Objective Crohn's disease (CD) is a chronic inflammatory bowel disease that is associated with malnutrition. Sarcopenia is a malnutrition condition characterized by skeletal muscle loss that impairs the physical function. We investigated the clinical characteristics of patients with CD with sarcopenia and sarcopenic obesity (sarcopenic-o).

Methods The body composition of patients with CD was evaluated using a bioelectrical impedance analysis. The clinical characteristics of patients with sarcopenia and sarcopenic-o were analyzed, and a predictive model for sarcopenia was developed.

Patients Patients with CD recruited from 2019 to 2021 were included.

Results Among the 104 patients, 35 (33.7%) and 10 (9.6%) had sarcopenia and sarcopenic-o, respectively. In the sarcopenia group, the skeletal muscle index (SMI) and body mass index (BMI) were lower than those in the control group (SMI, 6.3 kg/m² vs. 7.7 kg/m², p<0.01; BMI, 18.8 kg/m² vs. 22.6 kg/m², p<0.01), whereas the Crohn's disease activity index (CDAI) was higher than in the control group (114.2 vs. 42.0, p<0.01). The predictive models of sarcopenia using the BMI and CDAI revealed high performance with areas under the receiver operating characteristic curve (AUC) of 0.87 and 0.72, respectively, and high specificity (0.94) and sensitivity (0.71), respectively. Sarcopenic-o patients could not be screened using the BMI (25 kg/m²), and the SMI and body fat percentage were negatively correlated in patients with sarcopenia (p<0.01).

Conclusion Sarcopenia and sarcopenic-o are relatively common conditions among patients with CD. Sarcopenia can be predicted using the clinical parameters of BMI and CDAI. Sarcopenic-o can be a severe form of sarcopenia.

Objective Screening and surveillance methodologies for Lynch syndrome (LS) in Japan. This study assessed the changes in LS knowledge and practice trends.

Methods In 2020 and 2022, 2 questionnaire surveys were administered to 3,574 councilors of the Japanese Society of Gastroenterology to assess changes in LS-related knowledge and practices.

Materials Each questionnaire item was analyzed by comparing responses between the first and second surveys to determine the proportion of doctors selecting each option relative to the total number of respondents. The responses from doctors who completed both surveys were analyzed to assess the temporal changes in their responses.

Results The second survey showed a significant increase in the awareness of universal tumor screening (UTS), proportion of doctors selecting UTS for primary LS screening, use of BRAF V600E testing for chemotherapy selection, and number of newly diagnosed LS patients per doctor over the last three years. In addition, the number of patients currently under surveillance by doctors has also increased. Doctors who intensified primary screening for LS between surveys reported a greater increase in newly diagnosed cases. However, the rise in UTS suggests a potential bias from doctors with heightened interest in LS, which may have influenced the findings.

Conclusion The number of newly diagnosed and currently monitored patients with LS in Japan has been increasing, likely due to expanded screening practices. However, the potential bias introduced by the increased adoption of UTS should be considered when interpreting these results.

Objective Angiotensin receptor-neprilysin inhibitors (ARNIs) have been widely used as a treatment for heart failure. However, they have only been approved for the treatment of hypertension in a limited number of countries. We investigated the effect of ARNIs on arterial stiffness using the cardio-ankle vascular index (CAVI) in patients with hypertension to reveal the mechanism underlying cardiovascular interaction.

Methods We investigated the utility of ARNIs in patients in whom arterial stiffness was assessed using the CAVI before and after ARNI administration.

Patients In this retrospective observational study, we enrolled 50 patients with hypertension treated with ARNIs between January 2021 and 2023. Forty-eight of the 50 patients (96%) were switched from angiotensin II receptor blockers due to inadequate antihypertensive control.

Results The systolic blood pressure (BP), diastolic BP, and CAVI were significantly decreased by ARNI administration [systolic BP: 145 (135, 162) to 131 (123, 143) mmHg (p=0.000); diastolic BP: 92 (78, 100) to 82 (74, 89) mmHg (p=0.000); and CAVI: 9.9 (9.1, 10.5) to 9.5 (8.8, 10.2) (p=0.005), respectively].

Conclusion Hypertension treatment with ARNIs may improve not only the blood pressure but also the CAVI, reducing the afterload on the heart.

Objective Cortisol-producing adenoma (CPA) is a major subtype of functional adrenal tumors. CPAs are generally treated with adrenalectomy; however, it is difficult to predict the ideal duration of glucocorticoid replacement after resection. This study explored pre-operative factors predictive of glucocorticoid replacement therapy duration after CPA resection.

Mehtods This multicenter retrospective observational study was conducted as part of the Advancing Care and Pathogenesis of Intractable Adrenal Diseases in Japan (ACPA-J) from January 2006 to December 2015.

Patients This study was conducted at 10 referral centers and included 124 patients who received hydrocortisone replacement therapy after adrenalectomy for CPA.

Results The median duration of replacement therapy was 12 (interquartile range, 5-24) months. In the single regression analysis, the sex (p=0.04), morning adrenocorticotropic hormone (ACTH) level (p=0.02), morning serum cortisol level (p=0.003), midnight serum cortisol level (p<0.001), serum cortisol level after a 1-mg dexamethasone suppression test (p<0.001), presence of lumbar compression fracture (p=0.015), and Cushingoid appearance (p<0.001) were all significantly associated with the replacement therapy duration. In multiple regression analyses, the midnight serum cortisol level and presence of lumbar compression fracture were significantly correlated with the replacement therapy duration after adjusting for other parameters.

Conclusion Our results suggest that high midnight serum cortisol levels, which cause persistent suppression of the hypothalamic-pituitary-adrenal axis (HPA axis), contribute to a delay in HPA axis recovery. Lumbar compression fracture is an important symptom that reflects the severity and persistence of cortisol secretion.

Objective Our previous study indicated that the efficacy of metformin in lowering glycated hemoglobin (HbA1c) levels may be influenced by the pretreatment frequency of defecation (FD) in patients with type 2 diabetes mellitus (T2DM). This study aimed to further examine how FD and the metformin dose may affect HbA1c changes (ΔHbA1c) in T2DM patients.

Methods A retrospective analysis was conducted on inpatients who received antidiabetic treatment without altering dosages for six months post-discharge, except for minor insulin adjustments. For new patients, FD was assessed before (pretreatment FD) and after the initiation of antidiabetic therapy (posttreatment FD). For patients already on treatment, FD was evaluated during hospitalization (posttreatment FD). Patients were categorized based on their metformin use, and the relationship between FD and ΔHbA1c was assessed 1.5-6 months post-discharge. The impact of the metformin dose and posttreatment FD on the ΔHbA1c level was analyzed, along with other factors affecting posttreatment FD.

Results The analysis included 89 patients (41 on metformin, 21 newly treated; 48 not on metformin, 17 newly treated). Both pre- and posttreatment FD were linked to ΔHbA1c levels in the metformin group. The metformin dose correlated with posttreatment FD but not with pretreatment FD. A significant relationship was observed between ΔHbA1c and the metformin dose. A multiple regression analysis identified posttreatment FD and metformin dose as significant independent factors influencing ΔHbA1c levels. Additionally, diabetic peripheral neuropathy and diabetes duration were found to diminish the effectiveness of metformin, likely due to decreased posttreatment FD.

Conclusion FD may independently contribute to the dose-dependent HbA1c-lowering effects of metformin.

Objective To calculate the prevalence of anemia based on sex and age using large-scale occupational health examination data, confirm the Hb) distribution in the elderly, and examine the standard for screening for anemia in Japan.

Methods Using existing large-scale occupational health examination data of patients who underwent health examinations between April 2015 and March 2022, we calculated the prevalence of anemia based on sex and age.

Patients We included 416,366 patients aged 20-89 years who underwent health checkups at health checkup facilities in Japan.

Results Of the patients, 53.5% were men. The proportion of previously anemic individuals was 3.2% in men and 19.7% in women, with Hb levels (mean ± SD) of 15.1±1.1 g/dL in men and 13.1±1.2 g/dL in women. The prevalence of anemia showed a monotonic increase with age in men and a bimodal distribution, peaking in women in their 40s and 70s. Among women in their 20s-40s, 17.9% had anemia, and 60% were newly diagnosed. Furthermore, the mean -2SD of Hb in the elderly men (>65 years) was 12.0 g/dL, larger than the current standard (11.0 g/dL).

Conclusion It is imperative to consider the possibility of anemia in young women during health examinations and other screening opportunities, regardless of their history of anemia. In addition, elderly men with Hb levels <12 g/dL should be cautioned about the development of anemic symptoms and the progression of Hb decline.

Objective Dysphagia is a common and disabling symptom in patients with myasthenia gravis (MG). Moreover, it is caused by muscle weakness or fatigability in the pharynx, swallowing, and respiration discoordination. The current study aimed to establish a novel method for evaluating swallowing difficulty in patients with MG.

Methods The ventilation patterns and submental surface electromyography (sEMG) activity of the swallowing reflex were simultaneously recorded for 10 min during the continuous infusion of distilled water (150 mL/h) into the pharynx in 10 patients with MG and 22 healthy controls. Moreover, we assessed excessive expiratory flows, clusters of excessive expiratory flows, high-inspiratory flows, and prolonged EMG patterns.

Results Patients with MG who presented with dysphagia had abnormal excessive expiratory flows, clusters of excessive expiratory flows, high inspiratory flows, and prolonged EMG patterns compared to healthy controls (all p<0.05). Among these parameters, the incidence of an excessive expiratory flow, cluster of excessive expiratory flows, and prolonged EMG pattern significantly improved after treatment (p<0.05).

Conclusion Based on this study, the respiratory patterns and submental sEMG are likely to reflect the severity of pharyngeal muscle weakness/fatigability and thus can be used as a quantitative parameter for dysphagia in patients with MG.

Objective This study investigated the frequency and risk factors of acute liver injury (ALI) and the association between ALI and treatment in hospitalized patients with coronavirus disease 2019 (COVID-19).

Methods This was a single-center retrospective study of 631 hospitalized patients ≥18 years old who were diagnosed with COVID-19 and received treatment between July 1, 2020, and January 31, 2024. Demographic and clinical data were extracted from the electronic medical records. ALI was defined according to the consensus guidelines of the Asia Pacific Association of the Study of Liver. Patients were divided into two groups according to the presence of ALI to assess the risk factors for the occurrence of ALI.

Results Seventy-six patients (12.0%) developed ALI. Seven patients discontinued remdesivir owing to hepatic impairment, and only 1 patient (0.2%) had an increase in alanine aminotransferase (ALT) ≥10 times the upper limit of normal. ALI was associated with men [odds ratio (OR)=3.052, 95% confidence interval (CI)=1.456-6.398], a higher World Health Organization (WHO) ordinal scale score at admission (OR=1.408, 95% CI=1.036-1.912), higher ALT level at admission (OR=1.017, 95% CI=1.009-1.024), tocilizumab administration (OR=2.788, 95% CI=1.372-5.666), the absence of diabetes (OR=0.456, 95% CI=0.226-0.922) and the absence of dyslipidemia (OR=0.244, 95% CI=0.083-0.723). In the comparison of the propensity score-matched groups, neither remdesivir nor tocilizumab administration was associated with ALI.

Conclusion Men, severe COVID-19, and elevated ALT levels at admission were significantly associated with an increased risk of ALI in patients treated for COVID-19. ALI may have been associated with tocilizumab administration but not with remdesivir administration.

Emphysematous cholecystitis has a poor prognosis and necessitates prompt surgery or drainage. Emphysematous cholangitis (EC) is believed to have a similar clinical course, but reports are limited. We herein report the case of a 79-year-old woman who presented with epigastric pain and was diagnosed with EC. Rapid deterioration occurred after admission, requiring intensive and prolonged intravenous antibiotic therapy, despite immediate drainage and antibiotic therapy. We reviewed the background, causative organisms, treatment, and outcomes of EC through a literature search. Our findings provide insights into its similarities to emphysematous cholecystitis, emphasizing the need for expeditious drainage and anaerobic bacterial coverage during antibiotic therapy.

A 50-year-old man was diagnosed with type 1 autoimmune pancreatitis (AIP) following endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) and a histopathological examination. After six months of untreated follow-up, the serum IgG4 level decreased, and the diffuse pancreatic enlargement improved; however, a pancreatic head mass became apparent. EUS-FNA of this mass revealed pancreatic ductal adenocarcinoma (PDAC) with IgG4-positive plasma cells. In addition, the resected specimen revealed PDAC, without any features of AIP. After pancreatoduodenectomy, AIP did not recur. The development of AIP in this case could be related to paraneoplastic syndrome.

Among causes of liver failure, liver failure due to lymphocytic infiltration is rare. Unlike typical liver failure, some cases present with severe lactic acidosis and a poor prognosis. We herein report a case of diffuse large B-cell lymphoma with severe lactic acidosis and liver failure. We further discuss the characteristics of similar cases in the literature, suggesting that intrahepatic infiltration by hematological malignant cells should be considered as a differential diagnosis in the presence of severe lactic acidosis and liver failure. This study underscores the importance of early recognition and serves as a reference for future cases.

An 85-year-old man with a history of 2 open-heart surgeries [for aortic regurgitation and infective endocarditis (IE)] and pacemaker implantation for bradycardic atrial fibrillation presented with a fever. Transesophageal echocardiography revealed a pacemaker lead vegetation. Computed tomography showed a retrosternal abscess. He was diagnosed with acute heart failure and IE. Given the high surgical risk due to his age, acute heart failure, and surgical history, we decided against cardiac surgery. After lead extraction, a leadless pacemaker was inserted, and antimicrobial therapy was administered. The patient was discharged on day 48, highlighting a strategy for managing complex cardiac device-related IE.

A 60-year-old man who developed rhabdomyolysis and severe acute kidney injury (AKI) after the fourth coronavirus disease 2019 mRNA vaccination, necessitating renal replacement therapy (RRT). The patient presented to the hospital two days post-vaccination with muscle pain in both lower extremities and anuria. Diagnostic tests revealed elevated creatinine kinase (CK) levels of 160,000 IU/L and serum creatinine levels of 6.59 mg/dL, confirming AKI due to rhabdomyolysis. Intravenous therapy was ineffective, leading to the utilization of online hemodiafiltration. Following treatment, CK levels normalized, and the renal function fully recovered.

Nintedanib inhibits disease progression in patients with idiopathic pulmonary fibrosis (IPF) and dramatically improves the lung function in patients known to be super-responders (SRs). However, there are no reports on quantitative high-resolution computed tomography (HRCT), and the HRCT imaging characteristics of SRs remain unknown. We herein present the case of a 66-year-old man with IPF who was a SR to nintedanib treatment, which showed a marked improvement compared to other patients with IPF upon quantitative HRCT evaluation using the artificial intelligence (AI) software 3D Slicer. This study is worth reporting because a quantitative HRCT assessment using AI may be necessary to understand the SR characteristics.

Plasmablastic lymphoma (PBL) is an aggressive subtype of large B-cell lymphoma associated with immunodeficient states, such as HIV and EBV infections, and aging. PBL frequently harbor STAT3 mutations, which are crucial for their development. We herein report a rare case of PBL complicated by T-cell large granular lymphocytic leukemia (T-LGLL) and pure red cell aplasia (PRCA). The patient was treated with six cycles of chemotherapy and developed transfusion-dependent anemia. Large granular lymphocytes and T-cell receptor rearrangement were detected, thus leading to the diagnosis of T-LGLL and PRCA. This is the first documented case of PBL with T-LGLL and PRCA, offering insight into PBL's pathophysiology and the complications of PBL.

We herein report a case of concurrent immune thrombocytopenia (ITP) and ulcerative colitis (UC) that achieved remission following mesalazine treatment. A 16-year-old girl presented with severe thrombocytopenia, abdominal pain, and bloody stool. She was initially diagnosed with ITP and then was treated with prednisolone, resulting in an immediate improvement of symptoms. Upon tapering the steroids, the symptoms recurred, thus leading to a subsequent diagnosis of UC via colonoscopy. Treatment with mesalazine promptly induced the remission of both ITP and UC, which was sustained. We reviewed 24 previously documented cases in which the simultaneous flares of UC and ITP were successfully managed.

A 54-year-old woman with persistent fatigue and a high fever presented with central pontine myelinolysis (CPM) on brain imaging, despite the absence of neurological symptoms. The patient had a slightly low serum sodium concentration. Further investigation led to a diagnosis of primary splenic diffuse large B-cell lymphoma (PS-DLBCL). Brainstem auditory-evoked potentials (BAEP) indicated minor abnormalities. CPM was successfully resolved after splenectomy and immunochemotherapy. This is the first reported case of CPM in a patient with PS-DLBCL confirmed by a pathological diagnosis following splenectomy and BAEP-detected abnormalities that improved with immunochemotherapy.

Direct compression of the oculomotor nerve (OcN) is usually accompanied by OcN palsy, with an abnormal pupillary function. We herein present the case of a 76-year-old man with pupil-sparing paresis of the right inferior rectus muscle. A radiological examination revealed an epidermoid cyst at the right cerebellopontine angle, which came in contact with the right OcN. However, surgical intervention revealed compression of the inferomedial aspect of the OcN by the right posterior cerebral artery. Decompression of the nerve gradually improved the ocular movement. This unique case offers insights into the potential topographical arrangement of the OcN fibers.

We encountered a 67-year-old Japanese man with GNE myopathy and homozygous variants (c.1807G>C, p.V603L) of the GNE gene. The patient developed weakness in the left foot at 24 years old and could only move his wrist joints and hands 43 years after the onset. This genotype is the most common variant and causes severe muscle involvement; however, the distal upper extremities are preserved until the end-stage of the disease. Although severe heart failure is rare in GNE myopathy, mild cardiac dysfunction (ejection fraction 46.1%) was observed. Furthermore, respiratory dysfunction was noted with a preserved diaphragm.

Myasthenia gravis (MG) is an autoimmune disease characterized by muscle weakness caused by antibodies targeting acetylcholine receptors (AChRs) or muscle-specific kinase (MuSK). Double-seropositive MG (DSP-MG) caused by both antibodies is rare. We herein report a 78-year-old woman who developed DSP-MG after coronavirus disease 2019 infection. She presented with neck muscle fatigue and upper-limb weakness. Elevated levels of anti-AChR and anti-MuSK antibodies were detected. The patient responded well to the immunosuppressive therapy. This case highlights the potential for various autoimmune neurological conditions post-viral infection and the significance of prompt recognition and treatment.

Familial Mediterranean fever (FMF) is the most prevalent hereditary autoinflammatory disease and is caused by the MEFV gene. In patients carrying MEFV exon 10 variants, FMF usually develops at an early age. A 76-year-old Japanese man presented with a periodic fever lasting 2-3 days, chest pain, and abdominal pain. An MEFV gene analysis revealed compoundheterozygous M694I/E148Q/L110P. Colchicine treatment (0.5 mg/day) improved the patient's symptoms. This is the first case report of an elderly Japanese patient with FMF onset in the 70s carrying the MEFV exon 10 variant.

A 40-year-old Japanese man with human immunodeficiency virus presented with disturbance of consciousness 2 years after dropping out. He was emaciated without any zoster skin lesions, and his CD4+ lymphocyte count was 4 cells/μL. Disseminated Mycobacterium avium infection and pneumocystis pneumonia were diagnosed, and a brain biopsy revealed varicella zoster virus (VZV) encephalitis with multiple real-time polymerase chain reaction tests and immunochemistry. Antiretroviral therapy and intravenous acyclovir were administered, and the patient survived with neurological sequelae. VZV encephalitis is rare, particularly among people with acquired immunodeficiency syndrome, but it is a significant disease with a poor prognosis.

Mild glucose intolerance (positive urine glucose or mild hyperglycemia) during health checkups in young, lean women may require specialist consultation. We herein report a 31-year-old Japanese woman with mild hyperglycemia detected during a checkup who was diagnosed with overt diabetes in pregnancy and HNF4A-MODY postpartum, without prior follow-up. This case highlights the following: MODY may be present in young, lean women with mild glucose intolerance, and preconception care may improve pregnancy outcomes. Fetal outcomes vary with MODY subtype. Preconception care is important.