Objective With recent advances in endoscopic modalities, small bowel vascular lesions (SBVLs) are often now detected in patients with gastrointestinal bleeding. Given the high invasiveness of endoscopic treatment, it is important to select patients at high risk for bleeding. To assess the risk of rebleeding in patients with SBVLs as a systemic disease rather than a gastrointestinal disease in relation to their general health.
Methods We retrospectively analyzed the clinical data of 55 patients with SBVLs among patients with obscure gastrointestinal bleeding. The possible association between the clinical findings and the updated Charlson comorbidity index with rebleeding was evaluated.
Results Gastrointestinal rebleeding occurred in 20 patients (36.4%) during the follow-up period. The presence of multiple comorbidities as indicated by an updated Charlson comorbidity index of ≥4 was a risk factor for rebleeding (hazard ratio, 3.64; p=0.004). Other risk factors were arteriosclerosis of the superior mesenteric artery and multiple SBVLs. Endoscopic hemostasis and the discontinuation of antithrombotic medications were not significantly associated with rebleeding. Patients with a high updated Charlson comorbidity index had a high risk of death of causes other than gastrointestinal rebleeding.
Conclusion Gastrointestinal rebleeding is not a rare condition among patients with SBVLs. Patients with poor general health may therefore have a higher risk of rebleeding.
Objective Coronary plaques with low attenuation on computed tomography (CT) angiography may indicate vulnerable plaques. However, plaque CT attenuation is reported to be significantly affected by intracoronary attenuation. Recently, the diluted-contrast injection protocol was established to facilitate more uniform intracoronary attenuation than can be achieved with the generally used body-weight-adjusted protocol. We validated the relationship between low-attenuation plaque on CT and lipid-rich plaque using integrated backscatter-intravascular ultrasound (IB-IVUS) as the standard reference.
Methods Plaques were divided into tertiles (T1, T2, and T3) according to the plaque CT attenuation, calculated as the average of five intra-plaque regions of interest, and compared with the plaque characteristics noted on IB-IVUS.
Patients Patients who underwent both CT angiography using a diluted-contrast injection protocol and IB-IVUS were retrospectively analyzed.
Results Thirty-nine plaques in 32 patients were analyzed by CT angiography and IB-IVUS. The median plaque CT attenuation (Hounsfield units) of each tertile was 30 (T1), 48 (T2), and 68 (T3). Although no significant difference was noted in conventional quantitative IVUS parameters (e.g. plaque burden), the T1 with lowest plaque CT attenuation had the highest percentage lipid area by IB-IVUS [75.1% (T1), 57.8% (T2), and 50.8% (T3), respectively, p<0.01]. Furthermore, the plaque CT attenuation had a significant negative correlation with the percentage lipid area (r=-0.59, p<0.01).
Conclusion CT angiography-based plaque characterization using a diluted-contrast injection protocol may aid in the quantitative detection of lipid-rich plaque.
Objective This retrospective study was aimed at determining whether or not stress phase bandwidth (SPBW), a left ventricular (LV) mechanical dyssynchrony index, predicts major cardiac events (MCEs) and stratifies the risk of those in patients with coronary artery disease (CAD) who undergo revascularization.
Methods Patients were followed up to confirm the prognosis for at least one year. The SPBW was calculated by a phase analysis using the Heart Risk View-F software program. The composite endpoint was the onset of MCEs, consisting of cardiac death, non-fatal myocardial infarction, unstable angina pectoris, and severe heart failure requiring hospitalization.
Patients The study subjects were 332 patients with CAD who underwent coronary angiography and revascularization after confirming ≥5% ischemia detected by rest 201Tl and stress 99mTc-tetrofosmin electrocardiogram-gated single-photon emission computed tomography myocardial perfusion imaging.
Results During the follow-up, 35 patients experienced MCEs of cardiac death (n=5), non-fatal myocardial infarction (n=3), unstable angina pectoris (n=11), and severe heart failure requiring hospitalization (n=16). A receiver operating characteristics analysis indicated that the optimal cut-off value of the SPBW was 52° for predicting MCEs, and the MCE rate was significantly higher in the patients with an SPBW >52° than in those with an SPBW ≤52°. Results of the multivariate analysis showed the SPBW and estimated glomerular filtration rate to be independent predictors for MCEs. In addition, the cut-off value of the SPBW significantly stratified the risk of MCEs according to the results of the Kaplan-Meier analysis.
Conclusion Evaluating the SPBW before revascularization may help predict future MCEs in patients with CAD who intended to undergo treatment.
Objective The coronavirus disease 2019 (COVID-19) pandemic has had a significant impact on global healthcare systems. Some studies have reported the negative impact of COVID-19 on ST-elevation myocardial infarction (STEMI) patients; however, the impact in Japan remains unclear. This study investigated the impact of the COVID-19 pandemic on STEMI patients admitted to an academic tertiary-care center in Tokyo, Japan.
Methods In this retrospective, observational, cohort study, we included 398 consecutive patients who were admitted to our institute from January 1, 2018, to March 10, 2021, and compared the incidence of hospitalization, clinical characteristics, time course, management, and outcomes before and after March 11, 2020, the date when the World Health Organization declared COVID-19 a pandemic.
Results There was a 10.7% reduction in hospitalization of STEMI patients during the COVID-19 pandemic compared with that in the previous year (117 vs. 131 cases). During the COVID-19 pandemic, the incidence of late presentation was significantly higher (26.5% vs. 12.1%, p<0.001), and the onset-to-door [241 (IQR: 70-926) vs. 128 (IQR: 66-493) minutes, p=0.028] and door-to-balloon [72 (IQR: 61-128) vs. 60 (IQR: 43-90) min, p<0.001] times were significantly longer than in the previous year. Furthermore, the in-hospital mortality was higher, but the difference was not significant (9.4% vs. 5.0%, p=0.098).
Conclusion The COVID-19 pandemic significantly impacted STEMI patients in Tokyo and resulted in a slight decrease in hospitalization, a significant increase in late presentation and treatment delays, and a slight but nonsignificant increase in mortality. In the COVID-19 era, the acute management system for STEMI in Japan must be reviewed.
Objective Evidence supporting the efficiency of clinically administered therapies against interstitial lung disease (ILD)-related cough is limited. Thus, we conducted a study to evaluate the efficacy of short-term use of chest bands on cough in patients with ILD.
Methods This pre-post intervention study was performed at two university hospitals between April 2017 and August 2020. Scores of the visual analog scale (VAS) for cough severity (in terms of frequency and intensity), Leicester Cough Questionnaire (LCQ)-acute, and frequency scale for symptoms of gastroesophageal reflux disease (FSSG) were assessed before and after the use of the chest band (24/48 hours).
Patients The study included patients with idiopathic interstitial pneumonias (IIPs) or connective tissue disease-associated interstitial lung disease (CTD-ILD).
Results Four patients with IIPs and seven with CTD-ILD were included in the analysis. The cough intensity and LCQ-acute total score improved significantly after the use of the chest band (p=0.007 and p=0.005, respectively), although the cough frequency showed no significant reduction (p=0.074). Furthermore, the FSSG total and acid-reflux symptom scores improved (p=0.018 and p=0.027, respectively), and a negative correlation between the change in LCQ-acute total score and that in FSSG score for acid-reflux symptoms was observed (Spearman rho =-0.841, p=0.001).
Conclusion The results of the current study suggest that chest bands might be useful for treating chronic refractory cough in patients with ILD and gastroesophageal reflux disease. However, these results should be interpreted with caution due to methodological limitations associated with this study.
Objective Viral pneumonia is not rare in community-acquired pneumonia (CAP). Mixed or secondary pneumonia (coinfection) can be seen in viral pneumonia; however, its frequency in coronavirus disease 2019 (COVID-19) has only been investigated in a few studies of short duration, and its significance has not been fully elucidated. We investigated the frequency and significance of co-infection in patients with COVID-19 over a 1-year study period.
Methods Coinfection was investigated via multiplex polymerase chain reaction (PCR), culture of respiratory samples, rapid diagnostic tests, and paired sera. We used logistic regression analysis to analyze the effect of coinfection on severity at admission and Cox proportional-hazards model analysis to analyze the effect of coinfection on need for high-flow nasal cannula, invasive mandatory ventilation use, and death, respectively.
Patients We retrospectively investigated 298 patients who suffered CAP due to severe acute respiratory syndrome coronavirus-2 infection diagnosed by PCR and were admitted to our institution from February 2020 to January 2021.
Results Primary viral pneumonia, and mixed viral and bacterial pneumonia, accounted for 90.3% and 9.7%, respectively, of COVID-19-associated CAP, with viral coinfection found in 30.5% of patients with primary viral pneumonia. Influenza virus was the most common (9.4%). Multivariable analysis showed coinfection not to be an independent factor of severity on admission, need for high-flow nasal cannula or invasive mandatory ventilation, and mortality.
Conclusion Viral coinfection was common in COVID-19-associated CAP. Severity on admission, need for high-flow oxygen therapy or invasive mandatory ventilation, and mortality were not affected by coinfection.
Objective Alström syndrome is an autosomal recessive genetic disease caused by a mutation in the ALMS1 gene. Alström syndrome is clinically characterized by multisystem involvement, including sensorineural deafness, cone-rod dystrophy, nystagmus, obesity, insulin resistance, type 2 diabetes and hypogonadism. The diagnosis is thus challenging for patients without this characteristic set of clinical symptoms. We explored the effectiveness of whole-exome sequencing in the diagnosis of Alström syndrome.
Methods A girl with symptoms of Alström syndrome was tested and diagnosed with the disease by whole-exome sequencing.
Results Whole-exome sequencing revealed two novel variants, c.6160_6161insAT: p.Lys2054Asnfs*21 (exon 8) and c.10823_10824 delAG:p.Glu 3608Alafs*9 (exon16) in the ALMS1 gene, leading to premature termination codons and the domain of ALMS1 protein. Blood sample testing of her asymptomatic parents revealed them to be heterozygous carriers of the same mutations. Assembly showed that the mutations on both alleles were located in conserved sequences. A review of the ALMS1 gene nonsense mutation status was performed.
Conclusion We herein report two novel variants of the ALMS1 gene discovered in a Chinese Alström syndrome patient that expand the mutational spectrum of ALMS1 and provided new insight into the molecular mechanism underlying Alström syndrome. Our findings add to the current knowledge concerning the diagnosis and treatment of Alström syndrome.
Objective The establishment of a department of general internal medicine (GIM) has been shown to improve the clinical outcomes among patients treated in GIM departments but the effect on practice patterns in other departments remains unclear. We evaluated the association between the establishment of a GIM department and the use of blood cultures, an indicator of quality of care of infectious diseases, in other departments.
Methods This study was conducted between 2013 and 2017 in a community hospital which established a new GIM department in 2015, with a mandate to improve the quality of care of the hospital including infectious disease management. The primary outcome was the change in the number of blood culture episodes per calendar month in other departments before and after establishment of the GIM department. The secondary outcome was the change in the blood culture episodes per month, indexed to 1,000 patient-days, during the same time. Using 2015 as the phase-in period, interrupted time series analyses were used to evaluate the change in the outcome variables.
Results In departments other than GIM, there were 284 blood cultures prior to the establishment of the GIM department (2013-2014) and 853 afterwards (2016-2017). The number of blood culture episodes in other departments increased by 10.7 (95%CI: 0.39-21.0, p=0.042) per calendar month after the establishment of the GIM department; blood culture episodes/calendar month/1,000 patient-days increased by 0.55 (95%CI: 0.03-1.07 p=0.037).
Conclusion These results indicate that a GIM department in a community hospital can improve the quality of care in other departments.
Campylobacter jejuni is common cause of enteritis, but biliary infection rarely reported. An 82-year-old woman with pancreatic head cancer underwent endoscopic biliary drainage for malignant biliary obstruction. She was subsequently admitted for management of diarrhea. C. jejuni was identified in stool culture. Her symptoms resolved temporarily without antibiotics but flared up with a fever a few days later. She was diagnosed with acute cholangitis and bacteremia with C. jejuni. Endoscopic biliary drainage and antimicrobial administration improved her symptoms. As complications of C. jejuni diarrhea are rare, antibiotics are not necessarily indicated but sometimes are needed to prevent complications.
A 57-year-old woman with a sudden-onset seizure was hospitalized. Brain magnetic resonance imaging findings led to a suspicion of leptomeningeal carcinomatosis (LMC) without a brain parenchymal tumor, and abdominal computed tomography showed a tumor in the pancreatic tail. Endoscopic ultrasonography-guided fine needle aspiration of the pancreatic mass revealed adenocarcinoma. Therefore, LMC from pancreatic ductal adenocarcinoma was strongly suspected. She received three courses of nab-paclitaxel plus gemcitabine and whole-brain radiation. Shortly thereafter, she developed a severe consciousness impediment and died. A pathological autopsy showed adenocarcinoma in a wide area of the leptomeninges.
An 82-year-old woman with a history of bladder cancer presented with dyspnea and loss of consciousness. Contrast-enhanced computed tomography revealed pulmonary embolism, and emergency thrombus aspiration therapy was performed, but the thrombus was not aspirated. Echocardiography showed mobile masses in the heart and a right-to-left shunt due to a patent foramen ovale (PFO). Magnetic resonance imaging showed multiple cerebral infarctions. Surgical thrombectomy and PFO closure were performed, and the patient was diagnosed with intracardiac metastasis of bladder cancer based on intraoperative histopathology. This is a rare case of concomitant pulmonary and cerebral tumor embolism and intracardiac metastasis from bladder cancer.
A 78-year-old woman was referred to our hospital because of repetitive suppurative arthritis at the artificial left knee joint. Her plasma brain natriuretic peptide level was 122 pg/mL. A 12-lead electrocardiogram showed a QS pattern in the inferior leads. A two-dimensional echocardiogram revealed hypokinesis at the inferior wall and hypertrophy at the apical lateral wall. Color flow imaging revealed this hypertrophic region to be a myocardial sinusoid, demonstrating diastolic coronary to left ventricular flow and early systolic flow vice versa. This was a very rare case of coronary to left ventricular fistula through a sinusoid without cyanotic congenital heart disease or severe coronary artery disease.
A 39-year-old man presented with peripheral eosinophilia, pulmonary eosinophilic infiltrate, and renal failure due to acute tubulointerstitial nephritis (TIN). He had experienced childhood asthma and was negative for anti-neutrophil cytoplasmic antibody (ANCA). He was tentatively diagnosed with ANCA-negative eosinophilic granulomatous polyangiitis (EGPA) or idiopathic hypereosinophilic syndrome (HES). Renal involvement of isolated TIN with eosinophil infiltration is rare in EGPA and HES and does not seem to have a good prognosis in the literature. However, his condition improved well with corticosteroids and mepolizumab. The revised classification of EGPA based on the etiology should dictate the proper treatment in suspected EGPA patients with nonsystemic vasculitis.
Fibrosing mediastinitis (FM) is a rare fibroinflammatory disease of the mediastinum with an etiology and clinical features that vary by world region. The characteristics of FM in Japan are still unknown. We herein report two Japanese patients with FM who were treated with corticosteroids and responded well. We also reviewed the Japanese literature on PubMed® and summarized the characteristics of 27 Japanese FM patients, including our two patients. In Japan, the predominant cases were those without a specific cause, were diffusely distributed, and responded well to corticosteroid therapy.
We report a case of a pulmonary necrotizing sarcoid granulomatosis (NSG)-like lesion possibly associated with coinfection of Mycobacterium avium and Propionibacterium acnes. A solitary nodule in the right middle lobe of the lung was notable for coagulative necrosis with aggregates of sarcoid-like epithelioid granulomas. Small arteries were damaged by granulomas. Both M. avium and P. acnes were detected in the lesion. Furthermore, more P. acnes genomes were detected in the granulomas than in the non-lesion lung. These findings blur the pathophysiologic boundaries among NSG, sarcoidosis, and mycobacteriosis, and suggest that NSG needs to be recognized as continuous spectra of sarcoidosis/mycobcteriosis.
A 58-year-old woman with rheumatoid arthritis (RA) visited our hospital complaining of a persistent cough and sputum for the past year. She had a high cold hemagglutinin titer and chronic sinusitis. Chest computed tomography revealed bilateral diffuse centrilobular nodules, bronchiectasis, and bronchial wall thickening. A surgical lung biopsy was performed that confirmed diffuse panbronchiolitis (DPB) because of the lymphocytic and plasmacytic infiltrates in the respiratory bronchioles. Her condition improved after the administration of clarithromycin. Several cases of RA complicating DPB have previously been reported, but only in Japan. We need to consider DPB as a bronchiolitis types accompanying RA among Japanese patients.
Congenital mutations of the Wilms' tumor 1 (WT1) gene can lead to various abnormalities, including renal/gonadal developmental disorders and cardiac malformations. Although there have been many reports of somatic WT1 mutations in patients with acute myeloid leukemia and myelodysplastic syndrome, congenital WT1 mutations have not been reported in hematological disorders. We herein report a patient with early-onset clonal cytopenia of undetermined significance that was associated with a congenital mutation of WT1 and an acquired mutation of DNMT3A [encoding DNA (cytosine-5)-methyltransferase 3A].
Hodgkin lymphoma (HL) is a hematologic malignancy that typically presents with lymphadenopathy. We herein report a patient with HL who presented with an intramuscular mass that required differentiation from an inflammatory lesion. A 65-year-old Japanese woman was referred to our hospital with a chief complaint of chronic and expanding tumor in her left thigh. By surgical resection, she was diagnosed with primary intramuscular, Epstein-Barr virus-positive, mixed-cellularity classic HL. She received combined modality therapy, resulting in a complete response. Primary intramuscular classic HL is extremely rare. It should be listed as a differential diagnosis of intramuscular tumors.
The primary central nervous system (CNS) presentation of lymphomatoid granulomatosis (LYG) is rare, and no standard therapy for LYG with primary CNS symptoms exists. CNS-LYG patients usually survive for only less than a year from diagnosis. This is the first report of high-grade primary CNS-LYG with monoclonality that was successfully treated with rituximab monotherapy, resulting in a durable remission for more than 1 year in a 66-year-old woman with pemphigus vulgaris who was also on immunosuppressive therapy.
A 56-year-old woman presenting with type II respiratory failure was transferred to our hospital. She did not exhibit muscle weakness or elevated serum myogenic enzymes, but needle electromyography revealed myogenic changes in the limb muscles, and her blood tests were positive for anti-mitochondrial antibodies (AMA). Muscle histopathological findings included immune-mediated necrotizing myopathy, so she was diagnosed with inflammatory myopathy associated with AMA. After treatment with corticosteroids and noninvasive positive pressure ventilation, her symptoms improved. If a diagnosis of type II respiratory failure is difficult, inflammatory myopathy associated with AMA should be considered as a differential diagnosis.
We herein report an 84-year-old woman with right middle cerebral artery (MCA) stenosis who presented with persistent left hemichorea preceding cerebral infarction. She visited our hospital on day 9 after the hemichorea onset. Magnetic resonance imaging (MRI) showed no acute cerebral infarction. Magnetic resonance angiography revealed right MCA stenosis. Her hemichorea persisted for 19 days and subsequently disappeared. On day 21, she developed left hemiplegia. Repeat MRI revealed a cerebral infarction in the right putamen. MCA stenosis can present with persistent hemichorea, even in the absence of cerebral infarction. Persistent hemichorea with MCA stenosis may presage cerebral infarction.
Intravascular large B-cell lymphoma (IVLBCL) is a subtype of B-cell lymphoma, characterized by lymphoma cell proliferation within small blood vessels. We herein describe a rare case with long spinal cord lesions caused by venous congestive myelopathy associated with IVLBCL. An 81-year-old man presented with paraplegia of the lower limbs and sensory disturbances. Magnetic resonance imaging revealed intramedullary longitudinal T2-hyperintensity lesions in the thoracic cords. The patient died three months after disease onset, and a neuropathological analysis revealed predominantly atypical B-lymphocytes located sparsely in the veins of the spinal cord. IVLBCL should be considered in the differential diagnoses of long spinal cord lesions.
The spontaneous regression (SR) of cancer is defined as either partial or complete, and temporary or permanent, disappearance without appropriate treatment for the disease, and this phenomenon is rare in the case of small cell lung carcinoma (SCLC). We herein report an 83-year-old woman who presented with left-sided hemichorea associated with anti-SOX1 (SOX1-Ab) and -CV2/CRMP5 (CV2/CRMP5-Ab) antibodies with SR following a 7-year interval free of disease progression of SCLC. Hemichorea can present with the coexistence of anti-SOX1 and CV2/CRMP5-Ab with SR after a long interval free of SCLC. The immune response associated with these onco-neural antibodies may become independent of the original tumor trigger and remain active for many years.
We herein report an unusual case of granulomatosis with polyangiitis (GPA) in a 65-year-old man in whom relapsed disease manifested as an anterior cheek nodule. Magnetic resonance imaging indicated the differential diagnoses of the subcutaneous nodule in the patient's anterior cheek to be inflammatory granulomatous lesions with GPA, malignancy, or infectious disease. A histopathological examination ruled out malignancy and infectious diseases, and necrotizing vasculitis was suspected. The subcutaneous nodule was successfully treated using rituximab, suggesting that it was associated with GPA, secondary to vasculitis. Clinicians should be aware of the possibility of such a rare manifestation of GPA.
A 73-year-old man previously treated with rituximab for his mucosa-associated lymphoid tissue lymphoma suffered a suboptimal humoral immune response against an acquired SARS-CoV-2 infection. A detailed serological description revealed discrepant antigen-specific humoral immune responses. The titer of spike-targeting, "viral-neutralizing" antibodies remained below the detection level, in contrast to the anti-nucleocapsid, "binding" antibody response, which was comparable in both magnitude and kinetics. Accordingly, viral neutralizability and clearance was delayed, leading to prolonged RNAemia and persistent pneumonia. The present case highlights the need to closely monitor this unique population of recipients of B-cell-targeted therapies for their neutralizing antibody responses against SARS-CoV-2.
Acquired vitamin B12 (VB12) deficiency is a rare cause of thrombotic microangiopathy (TMA). We experienced an 86-year-old Japanese woman who presented with coma, renal dysfunction, and microangiopathic hemolytic anemia. Although we initially considered thrombotic thrombocytopenic purpura, we eventually diagnosed her to have VB12 deficiency due to inappropriate dietary care based on her low serum VB12 level, social history, and negative parietal cell finding and the presence of intrinsic factor antibody. Because similar cases are expected to increase in today's aging society, our experience underscores the importance of including acquired VB12 deficiency in the differential diagnosis of TMA, even in elderly patients without a history of gastrectomy.