Objective Although marital status has been demonstrated to affect patients' decisions to undergo active treatment for pancreatic cancer (PC), the role of other household cohabitants remains uncertain. This study aimed to examine whether the presence or absence of other household cohabitants, apart from spouses, affects the treatment decisions of patients with PC.

Methods A retrospective analysis of patients diagnosed with PC at the Akita University Hospital between January 2014 and December 2020 was conducted. Information about the patients' backgrounds, including the number of persons residing in their households, was gathered. A multivariate regression analysis was conducted to ascertain the factors associated with patients' decisions to undergo aggressive treatment, such as surgery, chemotherapy, and radiotherapy.

Results A total of 214 patients with histopathologically confirmed PC were enrolled in this study. Those who lived with others were more likely to receive aggressive treatment than those who lived alone (84.9% vs. 54.5%; p<0.01). The multivariate analysis, adjusted for patients' age, performance status, and marital status indicated that the patient's decision to undergo aggressive treatment was found to be positively associated with living with other household members with an odds ratio (95% confidence interval): 4.5 (1.02-19.9), p<0.05.

Conclusion Our findings indicate that the influence of cancer treatment decisions extends beyond that of spouses to encompass other household cohabitation members. By providing comprehensive social and emotional assistance to patients residing in isolation, it is possible to facilitate active treatment and enhance the prognosis for patients with PC.

Objective This study investigated the association between smoking and myasthenia gravis (MG), a chronic autoimmune disorder that affects neuromuscular junctions.

Methods Data were collected from the Japan MG Registry 2021 survey conducted between April and October 2021. MG severity was assessed using the MG activities of daily living score and MG Foundation of America (MGFA) classification. The Brinkman Index, calculated as the number of cigarettes smoked per day multiplied by the number of years smoked, was used to evaluate the correlation between smoking exposure and MG severity. We also compared the smoking rates of individuals with and without MG stratified by age and sex.

Results Of the 1,402 patients selected from the Japanese registry, higher smoking rates were observed in patients with MG than in the general population. Compared with never-smokers, women with MG who smoked were younger and had a higher prevalence of ocular symptoms. A weak correlation was observed between MGFA and Brinkman indices among men with MG who smoked. No correlation was observed between smoking status and MG severity in women who smoked.

Conclusion This study utilized one of the largest datasets on MG and smoking; thus, it provides valuable insights into the association between smoking and MG.

Objective Corticosteroids are the primary immunosuppressants used to treat myasthenia gravis (MG). Their side effects, including Cushingoid appearance, are well documented. However, the precise relationship between corticosteroid usage and Cushingoid appearance has not been fully explored.

Methods This study investigated the frequency and severity of Cushingoid appearance and their relationships with corticosteroid use. In total, 1,321 consecutive patients with MG who were prednisolone users, enrolled in the Japanese MG registry study 2021, were examined, and the severity of Cushingoid appearance was evaluated using the Cushingoid appearance index.

Results The rates of Cushingoid appearance at survey and peak were 36.4% and 62.0%, respectively. Multivariate regression analyses revealed that the current Cushingoid appearance index was correlated with the current prednisolone dose, duration of prednisolone dose >10 mg/day, and female sex. The risk of Cushingoid appearance was classified according to prednisolone dose and duration using a decision tree. The Cushingoid appearance index significantly decreased with decreases in the prednisolone dose during the 6 years of follow-up. The Cushingoid appearance index was also significantly correlated with the MG-Quality of Life 15-revised score.

Conclusion This study elucidated the relationship between corticosteroid treatment and Cushingoid appearance in MG, suggesting that the high-dose and long-term use of corticosteroids should be avoided to improve the patient quality of life.

Objective To evaluate the efficacy and safety of amifampridine (3,4-diaminopyridine) phosphate in Japanese adults with Lambert-Eaton myasthenic syndrome (LEMS).

Methods The LMS-005 study was an uncontrolled, single-blind (patient blinded), multicenter, one-year phase 3 clinical study. The administration of amifampridine phosphate was started at 15 mg/day, and the dose was increased every 3 to 4 days to determine the optimal dose for each patient. After 7 days of treatment with the optimal dose, efficacy was assessed by evaluating quantitative myasthenia gravis (QMG), subject global impression (SGI), and Clinical Global Impression-Improvement scale (CGI-I) scores.

Patients Adult patients with LEMS were analyzed for safety [n=12, mean age±standard deviation (SD) of 61.1±14.6 years old] and efficacy (n=10, mean age±SD of 60.7±15.9 years old).

Results In the efficacy population, the mean±SD [median (interquartile range)] QMG score was 13.2±3.1 [13.5 (11.0, 15.0)] at baseline and 8.0±2.7 [8.0 (6.0, 9.0)] at the end of the treatment period, with a mean±SD [median (interquartile range)] change of -5.2±2.8 [-5.5 (-7.0, -3.0)]. All patients showed a decrease in the QMG score from baseline and experienced improvement in their LEMS symptoms. The SGI/CGI-I scores also improved. Efficacy was maintained until the end of the study. Five patients in the safety population experienced adverse drug reactions, the most common of which was dysesthesia (n=2).

Conclusion This study revealed the long-term efficacy and tolerability of amifampridine phosphate in Japanese adults with LEMS.

Objective This study evaluated the usefulness and effects of a remote vital sign monitoring system (RVSMS) using a wearable pulse oximeter device to detect acute illness in a home medical care setting.

Methods We conducted a historical control study of patients who provided home medical care services at a clinic in Japan. We selected patients between March 2023 and March 2024 who were at a high risk of deterioration (high-risk patients). Each patient was reviewed for a maximum of four months. The patients in the later period were instructed to use RVSMS as an intervention. The primary outcomes were the sensitivity and positive predictive value (PPV) of RVSMS for acute illness. The secondary outcomes were the differences in the emergency call rate (ECR), emergency visit rate (EVR), and emergency hospital admission rate (EHAR) between the two periods.

Patients A total of 38 and 36 patients were enrolled in the intervention and control groups, respectively. During the intervention period, 23 patients used the RVSMS.

Results The sensitivity and PPV for acute illness events were 68.2% (95% CI: 47.2-89.1) and 22.7% (95% CI: 3.9-41.6), respectively. The ECR decreased significantly during the intervention period. The EVR also showed a decreasing trend, although this was not statistically significant. The EHAR showed an unclear change.

Conclusion A wearable device-based vital sign monitoring system may indeed be a useful tool for the early detection of acute illness in Japanese home medical care settings; therefore, further study is warranted.

A 39-year-old woman undergoing breast cancer treatment presented with a loss of appetite and fatigue. Computed tomography (CT) revealed diffuse heterogeneity in the left hepatic lobe and thickened bile duct walls with contrast enhancement. Diffusion-weighted magnetic resonance imaging (MRI) demonstrated a high signal intensity throughout the left hepatic lobe, extending to the common bile duct. The case findings highlight the extreme rarity of diffuse hepatic metastases with common bile duct infiltration in breast cancer and underscore the critical roles of CT and MRI in identifying distinctive imaging findings that are pivotal for making a diagnosis and selecting timely management.

Intraventricular dissecting hematomas are rare and difficult to identify. We herein report the successful assessment, treatment, and follow-up of this condition using multimodality imaging. A 73-year-old man was hospitalized because of acute heart failure. The patient had a recent myocardial infarction in the left anterior descending artery. Echocardiography could not initially detect any obvious abnormalities, and anteroseptal wall thickness appeared to be preserved. However, cardiac CT and magnetic resonance imaging (MRI) revealed an intraventricular dissecting hematoma of the anteroseptal wall of the left ventricle with an impending rupture. The patient was successfully treated conservatively using medication. Cardiac CT and MRI were considered to be useful in the diagnosis and follow-up of hematoma.

Calciphylaxis is a rare, life-threatening condition in end-stage renal disease, often presenting with skin ulcers and potentially involving the internal organs. A 62-year-old man undergoing hemodialysis developed non-healing leg ulcers that were initially treated for cellulitis. The diagnosis was confirmed through a biopsy after ulceration and necrosis progressed. Despite multidisciplinary treatment, the patient died because of sepsis and intestinal perforation. Autopsy revealed extensive visceral calciphylaxis, highlighting the under-recognized systemic nature of the disease. This case emphasizes the need for heightened awareness and an early diagnosis as well as the role of autopsies in understanding calciphylaxis, particularly its potential to involve visceral organs, which may be underrecognized.

Coarctation of the aorta (CoA) is associated with other congenital cardiovascular anomalies, including ventricular septal defects (VSDs). These anomalies can cause a systolic murmur; however, CoA sometimes remains undiagnosed, even when a VSD is detected in newborns. The coexistence of CoA and VSD poses a high risk of infective endocarditis (IE), making an early diagnosis and treatment crucial. We herein report a case of IE complicated by CoA and VSD that was suspected based on auscultation and phonocardiographic findings. When a precordial systolic murmur is heard, auscultation of the patient's back is essential for CoA detection. In addition, phonocardiography is helpful for visually identifying coexisting cardiovascular diseases.

A 71-year-old woman with altered consciousness was diagnosed with primary hyperparathyroidism (PHPT) after presenting with severe hypercalcemia (serum Ca 15.3 mg/dL) and parathyroid adenoma. Unusually, her 1,25 (OH) ₂D₃ level was undetectable, despite PHPT. Hemodialysis and evocalcet failed to control the calcium levels, but denosumab effectively reduced hypercalcemia. However, both 25 (OH) D₃ and 1,25 (OH) ₂D₃ remained low. This case highlights the importance of vitamin D assessment in PHPT, as its metabolism can vary. Denosumab may be a viable treatment option for calcimimetic hypercalcemia.

A 47-year-old man with nephrotic syndrome and stage 4 chronic kidney disease was diagnosed with C1q nephropathy presenting with focal segmental glomerulosclerosis (FSGS) on a renal biopsy. A genetic analysis performed because of a family history of microscopic hematuria and lamellar glomerular basement membranes identified a heterozygous COL4A4 variant, leading to a genetic diagnosis of autosomal-dominant Alport syndrome (ADAS). Despite immunosuppressive therapy, the patient progressed to end-stage kidney disease much earlier than in previously reported cases of C1q nephropathy. This case suggests that the synergistic effects of C1q nephropathy-induced podocyte damage and abnormal glomerular basement membrane due to ADAS may promote rapid progression of FSGS.

Recent reports suggest that a transbronchial mediastinal cryobiopsy (TMC) offers a better diagnostic performance for benign diseases than endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) because it can obtain larger specimens. We herein report the utility of TMC in three patients with sarcoidosis at our institution. EBUS-TBNA failed to diagnose sarcoidosis in all three patients, whereas TMC provided a definitive diagnosis. Although no significant differences in either the safety or procedural complexity were observed between TMC and EBUS-TBNA, the cost of TMC remains a considerable challenge. Further research is required to determine the strategies regarding optimal patient selection for sarcoidosis patients who need a diagnosis of TMC.

Erdheim-Chester disease (ECD) is a rare histiocytic disorder that poses significant diagnostic challenges owing to its non-specific symptoms. We herein report the case of a 71-year-old woman with exertional dyspnea and bilateral edema who was initially suspected to have heart failure based on cardiomegaly and pericardial effusion. Pericardiocentesis with a cell block analysis revealed histiocytes that were positive for CD68 and negative for CD1a, thus increasing the likelihood of ECD. The bone biopsy results were consistent, thus leading to a diagnosis of ECD. This case highlights the diagnostic utility of a pericardial fluid cell block analysis, which offers a minimally invasive method to identify ECD in patients with significant pericardial effusion.

We used thrombopoietin receptor agonists (TPO-RAs) to treat immune thrombocytopenia (ITP) and aplastic anemia (AA). Molecular structures and methods of action differ among TPO-RAs; therefore, switching to another may be beneficial if one fails. In elderly people, planning and attending outpatient visits may complicate weekly subcutaneous romiplostim delivery. In two cases, the patient was initially unresponsive to eltrombopag, so we switched to romiplostim and then switched back due to hospital visits, which resulted in stabilizing platelet counts. We suggest better management techniques for patients, as ITP and AA require long-term management, which becomes increasingly challenging with increasing patient age.

Varicella zoster virus (VZV) infection can cause vasculopathy and stroke; however, the response of VZV-induced vasculopathy to treatment is still not well understood. We herein report the case of a 41-year-old man with a recurrent cerebral infarction following herpes zoster infection. The patient presented with a visual field disturbance and a history of myelodysplastic syndrome and chronic kidney disease. Magnetic resonance imaging revealed multiple vascular stenoses and occlusions. Despite initial antiviral and steroid therapy, his condition worsened and he required additional treatment. Vessel stenosis responded well to treatment, whereas occlusion did not. An early diagnosis and prompt treatment of VZV-induced vasculopathy are crucial for preventing irreversible vascular damage.

We describe a rare case of Miller Fisher syndrome (MFS) in a 37-year-old woman characterized by paralytic ileus and isolated internal ophthalmoplegia without any external ophthalmoplegia. The patient initially presented with severe abdominal pain and photophobia. A physical examination revealed mydriasis, impaired pupillary light reflexes, ataxia, and areflexia; however, her extraocular movements were preserved. The serum test results were positive for anti-GQ1b IgG antibodies. Treatment with intravenous immunoglobulin led to a near-complete resolution of the symptoms. This case demonstrates that MFS can manifest as significant gastrointestinal autonomic dysfunction and isolated internal ophthalmoplegia, which may not fit the classic triad presentation. Clinicians should include MFS in the differential diagnosis of patients with unexplained autonomic symptoms even in the absence of external ophthalmoplegia, and a thorough pupillary examination is crucial.

A 61-year-old woman with systemic lupus erythematosus was admitted with persistent pericardial effusion. Blood tests suggested thrombotic microangiopathy (TMA). Treatment with methylprednisolone, fresh-frozen plasma, and rituximab was initiated after tacrolimus discontinuation. A renal biopsy revealed endothelial injury and glomerular capillary thrombi, consistent with TMA. The patient developed severe anemia and bilateral lung opacities. Bronchoalveolar lavage revealed alveolar hemorrhaging and Stenotrophomonas maltophilia, indicating hemorrhagic pneumonia. Although chest radiography showed improvement with minocycline and levofloxacin treatment, she ultimately succumbed to Candida albicans sepsis. Hemorrhagic pneumonia-causing pathogens, such as S. maltophilia, should be considered when alveolar hemorrhaging occurs during immunosuppressive therapy.

A 64-year-old, previously healthy woman underwent repeated shunt removal and reinsertion for shunt dysfunction due to hydrocephalus. Mycobacterium fortuitum was detected in the culture solution at the end of the removed lumboperitoneal shunt approximately one year before the diagnosis; however, the result was considered to represent environmental contamination. The patient was hospitalized because of a high-grade fever, and M. fortuitum was detected in two blood cultures and a cerebrospinal fluid culture. We diagnosed the patient with disseminated nontuberculous mycobacterial infection due to M. fortuitum and removed the ventriculoatrial shunt. Multiple antimicrobial agents (imipenem/cilastatin, linezolid, ciprofloxacin, and trimethoprim/sulfamethoxazole) were administered for approximately two months, and the symptoms improved.