Objective Risk factors for progression from non-severe to severe acute cholangitis (AC) remain unclear. This study aimed to identify predictors of disease progression with a particular focus on the prognostic nutritional index (PNI).

Methods We retrospectively analyzed 239 patients with Grade I or II AC who underwent endoscopic biliary drainage within 24 h of hospital presentation. The patients were classified into 2 groups: progression (n=18) and non-progression (n=221). The clinical characteristics were compared between the two groups.

Results The progression group had a higher proportion of Grade II AC (83.3% vs. 47.5%, p=0.006) and received immunosuppressive therapy more frequently (27.8% vs. 5.9%, p=0.006) but had a lower PNI (36.67 vs. 39.81, p=0.003) than the non-progression group. The proportion of patients with low PNI (<38) was significantly higher in the progression group than in the non-progression group (66.6% vs. 37.6%, p=0.023). In addition, the mortality rate was significantly higher in the progression group than in the non-progression group (11.1% vs. 0.9%, p=0.029).

Conclusion Progression to severe AC may be associated with Grade II AC, immunosuppressive therapy, and a low PNI. Even after early biliary drainage, patients presenting with these conditions require close clinical monitoring.

Objective Sodium-glucose cotransporter-2 inhibitors (SGLT2is) induce body weight and muscle loss due to the excretion of glucose in the urine, which may worsen frailty in patients with heart failure (HF). However, the impact of SGLT2is on frailty-related events in patients with HF has not been fully elucidated.

Methods In this analysis of global electronic health records, patients with HF and type 2 diabetes mellitus (T2DM) who received SGLT2is were compared to those who received sitagliptin treatment from 2016 to 2021. A composite endpoint of death or new incidence of frailty-related events, including 1) mobility impairments, 2) falls and fractures, 3) pressure ulcers and weight loss, 4) incontinence, 5) dependency and caregiving, 6) dementia and delirium, and 7) anxiety and depression, was analyzed using propensity score (PS) matching.

Results After PS matching, 8,148 of the 16,989 patients in the SGLT2i group were matched to 8,148 of the 18,278 patients in the sitagliptin group. The incidence of the composite endpoint and its components was lower in the SGLT2i group than in the sitagliptin group [composite endpoint, 68.1% vs. 72.6%, hazard ratio (HR) 0.85 (0.82-0.89), p<0.001; mortality, 20.4% vs. 28.2%, HR 0.69 (0.65-0.74), p<0.001; frailty-related events, 63.2% vs. 66.6%, HR 0.87 (0.84-0.91), p<0.001]. All components of frailty-related events were observed less frequently in the SGLT2i group.

Conclusion SGLT2is correlated with a lower incidence of death and frailty-related events than sitagliptin in patients with HF and T2DM.

Objective It is important to identify the causative parathyroid gland before parathyroidectomy in patients with primary hyperparathyroidism; however, there are some cases of preoperative localization failure. This study assessed the characteristics of patients with failure to localize the causative parathyroid gland.

Methods This study included 114 patients with primary hyperparathyroidism. Physical and biochemical indices were measured, and neck ultrasonography, technetium-99m methoxy isobutyl isonitrile scintigraphy, and magnetic resonance imaging were performed. Localization of the causative gland was confirmed by consensus among multiple physicians.

Results The causative parathyroid glands were localized in 83 patients (73%) with primary hyperparathyroidism. Successful localization was defined as cases in which preoperative imaging accurately identified the causative parathyroid gland, and localization failure included cases with no preoperative identification or those requiring additional resections due to incorrect localization. Most preoperative localization diagnoses in the patients who underwent parathyroidectomy were confirmed to be accurate. Patients with localization failure were significantly older and exhibited lower levels of serum chloride, calcium, intact parathyroid hormone, and urinary calcium values, along with higher serum phosphorus levels, greater phosphate reabsorption, and a smaller tumor size than those with successful localization. Even after adjusting for the age, sex, and body mass index, intact parathyroid hormone levels were significantly associated with successful localization.

Conclusion A relatively old age; low levels of serum chloride, calcium, intact parathyroid hormone, and urinary calcium; high levels of serum phosphorus; phosphate reabsorption; and a relatively small tumor size were associated with localization failure of the causative parathyroid gland.

Objectives To investigate the association between malnutrition status on admission and short-term swallowing recovery in acute ischemic stroke.

Methods We retrospectively analyzed patients with acute ischemic stroke and dysphagia who were admitted to our institute between January 2018 and December 2020. The swallowing function was assessed using the Food Oral Intake Scale (FOIS), and patients with initial FOIS levels of 1-3, which represent tube-dependent nutrition, were enrolled. Poor swallowing recovery was defined as a FOIS score of 1-3 on day 14. The nutritional status at admission was assessed using the Geriatric Nutritional Risk Index (GNRI) and categorized as follows: >98, no nutritional risk; 92-98, mild nutritional risk; 82-92, moderate nutritional risk; and <82, severe nutritional risk.

Results Among 189 patients (median age, 85 years; male, 39%), 123 (65%) had poor swallowing recovery. This group had a higher NIHSS score, a lower rate of improvement with reperfusion therapy, a higher rate of aspiration pneumonia, and a lower GNRI. In a multivariate logistic regression analysis, a lower GNRI was independently associated with poor swallowing recovery [per-point decrease; adjusted odds ratio (OR) 1.03, 95% confidence interval (CI) 1.00-1.07]. Furthermore, when "severe" and "moderate" nutritional risk were analyzed as a single class, moderate or severe nutritional risk (GNRI <92) was independently associated with poor swallowing recovery (adjusted OR 2.78, 95% CI 1.14-6.67), relative to no nutritional risk (GNRI >98).

Conclusion Lower GNRI at admission was independently associated with poor short-term swallowing recovery in patients with acute ischemic stroke.

Objective This study aimed to investigate whether the swallowing function deteriorates more significantly following coronavirus disease 2019 (COVID-19) infection in comparison to other viral respiratory infections in patients with mild to moderate (grade 2) COVID-19.

Methods We conducted a propensity score-matched cohort study at St. Luke's International Hospital in Tokyo, Japan, from January 2010 to March 2024. Elderly patients (≥70 years) admitted for viral respiratory infections, including influenza and COVID-19, were included. Patients with suspected bacterial infections, patients who were incapable of oral intake at admission (including those with enteral nutrition and gastrostomy), and patients who died during hospitalization were excluded. The primary outcome was deterioration in the swallowing function, defined as downgrade in food texture category from admission to discharge.

Results During the study period, 505 COVID-19 patients and 242 patients with other viral infections were admitted. The mean age was 81.7 years (±7.6) and 401 patients (53.7%) were male. Patients with COVID-19 showed a significantly higher rate of swallowing deterioration in comparison to patients with other viral infections (44.7% vs. 36.6%, p=0.04). Specifically, among patients who consumed a full diet at admission, those with COVID-19 had a higher rate of swallowing deterioration than those with other viral infections (41.9% vs. 31.7%, p<0.01).

Conclusion COVID-19 was associated with a significant higher rate of downgrade in the food texture category in comparison to other viral respiratory infections, suggesting a potential impairment in the swallowing function. Given that this deterioration may contribute to prolonged hospitalization in COVID-19 patients, early intervention aimed at restoring and supporting swallowing function is warranted.

Objective Ewing sarcoma (ES) is a highly aggressive malignancy with a poor prognosis, particularly in metastatic disease. While localized disease treated with multimodal therapy achieves favorable survival rates, metastatic disease at presentation has a significantly poorer prognosis. The molecular hallmark of ES is the presence of EWSR1-ETS family fusion genes, with EWSR1-FLI1 being the most common gene.

To investigate the impact of additional EWSR1 rearrangements on treatment outcomes in ES by analyzing a national cancer genomic database.

Methods We conducted an exploratory retrospective analysis of the cancer genomic status and treatment response using the Center for Cancer Genomics and Advanced Therapeutics (C-CAT) database.

Patients This study analyzed clinical genomic testing results from the C-CAT database, focusing on bone and soft tissue tumors identified as ES (n=90). Representative cases were selected to illustrate the clinical implications of the molecular findings.

Results An analysis of the C-CAT database revealed that a subset of ES cases showed additional EWSR1 rearrangements beyond the primary fusion gene. Cases with coexisting rearrangements demonstrated higher rates of disease progression with first-line chemotherapy than those with isolated fusions. This finding was exemplified in our institutional experience, where ES with an isolated EWSR1-FLI1 fusion showed a good response to standard chemotherapy, whereas a case with an additional ETS1-EWSR1 rearrangement exhibited primary resistance to multiple lines of therapy.

Conclusion These findings suggest that coexisting EWSR1 rearrangements may have important prognostic implications and warrant further investigation to optimize treatment strategies in resistant cases.

Primary gastric alveolar rhabdomyosarcoma (RMS) is an extremely rare condition. We report a case of a 32-year-old man who presented with abdominal distension and anorexia. Imaging revealed a gastric mass with peritoneal dissemination and vertebral metastasis. Endoscopy revealed a large, ulcerative lesion. Histopathology revealed small round cells in an alveolar pattern that were positive for desmin and myogenin. Despite the initiation of multimodal therapy, the patient developed septic shock and died 10 days after treatment. This case highlights the aggressive nature of primary gastric alveolar RMS and emphasizes the importance of integrated diagnostic approaches, including histopathological, immunohistochemical, and molecular analyses, for its accurate diagnosis and appropriate management.

An impaired intestinal barrier exposes the microbiota to gut immune cells, triggering cytokine responses that cause Crohn's disease (CD). Barrier dysfunction is a prerequisite for CD development. However, the effects of biological therapies on barrier integrity remain poorly understood. We aimed to present a case of CD refractory to adalimumab and risankizumab. Treatment with infliximab improved refractory ulcers, and this effect was associated with alterations in the mucosal mRNA expression of claudins, cell-cell adhesion molecules that contribute to the maintenance of barrier integrity. These findings suggested that infliximab induces CD remission by regulating the expression of claudin.

A central venous (CV) port is an important device for cancer treatment, as it enables safe venous access. We herein report the case of an 82-year-old man with recurrent gastric cancer who experienced J-tip spring guidewire entrapment caused by the Chiari network during CV port implantation. The guidewire was pulled away, with a piece of tissue at its tip. The tissue comprised cardiomyocytes, stratified collagenous fibers, and elastic fibers. The condition was diagnosed as a Chiari network. Based on our experience, operators should be aware of the risk of Chiari network involvement even with a normal transthoracic echocardiogram result.

Becker muscular dystrophy (BMD)-associated cardiomyopathy may be overlooked in patients with dilated cardiomyopathy (DCM). We herein report a case of cardiomyopathy related to BMD in which anesthesia-induced rhabdomyolysis led to a diagnosis of BMD. The patient experienced anesthesia-induced rhabdomyolysis during cardiac resynchronization therapy. The patient became inotrope-dependent, and a left ventricular assist device was safely implanted by considering the use of anesthetic agents to avoid rhabdomyolysis. The abnormal immunohistochemical staining pattern of dystrophin protein in the myocardium and dystrophin gene mutation confirmed the diagnosis of BMD. BMD should thus be considered in patients with DCM to prevent perioperative complications including rhabdomyolysis.

Gastrojejunocolic fistula is a rare but serious complication of pancreaticoduodenectomy that can lead to malabsorption and severe undernutrition. A 59-year-old man presented with leg edema and significant weight loss 8 years after undergoing subtotal stomach-preserving pancreaticoduodenectomy. After preoperative nutritional improvement (guided by indirect calorimetry), the patient underwent fistula resection and Roux-en-Y reconstruction. The patient was discharged on postoperative day 37, and his nutritional parameters remained adequate at the one-year follow-up. This case demonstrates the validity of indirect calorimetry in optimizing nutrition, preventing refeeding syndrome, and managing perioperative care in patients with gastrojejunocolic fistulas after pancreaticoduodenectomy.

A 58-year-old man was admitted to our hospital in April 2013 with elevated serum creatinine levels, proteinuria, and hematuria. A renal biopsy revealed membranoproliferative glomerulonephritis-like changes. Immunofluorescence showed IgA1 and λ light chain deposits along the capillary walls, confirming IgA-λ type proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID). Complete remission was achieved with glucocorticoid monotherapy in December 2013. After a relapse in April 2021, immunosuppressive treatment, including intravenous cyclophosphamide and subsequent mycophenolate mofetil, led to a complete remission. This case indicates that conventional immunosuppressive therapy may be effective for IgA-type PGNMID.

We examined renal biopsy specimens from two men (ages 36 and 35 years) who had been diagnosed with chronic hepatitis or cirrhosis, were positive for hepatitis B virus (HBV)-e antigen and proteinuria, and diagnosed with HBV-associated membranoproliferative glomerulonephritis. Antiviral medications for HBV were effective in one patient who was negative for HBV-e antigen, and his proteinuria improved. However, in the other patient, only limited therapeutic efficacy was observed and nephropathy progressed, necessitating dialysis. The patient developed hepatocellular carcinoma and died. The efficacy of antiviral therapy for HBV was associated with renal and survival outcomes.

Two days after eating beef steak grilled rare, a 28-year-old man developed a fever, abdominal pain, and diarrhea. The diarrhea resolved after 5 days of ciprofloxacin treatment (200 mg). Stool cultures obtained at the time of the onset were subsequently confirmed as enterotoxigenic Escherichia coli type O6. However, the renal dysfunction worsened, and the patient developed proteinuria and urinary occult blood. A kidney biopsy revealed necrotizing glomerulonephritis in the majority of glomeruli. An enzyme-linked immunosorbent assay was negative for myeloperoxidase/proteinase 3 anti-neutrophil cytoplasmic antibody (ANCA), but indirect immunofluorescence (IIF) was positive for cytoplasmic ANCA (C-ANCA). This case suggests a relationship between necrotizing glomerulonephritis and IIF-positive C-ANCAs.

Sarcoidosis is a systemic granulomatous disease that affects the lungs, lymph nodes, and eyes, which but rarely affects the parotid glands. A 71-year-old Japanese woman developed acute bilateral parotid gland swelling leading to trismus with pain. She also had bilateral swelling of the hilar lymph nodes, suggesting sarcoidosis or lymphoma. A histological examination of a parotid gland biopsy specimen revealed noncaseating granulomas, and sarcoidosis was diagnosed. Treatment with glucocorticoids decreased parotid gland swelling, and trismus and pain resolved. In patients presenting with acute and progressive parotid gland lesions, the possibility of sarcoidosis should be considered.

We herein report the case of a 78-year-old woman with non-small cell lung carcinoma (NSCLC) harboring a HER2 exon 20 insertion, who developed grade 1 trastuzumab deruxtecan (T-DXd)-induced interstitial lung disease (ILD) during third-line therapy. Oral corticosteroid administration led to radiological improvement, enabling T-DXd re-administration at a reduced dose. She received eight additional cycles (nine in total), resulting in tumor regression without ILD recurrence. Given that T-DXd is currently the only approved HER2-targeted therapy for NSCLC in our country, this case underscores the importance of early detection and appropriate management of ILD to maximize the therapeutic benefits.

Owing to the lack of appropriate treatment, TAFRO syndrome often presents with multiple organ dysfunction and fatality. The Janus kinase (JAK)/signal transducers and activators of transcription (STAT) pathway has recently been shown to play an important role in the pathogenesis of inflammation in TAFRO syndrome, and inhibitors of the JAK/STAT pathway may be effective as therapeutic agents for TAFRO syndrome. We herein report the successful treatment using combination therapy with ruxolitinib and ropeginterferon alfa-2b of a case of TAFRO-like syndrome with a long history of polycythemia vera with JAK2 V617F refractory to several treatments.

We report the case of a 59-year-old HIV-negative male diagnosed with a brain abscess following vasculitis-associated stroke. The causative pathogen remains unidentified using conventional methods including culture and rapid multiplex PCR. Craniotomy and biopsy were performed to establish a definitive diagnosis, and metagenomic next-generation sequencing (mNGS) of the abscess tissue identified Candida tropicalis as the causative pathogen. This case highlights the utility of mNGS in identifying pathogens in culture-negative CNS infections, even when conventional methods fail to detect the causative agent, particularly when evaluating abscess pus.

This study describes a family of patients with distal muscle atrophy and oculopharyngodistal myopathy (OPDM). Patients with distal muscle atrophy exhibited slowly progressive distal-predominant muscle weakness without ptosis, ophthalmoplegia, or facial weakness. Long-read sequencing confirmed the presence of intermediate and pathogenic CGG repeat expansions in LRP12 in the patients with distal muscle atrophy and OPDM, respectively. This family demonstrated a similar phenotype-genotype correlation dependent on the LRP12 repeat length, as in a previous study, but the case with intermediate repeats could not be classified into a single etiology, even after comprehensive electrophysiological assessments and muscle biopsy.

A 36-year-old man presented with rapidly progressive lower-limb weakness that reached its nadir within 16 h of the onset. The initial nerve conduction study (NCS) revealed no significant abnormalities except for a decrease in F-waves. Intravenous immunoglobulin therapy was administered, resulting in a gradual improvement in muscle strength. Follow-up NCS revealed findings consistent with axonal damage, and subsequent testing revealed positivity for immunoglobulin G-class anti-GM1 antibodies. The patient was finally diagnosed with acute motor axonal neuropathy, a variant of Guillain-Barré syndrome (GBS). This case highlights the importance of considering GBS even in patients with hyperacute progression to nadir within 24 h.

We herein report a case of progressive multifocal leukoencephalopathy (PML) in a woman in her 70s with hepatitis B-related liver cirrhosis. She presented with cerebellar onset symptoms, including ataxia and dysarthria. The diagnosis was delayed despite the initial magnetic resonance imaging (MRI) findings of an asymmetric cerebellar white matter lesion. PML was ultimately diagnosed based on a re-evaluation of a brain biopsy specimen and a retrospective JC virus (JCV) PCR analysis of the cerebrospinal fluid. Treatment with mefloquine and mirtazapine resulted in viral clearance from the cerebrospinal fluid (CSF) and clinical stabilization. This case highlights that crescent-shaped cerebellar lesions should raise suspicion of PML, even in patients without severe immunodeficiency.

A 69-year-old man initially developed cognitive impairment followed by nonfluent aphasia and left hemiparesis five months later. Initial N-isopropyl-p-[¹²³I] iodoamphetamine (¹²³I-IMP) single-photon emission computed tomography (SPECT) revealed increased regional cerebral blood flow (rCBF) in a broad area of the right cerebral cortex, including the central sulcus, as well as in the left frontal lobe. Follow-up SPECT demonstrated a decreased rCBF in the right hemisphere. The patient was diagnosed with anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis. In this case, disease progression was monitored using ¹²³I-IMP SPECT, suggesting that rCBF alterations in AMPAR encephalitis may provide insight into the underlying pathophysiology.

Multicentric reticulohistocytosis (MRH) is a rare systemic non-Langerhans cell histiocytosis characterized by destructive polyarthritis and nodular skin lesions, often associated with malignancy. We present the case of a 74-year-old Japanese man initially suspected of having dermatomyositis due to polyarthritis, typical skin manifestations, and laboratory findings. Malignancy screening revealed invasive bladder cancer, which necessitated surgery and chemotherapy. Although the skin symptoms improved, aggressive joint destruction persisted, and histopathological analyses confirmed MRH. Remarkably, ultrasonography revealed significant inflammation and tendon involvement, highlighting its diagnostic utility. This case emphasizes the challenge of distinguishing MRH from autoimmune diseases and the critical role of ultrasound in capturing destructive joint damage.

Anterior cutaneous nerve entrapment syndrome (ACNES) and thoracic radiculopathy are rare forms of abdominal pain. Therefore, we herein report an older man with chronic right lower abdominal pain from coexisting ACNES and thoracic radiculopathy, probably triggered by asymptomatic thoracic compression fractures and significant weight loss. The diagnosis was confirmed based on a positive Carnett's sign and immediate pain relief after the diagnostic lidocaine injection. Imaging revealed a T12 compression fracture with narrowing of the right T11/12 intervertebral foramen. This case highlights the need to consider ACNES and spinal pathology in older patients with unexplained abdominal pain in the context of frailty and postural changes.

Various adverse reactions to COVID-19 vaccination have been reported; however, no case reports have focused on dysphagia. We herein present two cases of severe dysphagia after COVID-19 vaccination. Case 1: A 24-year-old man with Burkitt's lymphoma developed dysphagia and multiple cranial nerve palsies. Case 2: An 80-year-old man with Parkinson's disease developed aspiration pneumonia and dysphagia. Both patients underwent a detailed dysphagia evaluation and swallowing rehabilitation. According to the Vaccine Adverse Event Reporting System, dysphagia following COVID-19 vaccination is very rare. Clinicians should be aware of the appearance or worsening of dysphagia following COVID-19 vaccination.