Objective The ratio of hemoglobin to red blood cell distribution width (Hb/RDW) is a simple and readily available tool associated with adverse outcomes in chronic heart failure (HF). However, the association between the Hb/RDW ratio and mortality in patients with acute decompensated HF (ADHF) is unclear. The goal of this study was to investigate the relationship between the Hb/RDW ratio and mortality in patients after ADHF.

Methods This single-center study included clinical and laboratory data collected at baseline, with patients prospectively followed-up for a median period of 3.1 years. The patients were divided into two groups based on their median Hb/RDW ratio.

Patients We evaluated 250 consecutive patients hospitalized for ADHF at Shinshu University Hospital between July 2014 and March 2019.

Results In our study cohort [median age, 76 (66-83) years; 62.8% male], all-cause death was observed in 91 patients (incidence rate: 12.7 per 100 patient-years). A Kaplan-Meier analysis revealed that patients in the lower Hb/RDW ratio group (<0.24, n=131) had worse outcomes compared to those in the higher group (≥0.24, n=119) (cumulative incidence 44.1% vs. 19.5%, respectively; log-rank, p<0.001). After adjusting for demographics, HF severity, and laboratory biomarkers, a lower Hb/RDW ratio was significantly associated with a higher risk of mortality (hazard ratio, 1.89; 95% confidence interval, 1.04-3.45; p=0.038).

Conclusion A lower Hb/RDW ratio is associated with an increased risk of mortality in patients after ADHF, thus indicating its potential utility in identifying patients at an elevated risk for future cardiovascular events.

Objective This study aimed to examine the risk of diabetes mellitus induced by nilotinib, a second-generation tyrosine kinase inhibitor.

Methods This retrospective study included 25 patients with chronic myeloid leukemia (CML) treated with nilotinib at our hospital. Four patients had diabetes mellitus at the start of nilotinib administration (prior DM group), and five patients were newly diagnosed with diabetes mellitus after the start of nilotinib administration (new DM group). Sixteen patients who were not diagnosed with diabetes mellitus were classified into the non-DM group. Changes in the blood glucose and HbA1c levels were evaluated in each group at the time of nilotinib administration and two years later.

Results Molecular genetic remission of CML was achieved in 81.8% of patients with diabetes and 72.2% of patients without non-DM group. There were no cases in this study in which nilotinib was changed or discontinued owing to hyperglycemia. There was no difference in the blood glucose levels at the start of nilotinib treatment among the groups. Two years after starting nilotinib, the blood glucose levels in the new DM group [232 (186-296) mg/dL] and prior DM group [168 (123-269) mg/dL] were significantly higher than those in the non-DM group [100 (91-115) mg/dL]. ΔHbA1c levels in the new DM group [1.3 (0.9-2.2) %] and prior DM group [1.6 (0.7-1.7) %] were significantly higher than those in the non-DM group [-0.2 (-0.3-0.1) %].

Conclusion Nilotinib caused diabetes in 23.8% of the participants, but there were no hyperglycemia-related severe adverse events. Therefore, nilotinib may be safely continued with regular monitoring for the development of diabetes after nilotinib administration.

Objective In randomized clinical trials and real-world studies, calcitonin gene-related peptide (CGRP) monoclonal antibodies (mAbs), including erenumab, have demonstrated efficacy for migraine prevention. However, there have been no real-world studies focusing on erenumab in East Asia that investigated its efficacy on migraine-associated symptoms and patient-reported satisfaction levels.

Methods This single-center, observational, retrospective, real-world study examined patients who received at least three doses of erenumab at Keio University Hospital, Tokyo, Japan, between December 2021 and March 2023 as their first CGRP mAb treatment in a real-world setting. The patients were administered 70 mg of erenumab monthly. We assessed changes in monthly migraine days (MMDs), responder rates, migraine-associated symptoms including photophobia, phonophobia, nausea/vomiting, and patient-reported satisfaction levels. In addition, injection site reactions and other adverse events were recorded to investigate safety.

Results Nineteen patients were considered eligible for the analysis. At 3 months, erenumab decreased MMDs by 6.6 (95% confidence interval, 2.3-10.8; p<0.01). The 50% responder rate was 42%. A total of 83% (n=15), 56% (n=10), and 71% (n=10) of patients reported either improvement in or disappearance of photophobia, phonophobia, and nausea/vomiting, respectively, and 44% (n=8) and 28% (n=5) answered "very satisfied" and "somewhat satisfied", respectively, with erenumab treatment, leaving only 28% (n=5) as "unsatisfied". Injection site reactions (n=6, 32%) and constipation (n=4, 21%) were frequent adverse events.

Conclusion In a real-world setting in Japan, erenumab proved to be effective in not only reducing migraine and headache frequency but also improving migraine-associated symptoms and satisfying the majority of patients.

Undifferentiated carcinoma of the liver is a rare and difficult-to-detect form of primary liver cancer. We herein report the first case of undifferentiated carcinoma of the liver in a 70-year-old Japanese woman with primary biliary cholangitis. The patient was diagnosed with cStage IVA liver cancer (85 mm in diameter) and treated with hepatic arterial infusion chemotherapy, 30 Gy radiotherapy, and 11 courses of on-demand transarterial chemoembolization. Although the hepatic tumor had markedly shrunk (from 85 to 20 mm), the patient ultimately died 16 months after the diagnosis due to rapid growth of lymph node metastases.

A 73-year-old man presented with left hypochondral pain. Dynamic computed tomography (CT) revealed abnormal vessels surrounding the pancreas, leading to a suspected diagnosis of pancreatic arteriovenous malformation (PAVM). At the time of the initial examination, dynamic CT revealed mild acute pancreatitis, and PAVM was diagnosed based on the findings of dynamic CT. Although repeated abdominal pain was observed after the improvement of pancreatitis, distal pancreatectomy was performed. At >1 year after surgery, no recurrence of PAVM was observed. Surgical resection should be considered in patients with symptomatic PAVM.

Hydroxycarbamide, an antimetabolic agent used to treat myeloproliferative disorders, causes side effects, including myelosuppression, skin ulcers, and oral mucositis. Gastrointestinal ulcers are uncommon, and esophageal ulcers have not been previously reported. We present the case of a 74-year-old woman who developed esophageal and ileal ulcers after hydroxycarbamide treatment. Our case and previous reports suggest that hydroxycarbamide can cause ulcers throughout the gastrointestinal tract, which can improve rapidly after discontinuing medication. When new signs and symptoms occur, drug-induced etiologies should be considered as a potential cause. Timely diagnostic treatment with discontinuation of medication is crucial in such cases.

We herein report a case of Takotsubo syndrome in an 80-year-old woman with end-stage renal disease who hesitated to initiate hemodialysis for pulmonary edema. On hospital day 2, the patient experienced cardiac arrest. Coronary angiography after spontaneous return of circulation showed no substantial stenosis. Takotsubo syndrome was diagnosed based on echocardiography findings. On hospital day 3, she developed ventricular fibrillation but was spontaneously resuscitated. Patients with chronic kidney disease and those who postpone dialysis initiation may be at an increased risk of developing Takotsubo syndrome. Early and careful monitoring and adequate shared decision-making are essential for seamless initiation of dialysis.

Danon disease (DD) is a rare lysosomal storage disorder resulting from pathogenic variants of the lysosome-associated membrane protein type 2 (LAMP-2) gene. The disease is characterized by severe cardiomyopathy, which rapidly progresses to end-stage heart failure. This case, with DD caused by a missense variant, exhibited slow progressive cardiomyopathy and survived for an extended period despite being a male. A pathological analysis revealed that only a minority of the samples exhibited autophagic vacuoles with unique sarcolemmal features, which are typical of DD. Importantly, LAMP-2 expression was absent and the myocardial tissue contained a substantial amount of p62-positive aggregates.

We herein report a case of systemic capillary leak syndrome (SCLS) attributed to coronavirus disease (COVID-19) that emerged in 2019. A 56-year-old woman presented with a COVID-19 infection 7 days prior to the visit with upper respiratory symptoms, fatigue, and decreased appetite. Secondary SCLS due to COVID-19 was diagnosed, veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was initiated as mechanical support, and intravenous immunoglobulin was administered, marking the transition to the recovery phase with the initiation of fluid resuscitation. This case is noteworthy for successfully employing VA-ECMO in treating secondary SCLS due to COVID-19, mimicking the course of fulminant myocarditis.

Many cases of primary aldosteronism (PA) in patients who developed hypokalemia-induced rhabdomyolysis and underwent adrenalectomy for aldosterone-producing adenoma (APA) have been reported; however, the immunohistopathological and molecular features remain unknown. We herein report the case of a 28-year-old woman with PA who presented with hypokalemia-induced rhabdomyolysis and underwent adrenalectomy for unilateral APA. An immunohistochemical analysis revealed that most adenoma cells were positive for steroidogenic enzymes, including CYP11B2. A genetic analysis revealed a somatic mutation in the KCNJ5. These findings suggest a strong aldosterone production capacity in our patient's adenoma, which was presumably related to her severe hyperaldosteronism and the resultant hypokalemia-induced rhabdomyolysis.

A percutaneous renal biopsy (PRB) is a standard procedure for diagnosing renal disease, but can cause bleeding complications. Bleeding after a PRB can be classified as early- or late-onset, depending on the timing of the onset of the bleeding symptoms (<24 h or ≥24 h). We herein report two patients who experienced bleeding complications: one experienced early-onset bleeding from the 12th subcostal artery, and the other experienced late-onset bleeding from an arteriovenous fistula between a branch of the renal artery and renal vein. In both cases, the origin of the bleeding vessel was misjudged during the first examination. We discuss the diagnostic pitfalls of the origin of bleeding after a PRB and propose measures to avoid falling such pitfalls.

A 56-year-old man presented to our hospital with dyspnea on exertion for two months. Bilateral pleural effusions were found, and a close examination revealed a chylothorax, including adenocarcinoma. The primary tumor could not be identified by systemic examination. Therefore, the patient was diagnosed with cancer of unknown primary (CUP) presenting with chylothorax. Chemotherapy was administered for CUP, and thoracentesis, pleurodesis, ascites puncture, and nutritional therapy were performed for chylothorax and chylous ascites. Although drainage frequency and tumor marker levels (CA19-9, DUPAN-2, and Span-1) temporarily decreased, disease control deteriorated, and the patient died 12 months after the initial diagnosis.

Pulmonary sclerosing pneumocytoma (PSP) is a rare, benign tumor. Given the challenges of a bronchoscopic diagnosis, surgery is performed during the early stages of the disease. Therefore, little is known about the growth pattern of PSP. This case of PSP was not diagnosed despite bronchoscopy, resulting in lung resection eight years after the anomaly was first identified on computed tomography (CT). This report compares the long-term follow-up of CT and pathological findings and discusses the difficulty in making a diagnosis using a bronchoscopic forceps biopsy to aid in future PSP diagnoses and treatment planning.

Combined pulmonary fibrosis and emphysema (CPFE) is characterized by emphysematous lesions in the upper lung field and pulmonary fibrosis in the lower lung field and is often associated with pulmonary hypertension and severe exercise-induced hypoxemia (EIH). We herein report a 62-year-old man with CPFE who presented with severe EIH despite relatively preserved lung volumes. Cardiopulmonary exercise testing suggested exercise-induced right-to-left shunt (EIS) through a patent foramen ovale (PFO). EIS was attributed to exercise-induced pulmonary hypertension. In this case report, we highlight the possibility of EIS using PFO for CPFE. We also discuss potential treatments including pharmaceutical interventions and PFO closures.

Hangeshashinto is a traditional Japanese herbal medicine that is widely recognized for its efficacy in relieving mucositis induced by chemotherapy and radiotherapy. We herein present the cases of two patients with head and neck cancer who were clinically diagnosed with severe drug-induced interstitial lung disease (DILD) following Hangeshashinto administration for radiation-induced mucositis. Although Hangeshashinto has beneficial properties, it is also associated with a relatively low incidence of DILD, including some reports of death. To ensure patient safety, greater attention should be paid when prescribing Hangeshashinto, especially for elderly patients with factors predisposing them to develop severe DILD.

A 44-year-old woman underwent a follow-up examination for Crohn's disease 9 years ago. Chest computed tomography (CT) showed an infiltration shadow with a cavity in the right upper lobe. After a CT-guided lung biopsy, epitheloid granuloma was noted, and an acid-fast bacilli examination was smear-positive, but a culture examination was negative. Because the abnormal chest shadow with cavity gradually increased and right shoulder pain appeared, we performed bronchoscopy again six months later. Mycobacterium heckeshornense was isolated from the bronchoalveolar lavage fluid specimen, so we diagnosed her with pulmonary M. heckeshornense disease. Isoniazid, rifampicin, and ethambutol were administered, and the abnormal chest shadow improved.

We herein report a case of neurosyphilis that presented with isolated bilateral abducens nerve palsy. A 39-year-old man was referred to our department with diplopia. He had a history of homosexual relationships and showed only bilateral abducens nerve palsy upon a neurological examination. Positive syphilis tests in the serum and cerebrospinal fluid and a contrasting effect on the abducens nerve on magnetic resonance imaging (MRI) confirmed the diagnosis of active neurosyphilis. When a patient manifests isolated abducens nerve palsy, neurosyphilis can be a differential diagnosis, although rare, and contrast-enhanced MRI may help diagnose the disease.

When an ischemic stroke occurs due to bone or cartilage dynamically affecting vessels supplying the brain, it is called bony stroke. We herein report a patient with recurrent cryptogenic stroke that was thought to be a bony stroke. Dynamic three-dimensional computed tomography angiography revealed mechanical compression of the vertebral artery by the hyoid bone and thyroid cartilage. The patient had a recurrent stroke during antiplatelet therapy. Surgical removal of bone tissue prevents stroke recurrence.

We herein report two patients with anti-muscle-specific kinase (MuSK) antibody-positive myasthenia gravis who experienced rapid deterioration of weakness, particularly respiratory muscle weakness, necessitating non-invasive positive pressure ventilation (NIPPV) and were treated with efgartigimod. After treatment initiation, a rapid reduction in IgG levels and recovery from clinical symptoms were observed. NIPPV was no longer required two to three weeks after the first infusion of efgartigimod. These findings suggest that the reduction of IgG levels using efgartigimod is a good treatment option in patients with myasthenia gravis positive for anti-MuSK antibodies, even during the acute phase of the disease.

Managing tuberculous meningitis (TBM) is challenging because of its poor prognosis and the difficulty in making an early diagnosis due to the low sensitivity of cerebrospinal fluid (CSF) polymerase chain reaction (PCR) evaluations. A 75-year-old woman presented with fatigue and multiple enlarged lymph nodes and was initially suspected of having metastatic cancer of unknown primary origin. Differential diagnoses included carcinomatous meningitis, neurosarcoidosis, and TBM, as suggested by the presence of multiple enhancing cerebral nodules. Despite 11 negative PCR evaluations, including nested PCR of CSF and biopsied lymph nodes within the first 3 days of empirical anti-tubercular treatment, TBM was eventually confirmed by CSF cultures 32 days later. This case highlights the need for repeated sampling.

Progressive supranuclear palsy (PSP) is characterized by progressive postural instability, falls, and supranuclear vertical gaze abnormalities. In this report, we present the case of a 71-year-old woman with dopa-responsive rest tremor followed by tachyphemia and postural instability. She initially presented with dopa-responsive slowness and tremor in the right hand. Two years later, she developed speech difficulties (tachyphemia) and a propensity for falls. Based on the diagnostic criteria for PSP, the patient was diagnosed with probable PSP-RS. The clinical manifestations observed in our patient are unique and are considered important for illustrating a broad spectrum of PSP syndrome.

Macrophage activation syndrome (MAS) involves an excessive amount of acute inflammatory responses to inflammatory cytokines, particularly interleukin-6 (IL-6). IL-6 is also strongly associated with the pathophysiology of certain neuroimmunological diseases. However, there have so far been few reports of MAS being accompanied by neuroimmunological diseases. We herein report two cases of MAS comorbid with myasthenia gravis or neuromyelitis optica spectrum disorders, IL-6 related neuroimmunological diseases. Standard immunosuppressive therapies could not stabilize the symptoms in our cases until antibodies against the IL-6 receptor were administered. This finding suggests that it is important to consider the underlying pathophysiology of MAS in relation to these neuroimmunological diseases when treating affected patients.

Acute respiratory failure (ARF) due to lateral medullary infarction (LMI) can be a fatal condition, although its details remain unclarified. To clarify clinical aspects of ARF due to LMI, we reviewed eight applicable cases treated in a tertiary hospital. Of them, we focused on the detailed clinical course of one case. We clarified that even patients with a serious clinical course may have no apparent magnetic resonance imaging abnormalities in respiratory control centers at the initial examination. We should be mindful that respiratory monitoring is necessary even without infarction of regions responsible for respiratory control at the initial presentation.

A 77-year-old man presented with right inguinal lymphadenopathy and swollen parotid and submandibular glands bilaterally. Histopathology revealed germinal center B-cell type diffuse large B-cell lymphoma (DLBCL) in the inguinal lymph node. Lymphocyte and plasma cell infiltration in the submandibular gland with elevated serum IgG4 levels (13 g/L) prompted a diagnosis of IgG4-related disease (IgG4-RD). Systemic chemotherapy for DLBCL led to shrinkage of the lymph nodes and disappearance of the submandibular gland swelling, as confirmed by fluorodeoxyglucose-positron emission tomography/computed tomography. Although concomitant IgG4-RD and lymphoma have been reported, their simultaneous diagnosis is rare; therefore, a biopsy of all involved organs is crucial in cases with unusual organ involvement.

Giant cell arteritis (GCA) can result in visual loss and other sequelae. An 81-year-old man presented with a one-week history of fever. He had bilateral temporal headache, jaw claudication, tenderness of the temporal arteries and a recent skin rash. A temporal artery biopsy showed typical GCA, but the symptoms were self-limiting. We continued close observation, without administering prednisolone treatment. Five months later, the symptoms did not recur, and prednisolone again was not administered. Our patient presented with an atypical course of GCA that created a clinical dilemma. The final diagnosis was self-limiting GCA.

Nocardia exalbida, an uncommon Nocardia, was first identified in 2006. We herein report a 70-year-old man with pulmonary nocardiosis caused by N. exalbida after living-donor liver transplantation. We also review 11 previously reported cases of N. exalbida infections. To our knowledge, there are no case reports available on nocardiosis consequent to N. exalbida infection following transplantation, thus highlighting the importance of identifying bacterial species for the successful management of infection.

Echinococcosis, caused by Echinococcus spp., often affects the lungs and liver, and spinal involvement is rare. Echinococcus multilocularis is prevalent in Japan, particularly in Hokkaido. We herein report a rare case of spinal echinococcosis in a 31-year-old woman who was diagnosed in Tokyo. Spinal echinococcosis is uncommon and often leads to misdiagnoses. The patient likely contracted the disease via contaminated fresh produce transported from an endemic region. This study emphasizes the diagnostic challenges of spinal echinococcosis in non-endemic regions and highlights the public health concerns related to the spread of infections in non-endemic areas.