This review summarizes the current methods for the functional assessment of vascular damage (e.g., assessment of endothelial function, measurement of pulse wave velocity, and pressure wave analysis) and describes the association between vascular dysfunction and chronic cardio-renal syndrome. Vascular dysfunction may contribute to the development and progression of heart failure. Additionally, vascular dysfunction, especially increased arterial stiffness and abnormal pressure wave reflection and central hemodynamics, has been reported to accelerate renal function decline. Furthermore, renal dysfunction worsens vascular pathophysiological abnormalities. Therefore, the functional assessment of vascular damage may be useful in the management of cardio-renal syndrome.
Objective The impact of corticosteroids on acute respiratory distress syndrome (ARDS) mortality remains controversial following the publication of numerous trials, observational studies and meta-analyses. An updated meta-analysis is warranted, as a few original studies on this topic have been published since the last meta-analysis. Methods We searched for eligible articles using four databases. In particular, we included full-length original articles providing sufficient data for evaluating the impact of corticosteroid treatment on adult ARDS mortality in the form of odds ratios. A fixed model with the confidence interval method was used. An assessment of publication bias and sensitivity analyses were also conducted. Results We included 11 of 185 articles. The pooled odds ratio for corticosteroids with respect to all-cause mortality involving 949 patients was 0.77 [95% confidence interval (CI): 0.58-1.03, p=0.079] with strong heterogeneity (I2=70%, p<0.001). The results of the sensitivity analysis, Begg-Kendall test (τ=0.53, p=0.024) and funnel plot consistently suggested the existence of strong publication bias. After six potentially unpublished cohorts were filled using Duval's trim and fill method, the pooled odds ratio shifted to 1.11 (95% CI 0.86-1.44, p=0.427). In addition, the sensitivity analyses suggested that corticosteroid treatment has a different impact on mortality depending on the comorbidities and trigger events. Conclusion We were unable to confirm, based on the data of published studies, the favorable impact of corticosteroid therapy on mortality in overall ARDS cases. Published articles exhibit strong publication bias, and previous meta-analyses may be affected by this publication bias. Further research focusing on pathophysiology- or trigger event-specific ARDS is anticipated.
Objective Rapid eye movement (REM)-related sleep disordered breathing (SDB) is an entity in which the cessation or reduction of breathing occurs primarily during the REM period. Most studies have shown that REM-related SDB more frequently affects women, younger people and patients with mild or moderate SDB. The aim of this study was to prospectively investigate the prevalence and features of REM-related SDB in Japanese subjects compared with the findings of previous reports. Methods A total of 468 patients were evaluated in this study. The diagnosis of SDB was established using polysomnographic monitoring. The patient variables included age, gender, body characteristics, comorbidities, etc. Results REM-related SDB was more prevalent in women than non-REM-related SDB (male ratio; 66.3% vs. 79.5%, p=0.03). Moreover, the patients with REM-related SDB had lower body mass indexes (25.9±6.9 vs. 28.5±7.7; p=0.003), arousal indexes (31.8±10.7 vs. 61.0±29.1; p<0.001), apnea hypopnea indexes (15.0±8.0 vs. 54.9±35.9) and glycosylated hemoglobin (HbA1c) levels (5.5±0.9 vs. 5.9±2.6; p=0.02) than the patients with non-REM-related SDB. However, the overall and female gender prevalence of REM-related SDB among the Japanese subjects was lower than that shown in previous reports. The finding that REM-related SDB was not prevalent in younger individuals or severely obese patients was not consistent with the results of previous studies. Conclusion The present findings suggest that REM-related SDB may have different clinical characteristics in the Japanese population than that observed in previous reports.
Objective Despite the remarkable advances in chemotherapy and allogeneic hematopoietic stem cell transplantation (HSCT), adult T-cell leukemia-lymphoma (ATL) is still associated with a high mortality rate. It is therefore essential to elucidate the current features of ATL. Methods We retrospectively analyzed 81 patients with aggressive type ATL at our institution over a 7-year period based on Shimoyama's diagnostic criteria. Results Eighty-one patients with a median age of 67.5 years were classified as having acute (n=47), lymphoma (n=32), or chronic type (n=2) ATL. They were initially treated by either palliative therapy (n=25) or systemic chemotherapy [n=56; cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) therapy (n=25)/vincristine, cyclophosphamide, doxorubicin, and prednisone (VCAP)-doxorubicin, ranimustine, and prednisone (AMP)-vindesine, etoposide, carboplatin, and prednisone (VECP) therapy (VCAP-AMP-VECP) or CHOP-VMMV therapy (n=31)], and showed median survival durations of 16 and 277 days, respectively. Subsequent to the initial treatment, HSCT (n=6) was performed for certain patients, thus revealing that two-thirds (n=4) relapsed, and one-third (n=2) survived for 131 days and 203 days, respectively. The relapsed ATL patients were treated with conventional salvage therapy (n=29) or anti-CC chemokine receptor 4 antibody (mogamulizumab) (n=3). The patients treated with mogamulizumab demonstrated complete response (2) and partical response (1) with short duration periods of 82 days, 83 days, and 192 days, respectively. Among the five long-term survivors (>5 years) who received chemotherapy, most showed a low and intermediate risk according to the ATL prognostic index. Conclusion In our study, the overall survival of ATL remains poor due to the advanced age of the patients at diagnosis, a high proportion of patients receiving palliative therapy, and a small proportion of long-term survivors receiving chemotherapy and undergoing HSCT. This study illustrates the current clinical features, treatment strategies, and outcomes in clinical practice.
Objective This study was performed to investigate the factors influencing the correlation between physician satisfaction and patient satisfaction in an outpatient setting. Methods New patients attending the General Medicine Outpatient Clinic of Chiba University Hospital and their physicians were enrolled. After the initial consultation, both the patients and the physicians completed an anonymous questionnaire. Results There were 875 patients (381 men and 494 women; mean age: 54 years) and 10 physicians (4 men and 6 women; mean experience: 6 years). The satisfaction of the patients and the physicians was not correlated (r=0.14, p<0.001). A logistic regression analysis revealed that the factors associated with greater physician satisfaction were "guidance/advice from senior colleagues" [odds ratio (OR)=2.03; 95% confidence interval (CI)=1.76-2.34] and "confidence in the diagnosis" (OR=1.52; 95%CI=1.37-1.69), while "a difficult patient" (OR=0.73; 95%CI=0.68-0.78) was associated with reduced satisfaction. The factors associated with greater patient satisfaction were "the doctor listened carefully" (OR=1.98; 95%CI=1.62-2.42) and "my diagnosis is correct" (OR=1.57; 95%CI=1.41-1.74). One item in the questionnaire for the physicians, "I diagnosed psychogenic illness" (OR=0.87; 95%CI=0.81-0.94), was associated with lower patient satisfaction. Conclusion The satisfaction of the patients and the physicians was not correlated. If only the factors promoting the satisfaction of one party are targeted, the satisfaction of the other party will not increase. The satisfaction of the physicians may be increased by receiving advice from mentors and an improved diagnostic ability, while the patients wants a physician who listens carefully and makes the correct diagnosis.
Epstein-Barr virus (EBV)-associated gastric carcinoma accounts for nearly 10% of all gastric carcinomas and has distinct demographic, clinical and pathological features compared with EBV-negative gastric carcinoma. We herein report the case of a patient with EBV-associated gastric carcinoma followed up for 12 years during the natural course of the disease. The appearance of the tumor on gastroscopy and computed tomography gradually changed, and the size of the lesion increased very slowly during the 12 years, without metastasis. The present case indicates that some EBV-associated gastric carcinomas progress very slowly.
Hereditary spherocytosis is the most common form of hemolytic anemia and is characterized by spherical, osmotically fragile erythrocytes that are selectively trapped by the spleen. Hereditary spherocytosis is typically diagnosed in childhood. We herein experienced a rare case of hereditary spherocytosis diagnosed in middle age. The patient presented with cholelithiasis and hyperbilirubinemia. He had no anemia and was asymptomatic with mild splenomegaly. In the differential diagnosis of these symptoms, the possibility of hereditary spherocytosis should be considered, even in patients who are middle-aged and lack anemia.
A 66-year-old man was admitted to undergo treatment for the sudden onset of hypogastric pain radiating to the left flank. Based on the clinical presentation and radiological findings, left adrenal hemorrhage secondary to hepatocellular carcinoma was diagnosed. Although the patient was hemodynamically stable, anemia progressed over the first four days after admission and a blood transfusion was subsequently initiated, which was effective. According to the results of interval imaging with computed tomography, elective transcatheter embolization (TAE) was performed nine days after admission to treat the bleeding from the adrenal tumor. After TAE, the patient's abdominal pain resolved, with no further progression of anemia.
A 58-year-old Chinese man presented with intermittent seizure episodes after being misdiagnosed with epilepsy for eight years. MRI revealed an abnormally strong signal in the distal pancreas. The patient was subsequently diagnosed with pancreatic insulinoma based on the histological findings, and his symptoms improved following surgical removal of the tumor. The appearance of unusual manifestations of insulinoma makes it difficult to diagnose the condition. This disorder should be included in the differential diagnosis of epilepsy and mental illness.
A 15-year-old boy was referred to our department due to gout. The laboratory findings showed hyperuricemia with a decreased erythrocyte hypoxanthine phosphoribosyl transferase (HPRT) activity. The HPRT cDNA sequence was revealed to be 206A>T, which has not been previously reported. In addition, direct sequencing of genomic DNA showed the patient to possess four variants reported to be associated with hyperuricemia. This is the first case report of partial HPRT deficiency due to a novel HPRT mutation accompanied by variants associated with hyperuricemia. Combination treatment consisting of benzbromarone and febuxostat had a significant effect in reducing the urate level in our patient.
A 68-year-old man was admitted with hemoptysis. Bronchoscopy showed bronchial obstruction at the right B3bii proximal to the broncholith. The distal end of the bronchoscope was placed in a wedge position at the right B3bii. After repeated injection of saline solution through the working channel of the bronchoscope, we observed that the broncholith was freed from the bronchial wall. The broncholith was easily removed with forceps through the working channel of the bronchoscope. Bronchoscopic saline solution injection was safe and effective in confirming the mobility of the broncholith as bronchoscopy alone could not detect the broncholiths due to bronchial occlusion.
Extragonadal yolk sac tumors (YSTs) are rare. We herein report the case of a 66-year-old man with mediastinal, lung and liver tumors. The largest mass was located in the liver and contained a high concentration of protein induced by vitamin K absence or antagonist-II (PIVKA-II) and alpha-fetoprotein. Therefore, the lesion was difficult to distinguish from hepatocellular carcinoma. Finally, YST was diagnosed based on the results of a liver biopsy. Although chemotherapy was effective, the patient died of respiratory failure. The autopsy revealed primary mediastinal YST. In the current report, we describe this case of PIVKA-II-producing YST and review previous cases of PIVKA-II-producing tumors other than hepatoma.
A 30-year-old woman was diagnosed with severe infectious mononucleosis (IM). The Epstein-Barr virus (EBV) had infected both CD19- and CD8-positive cells, and clonal proliferation of EBV-infected cells and T-cells was detected. Although we suspected malignant lymphoma, her condition improved following immunosuppressive therapy. A similar case was recently reported; therefore, this case is the second case of IM with EBV-infected CD8-positive cells and clonal proliferation of EBV-infected cells. Our results demonstrate that the clonal proliferation of EBV-infected cells is not always an indication for chemotherapy in the primary infection phase and that monitoring the EBV viral load is useful for therapeutic decision-making.
Guillain-Barré syndrome (GBS) is usually triggered by viral or bacterial infection. In addition, it was recently reported that infection with hepatitis E virus (HEV) also causes GBS. A 49-year-old man presented with acute-onset paralysis in all extremities and dysgeusia during an episode of acute hepatitis. Serological tests showed the presence of anti-HEV IgM antibodies and HEV-RNA in the serum. As an electrophysiological examination showed acute demyelinating polyradiculoneuropathy, the patient was diagnosed as HEV-associated GBS. Following the initiation of treatment with intravenous immunoglobulin, his paralysis and dysgeusia rapidly improved. This case suggests that HEV-associated GBS may rarely be complicated by dysgeusia.
A 71-year-old immunocompetent man developed cognitive decline and gait disturbance. Brain magnetic resonance imaging (MRI) revealed bilateral diffuse leukoencephalopathy without a mass lesion. An analysis of the cerebrospinal fluid (CSF) showed elevated levels of interleukin (IL)-10. The condition of the patient progressively deteriorated, and intravenous high-dose steroids proved ineffective. Detection of non-destructive, diffusely infiltrating, large B-cell lymphoma in biopsy and autopsy specimens led to a diagnosis of lymphomatosis cerebri (LC). On serial MRI, the basal ganglia and white matter lesions increased in parallel with the levels of IL-10. These findings suggest that the IL-10 level in the CSF may represent a potentially useful biomarker for the early diagnosis and monitoring of the disease progression in LC.
A 36-year-old man with a 16-year history of refractory Behçet's disease (BD)-associated uveitis and chronic renal failure requiring hemodialysis suffered from frequent ocular attacks despite treatment with systemic corticosteroids and cyclosporine A. Following infliximab administration, the patient's BD ocular attack score 24 and visual acuity improved. Although he developed mild acute gastroenteritis, he did not experience any other adverse events. In our review of the literature, we identified seven patients on hemodialysis with inflammatory disease successfully treated with infliximab. Infliximab may be effective and safe in cases of BD and other diseases, including in patients under hemodialysis.
A 76-year-old previously healthy Japanese man experienced severe diarrhea (8,000 mL per day) after undergoing laparotomy for small bowel obstruction and was diagnosed with Clostridium difficile infection. Although he developed a maculopapular rash secondary to the systemic absorption of enteral vancomycin (VCM), the patient was successfully treated with the continuous administration of VCM through a long intestinal tube placed in the terminal ileum. This method ensured the reliable delivery of VCM to the colon, while the continuous administration maintained high fecal levels of the drug. This treatment approach is an effective minimally invasive option for patients with severe diarrhea.
A 79-year-old man with diabetes and partial gastrectomy visited our hospital due to gradually worsening epigastric pain on exertion. Unstable angina was suspected and coronary angiography was performed, which revealed severe stenosis of the left ascending artery. Despite successful intervention, the pain persisted. A careful physical examination finally revealed the point of tenderness on the xiphoid process, and the patient was diagnosed with xiphodynia. The severe epigastric pain resolved immediately after xiphoidectomy. This case demonstrates that symptoms of xiphodynia may mimic those of various types of disorders, such as angina, and that careful palpation is warranted during routine physical examinations.