The prognosis of congenital heart disease (CHD) has improved, and most patients now reach adulthood. Owing to residual disease and comorbidities, it is recommended that adult CHD (ACHD) patients transition to adult care for lifelong monitoring and treatment. However, this transition period can be challenging for CHD patients owing to obstacles such as independence from their parents and establishing self-management. To achieve a seamless shift from pediatric to adult care and ensure continuity, it is important to educate and motivate patients appropriately, and an established transition system is needed that involves collaboration between CHD specialists and healthcare providers across medical specialties. The present review describes the epidemiology of ACHD and notable points in patient care as the background of transition. The concepts and an overview of transition systems, educational systems, and potential lapses in the care of their relevant causes are then provided with supporting evidence.

Allergic bronchopulmonary aspergillosis/mycosis (ABPA/ABPM) is characterized by increased serum levels of total and fungi-specific immunoglobulin E (IgE) and eosinophilic mucus plugs in the airways. Its classification as either an allergic or eosinophilic disease remains controversial. In the present review, we explored this topic based on three clinical studies that analyzed the clinical characteristics of ABPA/ABPM using a cluster analysis, factor analysis, and comparison between ABPM caused by Schizophyllum commune and ABPA. We also compared therapeutic responses to biologics targeting either IgE (omalizumab) or eosinophils (mepolizumab/benralizumab) to elucidate the role of these components in the pathogenesis of ABPA/ABPM. Based on these analyses, eosinophilic mucus plug formation in the airways is considered a cardinal feature of the development of ABPA/ABPM, whereas IgE responses to fungi are important factors that modulate disease manifestation.

The diagnosis of pneumonia is based on respiratory and systemic symptoms, blood test findings, chest radiographic findings, and the condition of the patient. Physicians in aging or aged societies such as Japan carefully evaluate the comprehensive situation of each pneumonia patient with adequate evaluation and treatment according to "the Japanese Respiratory Society (JRS) guidelines for the management of pneumonia in adults in 2024." These guidelines categorize pneumonia into three types: community-acquired, nursing- and healthcare-associated, and hospital-acquired. The selection of treatment settings and empirical antimicrobials for pneumonia depends on pneumonia classification, severity, and risk factors for potential drug-resistant bacteria, as outlined in the JRS guidelines. This review concisely describes the historical evolution of the diagnosis and treatment of pneumonia in elderly societies, including aspiration pneumonia, from multiple perspectives. In addition, it explores the differential diagnoses when antimicrobial treatment for pneumonia is ineffective, highlighting key aspects through chest radiography and computed tomography.

Objective To determine whether nutritional status is related to the incidence of thrombosis and mortality in patients with coronavirus disease 2019 (COVID-19).

Methods A total of 496 consecutive patients who were admitted and diagnosed with COVID-19 between April 2020 and March 2023 were retrospectively analyzed. The geriatric nutritional risk index (GNRI) on admission was calculated as follows: 14.89×serum albumin (g/dL) +41.7×body mass index/22. Patients were divided into two groups according to the median GNRI values. The endpoint of this study was a composite of in-hospital thrombotic events and mortality.

Results The median GNRI value was 99.3. Patients in the low GNRI (≤99.3) group were older (75±21 vs. 51±20 years, p<0.001) and more likely to be female (55.6% vs. 41.1%, p<0.05). In addition, patients with a low GNRI often exhibited hypertension (43.5% vs. 28.2%, p<0.001) and had a history of cardiovascular disease (34.3% vs. 14.5%, p<0.001). Under these conditions, the median D-dimer levels on admission were significantly higher in patients with a low GNRI (0.90 μg/mL; interquartile range (IQR), 0.49-1.64 μg/mL) than those with high GNRI (0.36 μg/mL; IQR, 0.26-0.51 μg/mL, p<0.001). During hospitalization, the composite endpoint was observed in 32 patients. In the logistic regression analysis, a low GNRI was significantly associated with the composite endpoint adjusted using inverse probability of treatment weighting (odds ratio, 3.24; 95% confidence interval: 1.51-6.93, p<0.05).

Conclusion Assessment of the GNRI provides useful information for predicting in-hospital thrombosis and mortality in COVID-19 patients.

Objective Despite the critical role of bone marrow aspiration and a trephine biopsy (BMAT) in the diagnosis and management of hematological diseases, research on effective teaching methods is limited. Medical students traditionally learn to perform BMAT through observation and replication, which poses a risk to patient safety. Therefore, we developed a novel BMAT simulator for undergraduate medical students using a simulation-based education program.

Methods This program, designed for fourth- and fifth-year medical students at Okayama University Medical School, included pre-study materials and one hour of simulation training. Internists practicing hematology served as the controls. Before and after the simulation training, the students completed questionnaires regarding self-confidence, self-evaluation, interest, and knowledge. The procedures were evaluated objectively using a checklist at the end of the program.

Results There were significant improvements in self-evaluation, self-confidence, interest, and knowledge acquisition after the simulation program (p≤0.001). The checklist revealed that the mean overall proficiency level of the students was 76.9%, which was significantly higher than that of internists (63.5%) (p≤0.01).

Conclusion Our simulation-based education program using the novel BMAT simulator improved medical students' BMAT knowledge and skills.

Objective The efficacy of maintenance intravenous immunoglobulin (IVIg) therapy has been established to prevent relapse in chronic inflammatory demyelinating polyneuropathy (CIDP). This prospective post-marketing surveillance study evaluated the treatment duration, efficacy, and safety of maintenance IVIg therapy in Japanese patients with CIDP.

Methods Patients were registered between June 2017 and December 2018. After induction of IVIg therapy (0.4 g/kg/day for 5 consecutive days), patients received maintenance IVIg (1.0 g/kg every 3 weeks). The observation period was 18 months. Efficacy in preventing relapse was assessed using the Inflammatory Neuropathy Cause and Treatment (INCAT) disability score. Relapse was defined as a worsening of the INCAT score by ≥1 from baseline.

Patients The efficacy population comprised 103 patients (80 with typical CIDP and 23 with CIDP variants).

Results During the observation period, 86 (83%) patients were scheduled to continue maintenance IVIg therapy during the observation period, and the relapse rate was 24% (21/86). In the remaining 17 patients who showed continuous remission, maintenance IVIg therapy was stopped (mean, 136 days after the start), and the relapse rate was 24% (4/17). One serious adverse drug reaction of cardiac failure was reported.

Conclusion In this post-marketing surveillance, most (83%) patients with CIDP were scheduled to continue maintenance IVIg for 18 months, with a relapse rate of 24%, indicating long-term efficacy. Maintenance IVIg therapy was rarely withdrawn, and the relapse rate after withdrawal was 24%. Further studies are required to determine the optimal maintenance IVIg dose and duration.

Objective Although patients with neuroimmunological disorders often need to be treated with glucocorticoids and are at risk of developing glucocorticoid-induced osteoporosis, no research has focused on the treatment of glucocorticoid-induced osteoporosis in such patients.

Methods We compared the efficacy of denosumab and bisphosphonates in glucocorticoid-induced osteoporosis in neuroimmunological diseases. In 57 patients with neuroimmunological disorders treated with corticosteroids (34 with neuromyelitis optica spectrum disorders, 16 with myasthenia gravis, and 7 with others), we retrospectively studied the long-term effects of denosumab (n=23) and bisphosphonates (n=34) on spine and total hip bone mineral density (BMD) measured by dual energy X-ray absorptiometry.

Results There were no significant differences in the age, lumbar spine BMD, or mean dose or duration of prednisolone administration at baseline between the denosumab and bisphosphonate groups. During the follow-up period of up to 6 years, the increase in the lumbar spine and total hip BMD was greater in the denosumab group than in the bisphosphonate group (p<0.01). Insufficient bone fractures were observed in 2 (9%) of the 23 patients in the denosumab group and in 2 (6%) of the 34 patients in the bisphosphonate group (not significant).

Conclusion Denosumab is more effective than bisphosphonates in increasing the BMD of patients with neuroimmunological disorders receiving glucocorticoids.

We herein report a 67-year-old Japanese woman with liver cirrhosis caused by primary biliary cholangitis. The patient was admitted to the hospital with loss of consciousness. Hepatic encephalopathy (HE) was diagnosed after diagnostic imaging and symptom assessments. Molecular biology tests were performed on oral saliva and stool samples. The test results indicated sequence similarity between urease-positive S. salivarius in both oral saliva and stool, as revealed by the signals in the overlapping peaks. This bacterium can potentially increase ammonia production in the gut, leading to HE in patients with liver cirrhosis.

Esophageal cell tumors are rare. Esophagogastroduodenoscopy performed on a 48-year-old woman revealed an elevated esophageal lesion and the presence of long-segment Barrett's esophagus. Endoscopic ultrasonography showed a 15 mm homogeneous hypoechoic tumor extending from the lamina propria mucosa to the submucosa. Pathological examination of the biopsy tissue revealed a sheet-like cluster of histiocytoid cells with an abundant eosinophilic granular cytoplasm. Immunohistochemical examination revealed S-100(+) and CD68(+), thus suggesting the diagnosis of a granular cell tumor. The tumor was resected by endoscopic submucosal dissection. Pathologically, the background mucosa was Barrett's mucosa. This is the first reported case of an esophageal granular cell tumor in long-segment Barrett's esophagus.

During follow-up of a 60-year-old patient with dilated cardiomyopathy, a Holter electrocardiogram revealed monomorphic premature ventricular complexes (PVCs) accounting for 21-30% of total beats. Oral beta-blockers led to no improvement in PVC burden. The first radiofrequency catheter ablation attempt identified the PVC arising from the left ventricle summit communicating vein (CV) but failed to eliminate the PVC's origin. The second ablation attempt with selective infusions of 100% ethanol into the summit CV resulted in immediate termination of PVCs. The post-ablation course was uneventful. Echocardiography showed an improved ejection fraction, and a repeated Holter electrocardiogram showed no recurrence of PVCs during follow-up.

A 74-year-old Japanese man was admitted to our hospital for catheter ablation of paroxysmal atrial fibrillation. Transthoracic echocardiography revealed basal interventricular septal hypertrophy without apical sparing. Cardiac magnetic resonance imaging revealed late gadolinium enhancement in the hypertrophic lesions. The Kumamoto criteria was one point, and the patient had no carpal tunnel syndrome. However, technetium-99m pyrophosphate scintigraphy revealed an accumulation in the basal region of the left ventricle. A skin biopsy revealed transthyretin (TTR) amyloid deposition. A TTR gene examination revealed no variants. This case suggests that amyloid deposition in TTR may occur in the basal area of the interventricular septum.

A six-year-old boy presented with short stature and gingival fibromatosis (GF). Dysmorphic features included slant optic fissures, a high-arched palate, thick earlobes, and an edematous face. Laboratory tests showed low levels of serum insulin-like growth factor-1 and serum free thyroxine but normal serum thyrotropin levels. Provocative tests suggested growth hormone deficiency, central hypocortisolemia, and hypothalamic hypothyroidism. At 12 years old, hypogonadotropic hypogonadism was observed. Next-generation sequencing revealed a heterozygous missense variant, KCNQ1 p. (P369L), in the proband and mother. The coexistence of multiple pituitary hormone deficiencies and GF helps diagnose KCNQ1-variant dysmorphic syndrome through genetic testing.

An underestimated side effect of rituximab is late-onset neutropenia (R-LON), which often resolves spontaneously and rarely results in a severe infection. We herein report a case of febrile neutropenia due to R-LON in a 91-year-old woman with renal failure who was treated with rituximab to induce remission of myeloperoxidase antineutrophil cytoplasmic antibodies-associated vasculitis. Fifty-four days after the last rituximab administration, the patient was hospitalized for febrile neutropenia due to R-LON, which improved with granulocyte colony-stimulating factor and antibiotics. Although R-LON may resolve spontaneously and remain unnoticed, it can cause severe infections in the elderly and patients with renal failure.

Osimertinib, a third-generation epidermal growth factor receptor tyrosine kinase inhibitor, is the standard first-line treatment for EGFR mutation-positive non-small-cell lung cancer (NSCLC) and demonstrates favorable disease control. Conversely, immune checkpoint inhibitors (ICIs) that target programmed cell death-1/programmed cell death ligands demonstrate a restrictive tumor response. We herein report a patient who achieved a durable response to pembrolizumab following early progression within two months of osimertinib administration for EGFR mutation-positive lung adenocarcinoma. Our findings suggest that treatment with ICIs for patients with EGFR mutation-positive NSCLC experiencing early progression to osimertinib as first-line treatment might represent a viable approach.

A 75-year-old woman presented with significant muscle weakness after statin use. A muscle biopsy revealed necrotizing myopathy, and the patient tested positive for serum anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibodies, leading to a diagnosis of anti-HMGCR immune-mediated necrotizing myopathy (IMNM). Computed tomography revealed intraperitoneal lymphadenopathy, which was diagnosed as a diffuse large B-cell lymphoma. Immunostaining confirmed HMGCR expression in the lymphoma cells. The patient received chemotherapy and achieved complete remission of the lymphoma, along with nearly complete recovery from IMNM. Although the etiologies of IMNM and lymphoma remain unclear, HMGCR expression in lymphoma cells is likely to be associated with the development of IMNM.

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare disease associated with the presence of anti-glycine receptor (GlyR) antibodies. We herein report an autopsy case of an 80-year-old man diagnosed with anti-GlyR antibody-positive PERM who presented with symptoms of oculomotor dysfunction and autonomic failure. Despite intensive immunotherapy, the neurological symptoms showed almost no improvement, and the patient succumbed to aspiration pneumonia and bacterial translocation. Postmortem pathology revealed mild inflammatory changes and neuronal loss that were disproportionate to a severe clinical presentation. These results suggest that the clinical symptoms of PERM may result from antibody-mediated GlyR internalization, leading to neuronal disinhibition, rather than a neuroinflammatory signature.

Libman-Sacks endocarditis is an important cause of embolic stroke in systemic lupus erythematosus, although the detailed pathogenesis of stroke remains unclear. We herein report two cases of stroke with Libman-Sacks endocarditis in which the emboli were retrieved by mechanical thrombectomy. The embolus consisted of eosinophilic homogeneous acellular structures, whereas fibrin-platelet thrombi were hardly observed in the embolus. Immunohistochemistry showed immunoglobulin deposits in the embolus, suggesting that immunological mechanisms were involved in the growth of the embolus. A pathological analysis of the embolus retrieved by mechanical thrombectomy provided useful information on the etiology, leading to optimal treatment.

Anti-melanoma differentiation-associated gene 5 (MDA-5) antibody-positive dermatomyositis is associated with rapidly progressive interstitial lung disease (RP-ILD). We encountered a man in his 40s who presented with a history of a fever and dry cough. Based on laboratory tests and computed tomography scans of his chest, he was diagnosed with anti-MDA-5 antibody-positive dermatomyositis with RP-ILD refractory to antimicrobial agents. Although the patient was treated with glucocorticoids, calcineurin inhibitors, intravenous cyclophosphamide, and plasma exchange, ventilatory management was still required. The patient survived additional therapy with tofacitinib; however, he developed a catheter-related pulmonary embolism as a complication.