality; hospital readmission within 30, 60, and 90 days after index hospitalization discharge; drug/treatment utilization and patterns; number of patients examined for mucormycosis during the index hospitalization; and index stay inpatient costs.

Effects of Culture Conditions (Microplate and Solvent) on in vitro Antifungal Activity of Caspofungin Against Candida Species.

Effects of the type of microplates and solvent for preparation of caspofungin (CPFG) on antifungal susceptibility testing of CPFG against clinical isolates of Candida albicans, Candida glabrata, and Candida krusei (20 strains each) by broth microdilution method according to the Clinical and Laboratory Standards Institute were evaluated. When CPFG was dissolved in water, MICs against the three Candida species decreased 3.1-6.

The clinical and economic burden of cytomegalovirus management post allogeneic hematopoietic stem cell transplantation in Japan - a retrospective database study.

Reactivation of cytomegalovirus (CMV) infection is a major threat and it causes significant morbidity and mortality after allogeneic hematopoietic stem cell transplantation (allo-HSCT). There remains, however, a paucity of evidence regarding the economic burden of current CMV management in Japan. The aim of this study is to characterize the healthcare resource utilization (HCRU) and cost incurred for CMV management post allo-HSCT, using a Japanese hospital claims database.

Lambda-like J wave due to acute myocardial infarction of the diagonal branch.

The culprit lesion of acute myocardial infarction could be predicted by electrocardiogram findings. However, we experienced some cases with coronary angiographic finding in the area of ST-T elevation that was different from that predicted. The lambda-like J wave could be caused by ischemia although the mechanism has not been fully elucidated.

Association of Decreased Docosahexaenoic Acid Level After Statin Therapy and Low Eicosapentaenoic Acid Level with In-Stent Restenosis in Patients with Acute Coronary Syndrome.

Aim: It is speculated that statin therapy modulates the synthesis of polyunsaturated fatty acids (PUFA), including eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA). However, the data available on the effects of statin therapy on the serum levels of PUFA and the subsequent impact on in-stent restenosis (ISR) in patients with acute coronary syndrome (ACS) are limited.

Methods: A total of 120 ACS patients who received emergent coronary stent implantation, follow-up coronary angiography to evaluate ISR, and new statin therapy were enrolled.

Effect of Continuous Pharmacist Interventions on Pain Control and Side Effect Management in Outpatients with Cancer Receiving Opioid Treatments.

For improving the QOL of patients diagnosed with cancer, early palliative care is recommended, aiming to minimize pain and opioid-induced side effects. Herein, we evaluated the effect of continuous interventions for pain management and opioid-induced side effects in outpatients with cancer. Pharmacists continuously performed interventions on patients on their hospital visits, starting from the first visit for opioid introduction to intervention via telephone.

Edoxaban improves acute venous thromboembolism while preserving protein C and protein S levels.

Background: It is well known that warfarin inhibits the synthesis of vitamin K-dependent anticoagulants, including thrombin, protein C and S, and factor Xa, leading, paradoxically, to an initial hypercoagulable state. Edoxaban, a direct inhibitor of activated factor X is widely used for the treatment of acute venous thromboembolism (VTE). However, the effect of edoxaban on circulating coagulation factors, in patients with acute VTE, remains unknown.

Response Prediction and Influence of Tolvaptan in Chronic Heart Failure Patients Considering the Interaction of the Renin-Angiotensin-Aldosterone System and Arginine Vasopressin.

The renin-angiotensin-aldosterone system (RAAS) and arginine vasopressin (AVP) regulate body fluids. Although conventional diuretics have been used for treating heart failure, they activate RAAS and exacerbate renal function. Tolvaptan, a newly developed vasopressin-2 receptor antagonist, elicits aquaresis and improves volume overload in heart failure patients, however, the predictors of tolvaptan effectiveness and the influence on the RAAS and renal function according to tolvaptan therapy are not established.

Serum concentration of eicosapentaenoic acid is associated with cognitive function in patients with coronary artery disease.

Background: Recent studies have shown that intake of n-3 polyunsaturated fatty acids (PUFAs) is associated with reduced risk of cognitive impairment and coronary artery disease (CAD); however, it is currently unknown whether reduced serum n-3 PUFA is associated with cognitive impairment in patients with CAD.

Methods: We retrospectively evaluated cognitive function with the mini-mental state examination (MMSE), serum levels of PUFAs (including eicosapentaenoic acid [EPA], docosahexaenoic acid [DHA], dihomogammalinolenic acid [DGLA], and arachidonic acid [AA]), cardiovascular risk factors (hypertension, dyslipidemia, diabetes mellitus, cerebrovascular disease, and history of current/previous smoking), and parameters of cardiac function (left ventricular ejection fraction and brain natriuretic peptide levels) in 146 Japanese CAD patients. The associations between the MMSE scores and the other parameters were evaluated.

Perspectives of Japanese mothers with severe mental illness regarding the disclosure of their mental health status to their children.

This study examined Japanese mothers with severe mental illness and their perspectives about disclosing their mental health status to their children. Seventy-four outpatients diagnosed with schizophrenia or mood disorders were recruited. We utilized a cross-sectional design and a self-report questionnaire.

Relationship between positive self-recognition of maternal role and psychosocial factors in Japanese mothers with severe mental illness.

Mothers with mental illness have positive self-recognition of maternal role (PM), and it is important for parenting. The purpose of this study was to determine the psychosocial factors related to the PM. We recruited a total of 74 women diagnosed as having schizophrenia or mood disorders according to the DSM-IV-TR and who had minor children.

Narratives by Japanese mothers with chronic mental illness in the Tokyo metropolitan area: their feelings toward their children and perceptions of their children's feelings.

Women with mental illness generally have normal fertility rates. Mothers with mental illness think that their children are important for their lives but also experience difficulties in relating to their children. Therefore, it is important to understand the mothers' perception of what experiences influence them or their parenting practices in relationships with their children.