Objective Metabolic dysfunction-associated steatotic liver disease (MASLD) is a chronic liver disease associated with metabolic comorbidities. However, the risk factors for atherosclerotic cardiovascular disease (ASCVD) in these patients remain unclear. Therefore, this study investigated predictors of ASCVD in patients with MASLD.
Methods This single-center retrospective study examined 372 patients with MASLD ≥40 years old with liver biopsies and available Hisayama scores (median follow-up, 7.4 years; range, 0.6-22 years). We compared baseline characteristics, liver histology (stage, lobular inflammation, steatosis, and hepatocellular ballooning), fibrosis-4 (FIB-4) index (<1.3/1.3-2.66/≥2.67), and laboratory data between patients with and without ASCVD. A predictive model for the onset of ASCVD based on the Hisayama score (low/intermediate/high) and ASCVD incidence was evaluated according to the liver fibrosis stage and FIB-4 index.
Results ASCVD incidence was 11.1/1,000 person-years, with cumulative incidences of 4.4%, 9.0%, 14%, and 32% at 5, 10, 15, and 20 years, respectively. Regarding the incidence of ASCVD, the liver fibrosis stage and an FIB-4 index ≥2.67 were not significant predictors, but type IV collagen 7S was a significant predictor. The incidence of ASCVD was higher in the intermediate- and high-risk Hisayama score groups than in the low-risk group. In the multivariate Cox proportional hazards model, the Hisayama score and type IV collagen 7S predicted the incidence of ASCVD more accurately than an FIB-4 index ≥2.67.
Conclusion The Hisayama score predicted ASCVD risk in patients with MASLD. These findings will help predict and improve the prognosis of MASLD.
Objective Gastroesophageal reflux disease (GERD) is a condition characterized by reflux of gastric contents, leading to damage to the esophageal mucosa and/or unpleasant symptoms, with a global prevalence of 13%. Although acid-suppressive medications are currently the most effective treatments for GERD, they may be ineffective against its symptoms. We therefore investigated the efficacy of a physical exercise program for the treatment of intractable GERD symptoms.
Methods Fifteen participants who underwent upper gastrointestinal endoscopy were included in this study. They received acid-suppressive drugs for more than eight weeks and had a score of ≥8 on the Frequency Scale for the Symptoms of GERD (FSSG). Participants were instructed to perform a 2-week interventional exercise program comprising drinking saliva 10 times in the bridge position (bridge dry swallowing exercise). This involved swallowing saliva without any food or drink while the hip was raised in the supine position. Changes in the FSSG scores after exercise were examined (UMIN000047829).
Results Among the 15 participants, 7 were men, with a median age of 59 years old. Each participant had mild reflux esophagitis or no mucosal injuries. All participants completed the exercise program safely. Of the 15 participants, 13 demonstrated an improvement in the FSSG score after the intervention. The total FSSG score improved significantly from a median of 17 to 11 (p <0.001). Five participants discontinued acid-suppressive drugs after the intervention ended.
Conclusions Bridge dry swallowing exercises can improve the intractable symptoms of GERD. However, further studies are required to verify its efficacy and safety.
Objective To determine the clinical background factors of patients with type 2 diabetes who showed deterioration of defecation status after hospitalization.
Methods The defecation status of 128 patients with type 2 diabetes who were admitted to our department for diabetes education was evaluated for 7 days after hospitalization. New-onset constipation was diagnosed when patients without constipation before hospitalization met the criteria for constipation after hospitalization. Worsening constipation was diagnosed when patients with constipation required a larger laxative dose than that before hospitalization. New-onset constipation and worsening constipation were defined as the deterioration of defecation status. The major outcome was the incidence of deterioration of defecation status after hospitalization.
Results After hospitalization, 23 of 99 patients who had no constipation before hospitalization developed new-onset constipation. Among the 29 patients with constipation before hospitalization, 9 showed worsening constipation. Overall, 52 patients (41% of all subjects) had constipation, and 32 (25% of all subjects) were diagnosed with deterioration in defecation status. In univariate logistic regression analyses, patient age, albuminuria, diabetic peripheral neuropathy, aspartate aminotransferase, estimated glomerular filtration rate, HbA1c, and brachial-ankle pulse wave velocity were significantly associated with deterioration in defecation status. In the multivariate logistic regression analyses, albuminuria was the only factor that showed a significant association with the deterioration of defecation status after hospitalization.
Conclusion Constipation is highly prevalent among hospitalized patients with type 2 diabetes. Paying attention to albuminuria is useful for facilitating an appropriate response to the deterioration of defecation status in patients with type 2 diabetes after hospitalization.
Objective Nursing home residents with a high risk of multidrug-resistant organism infection pose a complex challenge to broad-spectrum empirical antimicrobial therapy, particularly those infected with extended-spectrum β-lactamase-producing Enterobacteriaceae. The present study compared the efficacy of piperacillin-tazobactam and carbapenems as empirical antimicrobial treatments for patients with sepsis from nursing homes.
Methods Using a nationwide inpatient database in Japan, we identified patients diagnosed with sepsis within two days of admission from nursing homes between 2018 and 2021. We selected patients who received intravenous piperacillin-tazobactam or carbapenems within two days of admission. In-hospital mortality was compared between the piperacillin-tazobactam and carbapenem groups using inverse probability of treatment weighting.
Results We identified 8,025 eligible patients. Of these, 3,391 (42%) received piperacillin-tazobactam, and 4,634 (58%) received carbapenems within 2 days of admission. The inverse probability of treatment weighting analysis showed no significant difference in in-hospital mortality between the groups (31.6% in the piperacillin-tazobactam group and 32.8% in the carbapenem group; risk difference, 1.2%; 95% confidence interval, -3.2% to 0.9%).
Conclusion Carbapenems and piperacillin-tazobactam as empirical antimicrobial therapy in patients with sepsis from nursing homes were associated with comparable in-hospital mortality rates. These findings highlight the importance of making decisions regarding broad-spectrum empirical antimicrobial therapy.
Amiodarone is an antiarrhythmic drug that is widely used for atrial fibrillation and other refractory arrhythmias. Although beneficial, its long-term administration is associated with adverse effects on various organs. One patient presented with amiodarone-induced liver injury, which led to liver failure. Computed tomography revealed a gradual increase in hepatic density over a long period following the initiation of amiodarone. Despite the discontinuation of the drug, the patient developed hepatic encephalopathy and subsequently died. This outcome highlights the drug's extended half-life, which caused persistent end-organ damage even after its withdrawal. Drug titration to the lowest effective dose and careful monitoring of annual liver function tests are important.
We herein report three patients with Fontan-associated liver disease (FALD) who developed hepatocellular carcinoma (HCC). All three patients had very high serum alpha-fetoprotein (AFP) concentrations, and their HCCs were located in the peripheral areas of the liver. To identify the characteristics of FALD-HCC, we reviewed previously published case reports that included descriptions of imaging. This review revealed that FALD-HCC is generally located in the peripheral areas of the liver and AFP concentrations are usually high. Thus, it is important that follow-up of patients with FALD include repeated AFP concentrations and abdominal imaging.
A 61-year-old woman who had been taking olmesartan for 7 years complained of epigastric pain, diarrhea, loss of appetite, and weight loss. Esophagogastroduodenoscopy revealed roughened mucosa and erosions in the stomach and duodenum. An endoscopic biopsy failed to identify the cause of the mucosal disorder. Small-bowel capsule endoscopy revealed villous atrophy in the small bowel, which led to suspicion of olmesartan-associated sprue-like enteropathy and gastritis. After the discontinuation of olmesartan, the symptoms and gastric mucosal findings improved. A final diagnosis of olmesartan-associated gastritis was confirmed. Olmesartan-associated gastritis should be considered in patients taking olmesartan for upper gastrointestinal symptoms.
An 81-year-old woman presented to our hospital with dyspnea. She had been treated with trastuzumab for nine years. Chest radiography revealed pleural effusion. Transthoracic echocardiography revealed dyskinesis of the interventricular septum (IVS). Cardiac magnetic resonance imaging revealed increased native T1 values at the IVS and apex, indicating myocardial edema or fibrosis in these areas. A transthoracic echocardiogram after half a year revealed an increase in left ventricular ejection fraction from 25% to 48%. Serial transthoracic echocardiography and cardiac magnetic resonance imaging were useful for evaluating the cardiac structure and function in the present case of delayed trastuzumab-induced myocardial injury.
We herein report the first Japanese case of familial hypercholesterolemia (FH) caused by a specific mutation in apolipoprotein E (APOE) [c.500_502delTCC (p.Leu167del)]. The proband was a 38-year-old man diagnosed with FH based on the clinical findings. Genetic testing revealed a rare pathogenic variant in APOE but no relevant mutation in any "FH genes," including low-density lipoprotein (LDL) receptor, proprotein convertase subtilisin/kexin type 9, apolipoprotein B, and LDL receptor adaptor protein 1. His LDL cholesterol level was well controlled by the introduction of statins, ezetimibe, and proprotein convertase subtilisin/kexin type 9 inhibitors. Cascade and reverse cascade screening identified his son and father as also having FH caused by this particular mutation.
A 67-year-old man with a history of heart failure (HF) secondary to ischemic cardiomyopathy, atrial fibrillation, and left atrial appendage occlusion (LAAO) developed worsening HF secondary to severe functional mitral regurgitation. Consequently, transcatheter edge-to-edge repair (TEER) was performed. Despite successful TEER, a large thrombus in the left atrium (LA) was unexpectedly discovered on postoperative day 3. Thrombus formation may be related to changes in the blood stream in the LA, endothelial injury by septal puncture, and an insufficient anti-thrombotic regimen. This case highlights the potential for the development of subacute LA thrombosis following TEER, even in patients with prior LAAO.
A 67-year-old man on intense immunosuppressive therapy after heart transplantation for end-stage hypertrophic cardiomyopathy 7 years ago developed severe dyspnea and was admitted to our hospital. His serum severe acute respiratory syndrome coronavirus 2 antigen test was positive, and he was diagnosed with coronavirus disease 2019 (COVID-19)-related pneumonia. He was started on ventilatory management for severe respiratory failure and remdesivir for COVID-19, with careful adjustment of immunosuppressive drugs. However, unexpectedly prolonged muscle weakness necessitated transfer to a rehabilitation facility. Although the COVID-19 pandemic has subsided, it is still considered a risk in post-transplant cases. Infection control is considered critical in heart transplant recipients, especially in those receiving intensified immunosuppressive drugs.
An 85-year-old woman with diffuse large B-cell lymphoma developed severe hypophosphatemia (serum phosphate 0.3 mg/dL) concomitant with acute kidney injury (serum creatinine 2.05 mg/dL) following chemotherapy. Because urine phosphate was undetectable, hypophosphatemia was likely due to the vigorous uptake of phosphate into the rapidly proliferating tumor cells, also known as tumor genesis syndrome (TGS), and acute kidney injury was potentially attributed to the antibiotics sulfamethoxazole/trimethoprim. Oral phosphate supplementation and antibiotic discontinuation alleviated both the abnormalities. This case was unusual, as tumorigenesis syndrome is seldom seen in patients with lymphoma, and acute kidney injury usually leads to hyperphosphatemia. The present case emphasizes the importance of vigilance in hypophosphatemia due to TGS during chemotherapy.
Erdheim-Chester disease (ECD) is a rare type of non-Langerhans cell histiocytosis, characterized by the infiltration of disease-specific foamy histiocytes, polymorphic granulomas, and fibrosis. Although cardiovascular involvement is observed radiologically in approximately half of ECD patients, only a few reports have described its pathological features. We herein report the autopsy of an ECD patient with pulmonary, cardiovascular, and retroperitoneal involvement that may have caused his death. Autopsy revealed the pathological association of coronary and renal arterial stenosis with the BRAFV600E gene mutation. BRAF mutations should be considered in patients with ECD, especially in those with arterial lesions.
A 45-year-old woman with Mycobacterium abscessus pulmonary disease was treated with multidrug therapy, including clofazimine, at Fukujuji Hospital. Six months after clofazimine initiation, the treatment was discontinued at the patient's request due to hyperpigmentation. Pigmentation is a critical side effect of clofazimine. We quantified clofazimine-induced pigmentation on her face using NeoVoirI® and measured the serum concentration of clofazimine during the administration period. The patient's skin tone score closely correlated with the serum concentration of clofazimine. However, 14 months after the discontinuation of clofazimine, clofazimine-induced pigmentation was still not completely resolved.
Nodal Epstein-Barr virus-positive T/NK-cell lymphoma (EB-nTNKL) is an extremely rare disease characterized by an aggressive clinical course and poor prognosis, for which treatment strategies have not yet been established. We herein report a young man with EB-nTNKL. Although initial chemotherapies, including L-asparaginase, failed to produce a good response, subsequent myeloablative allogeneic hematopoietic stem cell transplantation (alloHSCT) resulted in favorable disease control and a long-term disease-free survival. The prompt performance of alloHSCT using an available donor source at that time, regardless of whether or not the initial chemotherapy was effective, could be critical to saving patients with this otherwise fatal disease.
Up to one-third of lymphoma cases involve the nervous system. Miller-Fisher syndrome (MFS) associated with lymphoma is extremely rare. We herein report a case of primary central nervous system lymphoma initially mimicking MFS in a 70-year-old man who presented with subacute unsteady gait and diplopia. A neurological examination revealed unilateral ophthalmoplegia, ataxia, and areflexia. The patient tested positive for anti-GQ1b antibodies, so MFS was initially suspected. However, the progression extended over one month. Subsequently, disturbance of consciousness was observed. Cranial magnetic resonance imaging revealed lesions in the periventricular fourth ventricle, and a brain biopsy indicated diffuse large B-cell lymphoma.
Anti-GM1 antibodies are associated with Guillain-Barré syndrome (GBS), primarily peripheral neuropathy. However, there are cases of anti-GM1 IgG antibody-positive GBS with upper motor neuron (UMN) signs. We herein report a case of gastrointestinal infection followed by subacute gait disturbance with predominant signs of UMN on a neurological examination. The serum and cerebrospinal fluid tests were positive for anti-GM1 and anti-asialo-GM1 IgG antibodies. An electrophysiological evaluation revealed normal nerve conduction and prolonged central motor conduction times. No magnetic resonance imaging abnormalities were observed. The symptoms improved with treatment, which was accompanied by decreased antibody titers. This case highlights the fact that anti-GM1 IgG-associated disorders may present with predominant UMN involvement.
We herein report a 48-year-old man with neurosyphilis manifesting as limbic encephalitis, initially suspected to be autoimmune limbic encephalitis. The patient exhibited rapid behavioral changes, and magnetic resonance imaging showed high-intensity lesions in both medial temporal lobes. The diagnosis was based on symptoms, cerebrospinal fluid abnormalities, and positive serum tests for syphilis. Notably, the cerebrospinal fluid immunoglobulin G index was markedly elevated (4.91). This case highlights the diagnostic challenges in differentiating neurosyphilis from autoimmune limbic encephalitis, given the increasing atypical presentation. A markedly elevated immunoglobulin G index may serve as a valuable diagnostic indicator for neurosyphilis in such cases.
It is difficult to distinguish idiopathic multicentric Castleman disease (iMCD) from immunoglobulin G4-related disease (IgG4-RD). A 47-year-old man was diagnosed with venous thrombosis in the right lower extremity. Multiple lymphadenopathies and splenomegaly were incidentally detected. His serum IgG4 levels were high, and the biopsied lymph nodes showed high plasma cell infiltration with many IgG4-positive cells between the follicles. He was initially diagnosed with IgG4-RD and was administered prednisolone 30 mg/day; however, inflammation and IgG4 persisted. The patient was rediagnosed with iMCD and treated with tocilizumab, which led to an improvement of his condition. When diagnosing IgG4-RD, it is therefore important to consider iMCD in the differential diagnosis.
Pseudomonas otitidis bacteremia is rare. We herein report a case of bacteremia caused by P. otitidis in a patient with advanced appendiceal cancer. A 79-year-old Japanese man developed infection when he was admitted to our hospital for palliative care. Blood culture revealed the presence of Pseudomonas spp. Matrix-assisted laser desorption/ionization time-of-flight mass spectrometry was used to identify the organism accurately. The patient was treated with a two-week course of piperacillin/tazobactam, and the bacteremia was successfully controlled. Clinicians should be aware that P. otitidis can cause bacteremia, particularly in immunocompromised patients.
Pott's puffy tumor is a rare complication of frontal sinusitis characterized by frontal bone osteomyelitis with a subperiosteal abscess typically presenting with forehead swelling. We herein report a 21-year-old man with Pott's puffy tumor presenting as eyelid swelling on the opposite side of the sinusitis, without typical forehead swelling. Initially treated for sinusitis and pre-septal cellulitis with poor response, head magnetic resonance imaging revealed bilateral subdural abscesses and osteomyelitis of the frontal and bilateral parietal bones, leading to the diagnosis. When frontal sinusitis and eyelid swelling are unresponsive to antibiotics, Pott's puffy tumor should be considered, even in the absence of forehead swelling.
A 61-year-old man who underwent allogeneic bone marrow transplantation presented to our hospital with severe abdominal pain and no significant rash. Computed tomography (CT) revealed periarterial fat stranding around the celiac and superior mesenteric arteries. Skin and gastric biopsies revealed varicella-zoster virus (VZV) infection, leading to the diagnosis of VZV infection. Symptoms of VZV infection are severe and potentially fatal, making early intervention important. The characteristic findings of periarterial fat stranding around the celiac and superior mesenteric arteries on CT may play an important role in the early diagnosis of VZV infection.
We herein report a case of cutaneous squamous cell carcinoma (SCC) characterized by paraneoplastic hypercalcemia-leukocytosis syndrome. The patient presented with systemic symptoms, including anorexia, a fever, and a tumoral lesion on the upper arm. Laboratory test results revealed hypercalcemia and leukocytosis. A tissue biopsy confirmed SCC, and further investigation revealed elevated parathyroid hormone-related protein (PTHrP) and granulocyte-colony stimulating factor (G-CSF) levels. Immunostaining demonstrated G-CSF production by the tumor cells. Radiation therapy was administered, which improved leukocytosis and decreased G-CSF and PTHrP levels. Through a case report and literature review, we explored the clinical characteristics of tumors that produce G-CSF and PTHrP.