Objective To compare the effect of the FIB4 index on skeletal muscle mass (SMM) loss (i.e., pre-sarcopenia) with that of three other liver fibrosis markers [FIB3 index, aminotransferase to platelet ratio index (APRI), and platelet count] or sarcopenia-related factors such as age and body mass index (BMI) in patients with metabolic dysfunction associated with steatotic liver disease (MASLD, 2,093 men and 1,280 women; median age, 55 years).

Methods An SMM decrease was defined as a fat-free index, as tested by a bioelectrical impedance analysis, <18 kg/m² in men and <15 kg/m² in women. We compared the diagnostic ability of the above-mentioned six markers to induce an SMM decrease.

Results The median BMI was 25.0 kg/m². The median FIB4 index, FIB3 index, APRI, and platelet count were 0.99, -0.40, 2.93 and 25.2 × 10⁴/μL. The prevalence of SMM decrease in men and women was 21.7% (455/2,093) and 16.7% (214/1,280), respectively. For all cases, the percentage of a decreased SMM was 15.0% (372/2,479) in cases with FIB4 index <1.3, 32.1% (268/834) in cases with 1.3< FIB4 index <2.67 and 48.3% (29/60) in cases with FIB4 index >2.67 (overall p<0.0001). In almost all subgroup analyses, the cases were well-stratified according to the FIB4 index. In all cases, the area under the receiver operating characteristic curve (AUC) for SMM decrease was highest for BMI (AUC=0.94), followed by the FIB4 index (AUC=0.67). In all subgroup analyses, except for BMI ≥25 kg/m², the FIB4 index had the second highest AUC for SMM decrease after BMI.

Conclusion The FIB4 index may be useful for predicting pre-sarcopenia in patients with MASLD.

Objective Optimal medical therapy (OMT) is recommended for the secondary prevention of acute myocardial infarction (AMI) after percutaneous coronary intervention (PCI). We investigated the relationship between the OMT prescription rate at hospital discharge and clinical outcomes in patients with AMI who underwent successful PCI.

Methods We enrolled 294 consecutive AMI patients who underwent successful emergency PCI between January 2017 and December 2020. The patients were divided into two groups based on their medications at discharge: OMT, defined as a combination of statins, beta-blockers, angiotensin-converting enzyme inhibitors, or angiotensin receptor blockers; and non-OMT, defined as the absence of at least one of the aforementioned agents. The primary outcome measure was major adverse cardiovascular events (MACEs), defined as the composite of all-cause death, nonfatal myocardial infarction, nonfatal stroke, and heart failure requiring hospitalization.

Results According to prescription data, 186 patients (63.3%) were prescribed OMT at discharge. During a median follow-up period of 957 (591-1,308) days, 45 patients developed MACEs. Kaplan-Meier curves showed a significantly lower incidence of MACE in the OMT group than in the non-OMT group (log-rank p<0.001). In the multivariate analysis, OMT remained independently associated with a reduced risk of MACE (hazard ratio, 0.47; 95% confidence interval: 0.25-0.88; p=0.017).

Conclusion Fundamental OMT at discharge was associated with a reduced risk of MACE in AMI patients after successful PCI. Therefore, OMT may be necessary to improve the clinical outcomes of patients with AMI after discharge.

Objective Hepatitis B (HB) is a significant global public health concern, and the World Health Organization has emphasized the importance of HB vaccination programs. Currently, there are only two commercially available HB vaccines in Japan: HEPTAVAX^®-II and Bimmgen^®. Antibody acquisition and maintenance are crucial for HB vaccinations.

Methods In this study, we used a modified version of HEPTAVAX^®-II (mpHBV) with a doubled aluminum-to-phosphate ratio of the adjuvant aluminum hydroxyphosphate sulfate to assess the vaccine's antibody acquisition rate and antibody levels six months post-vaccination compared to conventional HEPTAVAX^®-II.

Patients A total of 741 second-year students from Showa University School of Medicine, Dentistry, and Pharmacy were included in this study.

Results The antibody acquisition rate (≥10 mIU/mL) for mpHBV was 84% (321/382) with a confidence interval (CI) of 0.803-0.877, while that for HEPTAVAX^®-II was 68.5% (246/359) with a CI of 0.637-0.734. The geometric mean titer (mIU/mL) for mpHBV was 55.0 mIU/mL with a CI of 46.9-64.5, while that for HEPTAVAX^®-II was 24.1 mIU/mL CI 20.7-28.2. Both the antibody acquisition rate and geometric mean titer were significantly higher for mpHBV than for HEPTAVAX^®II.

Conclusion The adjuvant-modified HB vaccine was effective in maintaining vaccine antibody acquisition rates and levels. Adjuvants are important for antibody acquisition and maintenance during vaccine production.

We herein report a 41-year-old woman with BRAFV600E-mutated intrahepatic cholangiocarcinoma and a concurrent loss of function in NF2. The patient had a large liver tumor, multiple lymph nodes, and metastases to the lungs and pleura. She underwent six cycles of gemcitabine, cisplatin, and durvalumab but was refractory to this treatment. Comprehensive genomic profiling revealed a BRAFV600E mutation along with a loss of function in NF2. The patient was treated with a combination of oral dabrafenib and trametinib therapy. After two months, imaging showed reduced pleural effusion, tumor shrinkage, symptom resolution, and improved performance status, without significant side effects.

A 51-year-old man was diagnosed with gastric mucosa-associated lymphoid tissue (MALT) lymphoma after presenting with fold convergence on upper gastrointestinal radiography and nausea. He tested positive for Helicobacter pylori, and eradication therapy decreased the size of the gastric MALT lymphoma. Eighteen years later, whitish subepithelial lesions were discovered in the middle thoracic esophagus which were diagnosed to be esophageal MALT lymphoma. Six months later, the left tonsillar swelling was identified as diffuse large B-cell lymphoma (DLBCL). Complete remission was achieved after treatment with rituximab and chemotherapy. To our knowledge, this is the first reported case of metachronous gastric and esophageal MALT lymphoma with tonsillar DLBCL.

A 69-year-old man underwent upper and lower gastrointestinal endoscopic examinations for a detailed evaluation of diarrhea that had persisted for several months, which revealed multiple polyposis in the stomach, duodenum, colon, and terminal ileum. The histopathological findings also led to a diagnosis of Cronkhite-Canada syndrome (CCS). On admission, the patient had hypoalbuminemia and electrolyte abnormalities, including hypokalemia, and developed paroxysmal atrial fibrillation (Paf), although there were no apparent organic lesions in the heart. The patient's sinus rhythm was restored by electrical cardioversion, steroid therapy for CCS improved diarrhea symptoms and endoscopic findings, and the electrocardiogram findings were normal.

There is a lack of established methods to explore the etiology of sudden cardiac arrest. We herein report sudden cardiac arrest as pulseless electrical activity in a young woman triggered by coronary artery spasm. There were no health issues except for situs inversus totalis, but the patient experienced sudden cardiac arrest after suffering chest pain. ST-segment depression was observed in leads V₃-V₆ on electrocardiography. However, urgent angiography did not reveal any coronary stenosis. Cardiac magnetic resonance imaging (MRI) revealed myocardial edema localized to the inferior and posterior segments without evidence of late gadolinium enhancement. Additionally, a provocation test with acetylcholine confirmed a coronary artery spasm. Inexplicable sudden cardiac arrest may result in missing the chance to detect hidden cardiac disease. Cardiac MRI may help to elucidate the etiology of sudden cardiac arrest.

A 71-year-old woman was diagnosed with anaplastic lymphoma kinase-positive metastatic lung adenocarcinoma staged as cT1cN3M1c (8th edition). Treatment with lorlatinib (100 mg) was initiated on a daily basis. Three weeks after the first dose, the patient was hospitalized because of lorlatinib-induced noncardiogenic pulmonary edema. The patient's condition improved with diuretic therapy, and lorlatinib was rechallenged at a reduced dose. Follow-up computed tomography revealed a partial response. This is a rare case of non-cardiogenic pulmonary edema as an adverse effect of lorlatinib.

TAFRO syndrome is a rare, severe, and sometimes refractory condition characterized by thrombocytopenia, anasarca, a fever, reticulin myelofibrosis, and organomegaly. Although several treatment options have been proposed for this condition, there are few reported cases in the literature on successful treatment using cyclophosphamide in patients with TAFRO syndrome. We herein report two cases of refractory TAFRO syndrome successfully treated with intravenous cyclophosphamide. In both cases, after the initial treatment with high-dose glucocorticoids and tocilizumab failed, the administration of intravenous cyclophosphamide led to significant improvement. These cases suggest that intravenous cyclophosphamide may be a viable therapeutic option for refractory TAFRO syndromes.

MYC amplification and overexpression are uncommon in acute myeloid leukemia (AML). An 82-year-old man developed leukocytosis during monoclonal gammopathy of renal significance. A chromosomal analysis revealed 46,XY,+r(8)[20]. Amplified MYC signals were detected on chromosome 8. The patient was diagnosed with AML and administered venetoclax and azacitidine. After the third course, clones with ring chromosome 8 had decreased in number, but clones unrelated to t(8;21)(q22;q22) had subsequently emerged. After the sixth course, the white blood cell count had markedly increased, and a chromosome analysis showed replacement of ring chromosome 8 with 46,XY,t(8;21)[20]. This case highlights the role of MYC amplification and overexpression in AML and suggests that BCL2 inhibition is a potential treatment.

A 31-year-old female presented at our hospital with fatigue and subcutaneous bleeding. Blood tests revealed hemolytic anemia. The patient had Coombs-negative normocytic anemia and thrombocytopenia; therefore, thrombotic thrombocytopenic purpura was ruled out. On day two, we noticed that the eating habits of the patient, hypersegmented neutrophils, and megaloblastic changes suggested malnutrition; therefore, we initiated vitamin supplementation. On day six, the vitamin C levels were <0.2 μg/mL, and the patient was therefore diagnosed with scurvy. Scurvy can mimic hemolytic anemia by causing normocytic megaloblastic anemia with a high reticulocyte count. Hypersegmented neutrophils and a detailed medical history are important for making a differential diagnosis.

Relapsing polychondritis (RP) is a rare autoimmune disease that may involve the central nervous system. We herein present the first reported case of hypertrophic pachymeningitis (HP) in an RP patient with a concurrent Klebsiella pneumoniae infection. A 48-year-old man developed transient left-sided weakness and numbness, and brain MRI showed diffuse pachymeningitis. Cultures from a dural biopsy identified K. pneumoniae, and RP was diagnosed using an auricular cartilage biopsy. Treatment with corticosteroids and infliximab resulted in symptom resolution. This case highlights the importance of considering bacterial infections in HP associated with RP, while also providing insight into its pathophysiology.

A 46-year-old woman presented with acute disturbance of consciousness, ataxia, and urinary retention. Although Hashimoto's encephalopathy was initially suspected due to the detection of serum anti-NH₂-terminal α-enolase (NAE) antibodies, glial fibrillary acidic protein (GFAP)-astrocytopathy was also considered probable due to urinary retention. An additional examination revealed positive results for both anti-GFAP and anti-NAE antibodies. This case highlights the importance of considering antibodies to be examined based on clinical features, even in the presence of antibodies.

Electroconvulsive therapy (ECT), an established treatment for psychiatric disorders, shows promise in alleviating both motor and non-motor symptoms in patients with Parkinson's disease (PD). We herein report a case of PRKN-associated PD presenting with characteristic psychosis and dystonic voice, which was effectively managed with maintenance ECT. A genetic analysis initially identified a single heterozygous PRKN deletion using multiple ligation-probe amplification. Subsequent Oxford Nanopore technology long-read sequencing revealed a second pathogenic variant, confirming a compound heterozygous PRKN mutation. This case highlights the utility of long-read sequencing in resolving genetic diagnoses and the potential of maintenance ECT for managing complex PD phenotypes.

Patients with ulcerative colitis (UC) treated with tumor necrosis factor (TNF) inhibitors can sometimes develop articular manifestations, but rarely rheumatoid arthritis (RA). We herein report the case of a 70-year-old woman who developed seropositive RA during adalimumab therapy for UC diagnosed 11 years previously. A literature review, including 30 patients with both UC and RA, identified three patients (10%) who developed RA after their diagnosis of UC. Notably, the present study is the only case in which RA developed during TNF inhibitor therapy for UC. This result demonstrates that arthritis in patients with UC can be caused by RA despite TNF inhibitor administration.

A misdiagnosis of syncope as a seizure can lead to catastrophic outcomes, and thus should be avoided. We herein present the cases of two young sisters who experienced transient loss of consciousness with convulsions and were diagnosed with epilepsy. After discovering that their asymptomatic mother had type-2 long QT syndrome (LQT2), it was found that the younger sister had LQT2, while the older sister did not. Although the 12-lead ECG of the younger sister showed notched T-waves, the corrected QT interval (422 ms) was normal, which precluded a diagnosis of LQT2. These cases provide clues to distinguish between LQT2-related arrhythmic events and epileptic events.