Objective To examine the trends and relationships between nonalcoholic fatty liver disease (NAFLD) and hypertension, type 2 diabetes mellitus (T2DM), and dyslipidemia from fiscal year (FY) 2008, when specific health checkups (SHCs) were initiated in Japan, to FY 2019 and the relationship between NAFLD trends and dietary nutrition.

Methods A total of 48,332 participants (25,121 men and 23,211 women) diagnosed with NAFLD who underwent health checkups, including ultrasonography, from FY 2008 to FY 2019 were included. A fatty liver was diagnosed using ultrasonography. The dietary nutrient intake status was based on data from the National Health and Nutrition Survey, Japan.

Results Over 12 years, NAFLD prevalence increased from 26.9% to 43.1% in men (p<0.0001) and from 9.9% to 17.9% in women (p<0.0001) in all body mass index (BMI) groups except for obese II (according to the World Health Organization Asia-Pacific criteria) in men and underweight in women and almost all age groups. T2DM prevalence increased in men (from 9.0% to 10.7%, p=0.0234), and obesity and higher waist circumference rates increased in women (from 16.0% to 18.0%, p=0.0059 and from 8.1% to 10%, respectively, p=0.0006). The dietary nutrient intake increased with regard to the total fat, fat/energy ratio, saturated fatty acids, monounsaturated fatty acids, and n6/n3 fatty acid ratio in both men and women, and these nutrient trends were correlated with NAFLD prevalence (all p≤0.0005).

Conclusion In Japan, NAFLD increased in both men and women regardless of the BMI and age, even after starting SHCs. An unbalanced fat intake may be one of the major reasons for this increase.

Objective Contrast agents used for radiological examinations are an important cause of acute kidney injury (AKI). We developed and validated a machine learning and clinical scoring prediction model to stratify the risk of contrast-induced nephropathy, considering the limitations of current classical and machine learning models.

Methods This retrospective study included 38,481 percutaneous coronary intervention cases from 23,703 patients in a tertiary hospital. We divided the cases into development and internal test sets (8:2). Using the development set, we trained a gradient boosting machine prediction model (complex model). We then developed a simple model using seven variables based on variable importance. We validated the performance of the models using an internal test set and tested them externally in two other hospitals.

Results The complex model had the best area under the receiver operating characteristic (AUROC) curve at 0.885 [95% confidence interval (CI) 0.876-0.894] in the internal test set and 0.837 (95% CI 0.819-0.854) and 0.850 (95% CI 0.781-0.918) in two different external validation sets. The simple model showed an AUROC of 0.795 (95% CI 0.781-0.808) in the internal test set and 0.766 (95% CI 0.744-0.789) and 0.782 (95% CI 0.687-0.877) in the two different external validation sets. This was higher than the value in the well-known scoring system (Mehran criteria, AUROC=0.67). The seven precatheterization variables selected for the simple model were age, known chronic kidney disease, hematocrit, troponin I, blood urea nitrogen, base excess, and N-terminal pro-brain natriuretic peptide. The simple model is available at http://52.78.230.235:8081/

Conclusions We developed an AKI prediction machine learning model with reliable performance. This can aid in bedside clinical decision making.

Objective Azacitidine (AZA) has been the standard of care for elderly patients with high-risk myelodysplastic syndromes (MDS). However, reliable clinical predictors of outcome have yet to be identified. The prognostic value of fetal hemoglobin (HbF) levels has been reported for decitabine therapy. We evaluated pretreatment HbF levels in AZA monotherapy as a prognostic marker in MDS/acute myeloid leukemia (AML).

Methods This study included chemotherapy-naïve patients who had received seven-day treatment schedules of AZA and whose HbF levels were measured at the onset of treatment between March 2011 and July 2020. Patients were grouped into HbF-normal (<1.0%) or HbF-elevated (≥1.0%) groups. Responses were classified according to the International Working Group 2006 criteria.

Patients Twenty-nine patients were included and classified as having either MDS (n=21), chronic myelomonocytic leukemia (n=5), myelodysplastic/myeloproliferative neoplasm unclassifiable (n=1), or AML with <30% marrow blasts (n=2) based on the World Health Organization 2016 diagnostic criteria. According to the revised International Prognostic Scoring System classification, 20/29 patients were at intermediate, high, or very high risk. Pretreatment HbF levels were elevated in 13/29 patients.

Results The median follow-up duration was 13.0 (range 1.5-93.5) months. The HbF-elevated group was associated with a significantly higher hematologic improvement rate (76.9% vs. 25%, p=0.009) and better overall survival (median, 21.0 vs. 13.0 months, p=0.048) than the HbF-normal group.

Conclusion These results suggest that elevated pretreatment HbF levels can predict better outcomes in patients with MDS/AML treated with AZA.

A 61-year-old woman was administered 35 cycles of pembrolizumab for the treatment of recurrent endometrial cancer, achieving a complete response. She presented with asymptomatic pancreatic enlargement and elevated hepatobiliary enzymes, but amylase and lipase levels were within the normal ranges. Intrapancreatic bile duct stenosis due to pancreatic enlargement was present, mimicking autoimmune pancreatitis on computed tomography performed before the onset of clinical manifestations. A histological examination of a biopsy specimen showed lymphocyte and plasma cell infiltration with dense fibrosis in the stroma. The patient was successfully treated with oral prednisolone. There were no manifestations of recurrent pancreatitis after tapering the prednisolone dose.

A 90-year-old man presented with a 3-day history of general malaise. He was febrile (39.3°C) but the initial evaluation did not reveal the cause of the fever. After admission, Bacillus subtilis and Fusobacterium nucleatum were grown from multiple sets of blood cultures. In addition, contrast-enhanced computed tomography revealed thrombi in the portal vein and superior mesenteric vein; he was diagnosed with pylephlebitis. After receiving antimicrobial treatment and anticoagulation, the patient was cured. Pylephlebitis is a rare condition and may be the cause of unknown fevers. This is the first reported case of pylephlebitis caused by Bacillus subtilis.

A 55-year-old patient was admitted for variceal treatment, a complication of chronic portal hypertension and liver cirrhosis. Imaging studies revealed prominent duodenal varices, the pancreaticoduodenal vein as its afferent pathway, a drainer vessel into the inferior vena cava, and a paraumbilical vein. We successfully performed complete obliteration of the varix, including its afferent and efferent vessels, via the paraumbilical vein approach.

An 86-year-old man presented to our hospital with symptoms of diarrhea and bloody stool, which had manifested two weeks after receiving his third severe acute respiratory syndrome coronavirus 2 mRNA vaccination. Colonoscopy revealed diffuse, rough-surfaced mucosa extending from the ascending colon to the rectum. Despite attempting probiotic treatment, the patient's condition did not improve, leading to admission. Endoscopic findings at admission worsened. Based on endoscopic and histopathological findings, the patient was diagnosed with ulcerative colitis. Corticosteroids and 5-aminosalicylic acid were administered, and the clinical symptoms improved. Subsequently, the disease worsened during steroid tapering, and filgotinib was added, leading to steroid-free remission.

We herein report a 49-year-old woman who developed "happy heart syndrome" while watching a family theater performance. She was followed up with cardiac magnetic resonance imaging (CMR). The time course of the CMR findings was similar to that previously reported for Takotsubo cardiomyopathy (TTC), including the form of "broken heart syndrome." CMR findings for "happy heart syndrome" have not been sufficiently investigated. However, the CMR findings in this case suggest that "happy heart syndrome" and other conventional TTC including "broken heart syndrome" are clinically a single disease, at least from an imaging aspect.

Myocardial calcification in myocarditis is rare and may be linked to poor outcomes. We herein report a case of fulminant myocarditis with massive myocardial calcification and its pathological outcomes at autopsy. A 49-year-old man experienced chest pain and was diagnosed with acute myocarditis. His cardiac function did not recover despite mechanical circulatory support in combination with V-A extracorporeal membrane oxygenation and IMPELLA CP^®. He eventually developed sepsis and gastrointestinal bleeding and died on day 27. Diffuse myocardial calcification was observed on computed tomography at autopsy. The pathological autopsy depicted that calcification filled every myocardial cell in the left ventricle.

Ruptured sinus of Valsalva aneurysm (RSOVA) is a rare cardiac condition associated with high morbidity and mortality rates. We herein report a 35-year-old man with a history of ventricular septal defect (VSD). He had a history of interrupted hospital visits and presented to the emergency department with dyspnea, palpitations, and dizziness for a few days. Auscultation detected a continuous murmur. Transthoracic echocardiography followed by transesophageal echocardiography demonstrated RSOVA in the right ventricle with an aorto-right ventricular fistula. The fistula was resected, and the aneurysm was surgically repaired. The patient made a good recovery.

A 29-year-old man presented with liver damage, and a liver biopsy was performed, but the cause was unclear. Thereafter, he was referred to our hospital. We found that he had been unable to consume carbohydrates in his diet and preferred fried chicken since childhood. In addition, he had shown disturbance of consciousness and abnormal behavior while he had been in prison, where dietary intake had been restricted. A plasma amino acid analysis revealed hypercitrullinemia. Therefore, we suspected adult-onset type II citrullinemia (CTLN2). Genetic testing showed pathologic variations in the SLC25A13 gene, which allowed us to make a definite diagnosis of CTLN2.

We herein report a case of bilateral pneumothorax after a unilateral transbronchial lung cryobiopsy (TBLC). A 73-year-old man with no history of cardiothoracic surgery underwent a TBLC for the reevaluation of interstitial lung disease. Five hours later, he developed bilateral pneumothorax, pneumomediastinum, and subcutaneous emphysema. He underwent bilateral chest drainage and was discharged 18 days later. The lung biopsy specimens obtained from the TBLC contained visceral pleura and bronchial cartilage, suggesting bronchial injury as the cause of the bilateral pneumothorax.

Interferon is an emerging treatment option for myeloproliferative neoplasms (MPNs), especially for polycythemia vera (PV). Previous studies of interferon used therapeutically for hepatitis C have demonstrated that one of the most important adverse events associated with interferon treatment is thyroid dysfunction, and a management strategy for thyroid dysfunction has been established. However, whether or not the recommendation is also suitable for MPN settings is unclear. In this study, one PV patient developed silent thyroiditis during a phase 2 study of ropeginterferon α-2b. This case suggests that thyroid dysfunction is an important clinical issue to consider in interferon treatment for PV.

Essential thrombocythemia is a myeloproliferative neoplasm. Ischemic stroke is frequently the first manifestation of essential thrombocythemia. We herein report a patient with JAK2V617 mutation-positive essential thrombocythemia who developed recurrent ischemic stroke with rapid development of intracranial artery stenosis and subsequently underwent successful mechanical thrombectomy. The high JAK2V617F allele burden in our patient (58.4%) may have affected the patient's condition. We discuss similar reports in the literature and the possible pathophysiologic mechanism of large artery involvement in these patients.

In primary Sjögren's syndrome, it is extremely rare to observe subacute progressive lower-body parkinsonism with severe sensory hearing loss responsive to corticosteroid therapy. Sjögren's syndrome can cause heterogeneous symptoms; therefore, its diagnosis and introduction of treatment are prone to be delayed, particularly in cases without sicca symptoms or seronegative cases, which are more likely to be seen in patients with neurological complications. This report may help clinicians identify atypical early neurological symptoms in primary Sjögren's syndrome.

Paraneoplastic cerebellar degeneration (PCD) is a paraneoplastic neurological syndrome that is rarely accompanied by seropositivity with a combination of multiple antibodies. We herein report a 50-year-old man with PCD accompanied by small-cell lung cancer (SCLC). This patient was seropositive for anti-glutamic acid decarboxylase 65, anti-SRY-related HMG-box gene 1 and anti-voltage-gated calcium channel antibodies. After chemoradiation therapy without immunotherapy, cerebellar ataxia of the trunk and limbs markedly improved, along with a notable amelioration of SCLC. This case suggests that tumor therapy should be started immediately and that a panel of anti-neuronal antibodies should be evaluated when PCD with SCLC is suspected.

We herein report a 78-year-old woman with Gaucher disease (GD) who was initially diagnosed with GD type 1, had been receiving long-term enzyme replacement therapy since 58 years old, and developed neurological manifestations in her 70s. The neurological manifestations included myoclonic seizures and progressive cognitive decline. Although it is rare for GD patients to first develop neurologic manifestations at such an advanced age, physicians engaged in long-term care for GD patients should be alert for this possibility.

A 58-year-old woman with rheumatoid arthritis was diagnosed with methotrexate-associated Hodgkin lymphoma. After receiving several chemotherapy regimens, she started nivolumab treatment. Two weeks later, she was hospitalized with worsening finger, wrist, and elbow joint pain. A synovial biopsy of the wrist joint showed villous synovial proliferation and linear infiltration of CD68-/CD3-positive T cells (with more CD8 than CD4 T cells) but no CD20-positive B cells or CD138-positive macrophages. These findings corresponded to synovitis associated with immune-related adverse events, which are induced mainly by T cells and are different from typical rheumatoid arthritis (RA), in which B cells play a central role.

A 69-year-old woman suffering with multiple myeloma developed coronavirus disease 2019 (COVID-19). Shortly after administration of remdesivir, she presented with symptoms of facial flushing, wheezing, and hypoxemia. Subsequently, thrombocytopenia and hypofibrinogenemia rapidly manifested, leading to a diagnosis of enhanced fibrinolytic-type disseminated intravascular coagulopathy (DIC). This clinical presentation was considered an immediate hypersensitivity reaction with associated coagulation abnormalities induced by remdesivir. Although remdesivir is generally considered safe and efficacious in the treatment of COVID-19, physicians should remain vigilant regarding the potential for severe adverse events associated with this medication.