Objective The standard treatment for submucosal esophageal cancer is esophagectomy or chemoradiotherapy (CRT). However, these treatment modalities could deteriorate the general condition and quality of life of the patients who are intolerant to invasive therapy. It is therefore important and beneficial to develop less invasive treatment protocols for these patients.
Methods The study included 43 patients who were clinically suspected of mucosa or submucosal esophageal cancer but underwent endoscopic submucosal dissection (ESD) as a primary treatment, due to the patients' poor performance statuses and/or preferences for less invasive therapy. According to the pathological findings and patient's general condition, whether the patient underwent additional treatments or remained hospitalized without additional treatments was thereafter decided for each patient. We retrospectively analyzed the outcomes of these patients.
Results Fifteen patients underwent additional surgery, 11 patients underwent CRT/radiation therapy (RT) and 17 patients were followed without additional treatments. During the 3-year follow-up period, the relapse-free survival rates in the patients who received or did not receive additional treatments were 88% and 64%, respectively (95% confidence interval, 0.45-0.76, p=0.04). The relapse-free and overall survival rates in the patients with additional treatments were equivalent or superior to those described in previous reports of the standard treatments. Preceding ESD contributed to reduce the local relapse significantly to approximately 3.5% and additional CRT-related toxicities.
Conclusion Preceding ESD is very effective for the local control of cancer, and useful for histologically confirming the high-risk factors of relapse, such as ≥submucosal layer 2 (SM2) invasion and lymphovascular involvements. ESD with additional therapy may be a promising strategy for optimizing the selection of therapy depending on the patient's general condition.

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Objective Hepatopulmonary syndrome (HPS) is characterized by vascular dilatation and hyperdynamic circulation, while portopulmonary hypertension (POPH) is characterized by vasoconstriction with fibrous obliteration of the vascular bed. Vasoactive molecules such as nitric oxide (NO) are candidate factors for cirrhotic complications associated with these diseases. However, oxidative stress balance is not well characterized in HPS and POPH. The present objective is to investigate the oxidative stress and anti-oxidative stress balance and NO pathway balance in patients with potential HPS and POPH.
Methods We recruited patients with decompensated cirrhosis (n=69) admitted to our hospital as liver transplantation candidates. Patients exhibiting partial pressure of oxygen lower than 80 mmHg and alveolar-arterial oxygen gradient (AaDO₂) ≥15 mmHg were categorized as potentially having HPS (23 of 69 patients). Patients exhibiting a tricuspid regurgitation pressure gradient ≥25 mmHg were categorized as potentially having POPH (29 of 61 patients). Serum reactive oxygen metabolites were measured and anti-oxidative OXY-adsorbent test (OXY) were performed, and the balance of these tests was defined as the oxidative index. The correlation between these values and the clinical characteristics of the patients were assessed in a cross-sectional study.
Results Potential HPS patients exhibited no correlation with oxidative stress markers. Potential POPH patients exhibited lower OXY (p=0.037) and higher oxidative index values (p=0.001). Additionally, the vascular NO synthase enzyme inhibiting protein, asymmetric dimethylarginine, was higher in potential POPH patients (p=0.049). The potential POPH patients exhibited elevated AaDO₂, suggesting the presence of pulmonary shunting.
Conclusion Potential POPH patients exhibited elevated oxidative stress with decreased anti-oxidative function accompanied by inhibited NO production. Anti-oxidants represent a candidate treatment for potential POPH patients.

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Objective Uraemic pruritus is a distressing symptom that has a negative impact on the quality of life for dialysis patients. The pathophysiology of pruritus in peritoneal dialysis (PD) patients is still poorly understood. The present study aims to investigate the prevalence and related risk factors of pruritus in continuous ambulatory peritoneal dialysis (CAPD) patients.
Methods In total, 362 CAPD cases were investigated from January 2012 to April 2013. Pruritus was assessed by visual analogue scale.
Results The prevalence of severe pruritus and mild to moderate pruritus was 12.7% and 52.5%, respectively. The patients with severe pruritus had the longest duration of PD (p<0.001), Pittsburgh Sleep Quality Index (PSQI) score (p<0.001), Beck Depression Inventory (BDI) score (p=0.003), intact parathyroid hormone (iPTH) level (p=0.009), and the lowest Medical Outcomes Short Form 36 Health Survey, Physical Component Score (SF-36 PCS) (p<0.001) among the three groups. The patients with mild to moderate pruritus had a significantly higher iPTH level (p=0.004) compared with the patients without pruritus. A multivariate logistic regression for pruritus showed that higher PSQI score [odds ratio (OR)=1.305, p=0.001], higher BDI score (OR=1.429, p=0.002), longer vintage (OR=1.039, p=0.004), and higher iPTH level (OR=1.317, p=0.014) were independently associated with pruritus.
Conclusion The prevalence of uraemic pruritus was 65.2% in CAPD patients. Sleep disorder, depression, longer vintage, and a higher iPTH level were independent associated factors for pruritus in CAPD patients.

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Objective The goals of this study were to assess the efficacy and tolerability of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF) and to identify predictors of tolerability to pirfenidone.
Methods We conducted a retrospective observational study. When the patient showed deterioration in the percent forced vital capacity (%FVC) or experienced acute exacerbations or severe adverse events, treatment of the patient with pirfenidone was discontinued. We classified the patients who did not display progression following six months of pirfenidone treatment as the tolerant group and the patients who did display progression as the intolerant group. We retrospectively analyzed differences between the two groups in terms of baseline characteristics. The efficacy of pirfenidone was evaluated by the changes in vital capacity (VC) and %FVC before and after the start of treatment in the tolerant group.
Patients A total of 20 patients who had been diagnosed with IPF were treated with pirfenidone.
Results In the tolerant group, the baseline %FVC (p=0.01) and the percentage diffusing capacity of the lungs for carbon monoxide (D_LCO, p=0.02) were significantly higher, and the baseline composite physiologic index (CPI) was significantly lower (p=0.009) than in the intolerant group. In the tolerant group, pirfenidone significantly reduced the decline in VC and %FVC of the patients after treatment. In the intolerant group, five patients discontinued pirfenidone treatment because of anorexia.
Conclusion We found that pirfenidone was better tolerated in patients with milder disease symptoms, as indicated by their baseline CPI, %FVC and %D_LCO, and that patients in the tolerant group could benefit from the use of pirfenidone.

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Objective To compare the utility of Gram staining, a urinary antigen detection kit and a sputum antigen detection kit were examined for the rapid and early detection of pneumococcal pneumonia and lower respiratory infectious diseases.
Methods A newly developed sputum pneumococcal antigen detection kit (RAPIRUN), Gram staining, and urinary antigen detection kit (BinaxNOW) were comparatively evaluated for their ability to detect Streptococcus pneumoniae in patients with pneumonia or lower respiratory tract infection. Sputum culture results were used as a standard for comparison. Furthermore, the pneumococcus-positive rates in culture and rapid tests were compared using polymerase chain reaction (PCR) as a reference.
Results Of the 169 patients studied, 54 (32.0%) tested positive for S. pneumoniae in culture. S. pneumoniae detection sensitivities for Gram staining, RAPIRUN, and BinaxNOW were 75.9%, 90.7%, and 53.7%, respectively; thus, RAPIRUN had a significantly higher sensitivity than BinaxNOW (p<0.001). For patients with ≥10⁵ copies/μg of pneumococcal surface protein A DNA PCR analysis, the detection rates of culture, Gram staining, and RAPIRUN were 85.2%, 72.1%, and 82.0%, respectively, however, the detection rate of BinaxNOW was only 47.5%. Comparisons among 45 patients with culture-positive pneumococcal pneumonia revealed that RAPIRUN had a significantly higher detection rate than BinaxNOW in the mild cases (p<0.006), regardless of the number of days from onset (p<0.03).
Conclusion RAPIRUN is a rapid testing kit that detects S. pneumoniae in sputum with a high sensitivity and specificity. It is a particularly more useful detection kit than BinaxNOW for early and mild community-acquired pneumonia in pre-treatment patients whose sputum specimens can be obtained.

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A 47-year-old woman with a 2-year history of rheumatoid arthritis (RA) undergoing methotrexate treatment developed a perforated ulcer in the ileum for which she underwent emergency surgery. A histological analysis of the extirpated specimen presented a possible Epstein-Barr virus (EBV) infection in the ulcerative lesion without a feature of lymphoproliferative disorder. Interestingly, the patient's serological tests with a paired serum diagnosed a primary EBV infection. The present case emphasizes the importance of being aware of severe enteritis as a possibility for patients with RA, for an accurate diagnosis.

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A 58-year-old Japanese man was diagnosed with differentiated adenocarcinoma of the stomach. Histological findings of the resected specimen revealed well- to moderately-differentiated tubular adenocarcinoma (tub1, tub2), 13 mm in diameter, which invaded into the submucosa (SM1, 300 μm) and lymphovascular lumen (ly1). Serum antibody against Helicobacter pylori (Hp) and the ¹³C-urea breath test were negative, and there were no atrophic changes in the tumor-adjacent mucosa. The immunohistochemical analysis showed that gastric mucin (MUC5AC) was strongly positive and intestinal mucin (MUC2) was weakly and partially positive. According to these results, the final diagnosis of Hp-negative well-differentiated early gastric cancer was made.

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A 64-year-old man presented with epigastralgia and nausea after an acute exacerbation of chronic pancreatitis. Abdominal computed tomography revealed remarkable thickening of the gastric wall and intramural hypodense areas. Esophagogastroduodenoscopy showed a large gastric ulcer surrounded by an edematous mucosa and mucopus. The results of a culture from a biopsy of the lesion indicated phlegmonous gastritis. The patient was successfully treated with an antibiotic without gastrectomy.

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Nail dystrophy, oral leukoplakia and abnormal skin pigmentation are the defining features of dyskeratosis congenita. Dyskeratosis congenita is a disorder of poor telomere maintenance and is known to increase the risk of developing multiple types of malignancy. However, there are few reports of liver tumors arising in dyskeratosis congenita patients. We herein report the second case of hepatic angiosarcoma arising from dyskeratosis congenita: a 23-year-old man was introduced to our hospital due to the detection of multiple tumors in the liver. A histological analysis showed angiosarcoma that stained positive for antibodies to both CD31 and blood coagulation factor VIII.

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Coils are typically utilized as one of the most popular embolization agents. Coil migration to the pulmonary artery rarely occurs and is associated with a high tendency of severe pulmonary or cardiovascular complications. We herein present a 25-year-old man with arteriovenous malformations in the right ear. Two coils 4-mm in diameter and 3-mm in length migrated to the pulmonary artery during embolization. No further damage related to the coil migration was found during a 2-year follow-up period. In addition, the possible mechanisms of coil migration are discussed.

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Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare and fatal cancer-related complication. We herein present a case of PTTM that diagnosed antemortem by lung scintigraphy and pulmonary microvascular cytology. The patient was treated with steroid pulse therapy. Although her symptoms temporarily improved, she died of respiratory failure. An autopsy showed PTTM, and an immunohistochemical analysis revealed the expression of osteopontin and CD44 in macrophages that had migrated into the PTTM lesions. These findings suggest that inflammation associated with the interaction between osteopontin and CD44 may play an important role in PTTM.

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Euthyroid Graves' disease (EGD) is a rare condition marked by the onset of thyroid-associated ophthalmopathy (TAO) in the absence of thyroid dysfunction. The pathogenesis of EGD remains unclear, and a consistent view of the pathological findings of the thyroid tissue has yet to be determined. We herein report a case of EGD in a 34-year-old woman with papillary carcinoma treated with total thyroidectomy. The entire thyroid specimen was investigated, and the thyroid tissue appeared normal. In this report, we discuss the relationship between the pathogenesis of TAO and thyroid dysfunction.

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A 73-year-old man with fever, renal insufficiency, and purpura was referred to our hospital to be evaluated for renal insufficiency. Renal biopsy revealed acute and chronic tubulointerstitial nephritis with no laboratory findings of sarcoidosis or connective tissue disease. Low C4 levels and elevation of rheumatoid factors suggested cryoglobulinemia, which was confirmed with quantitative analysis. CD34 staining of kidney tissue revealed peritubular capillaritis. Antineutrophil cytoplasmic antibodies were negative. The etiology of peritubular capillaritis was not clear in our patient; however, it might be associated with cryoglobulinemia because we cannot find any other diseases that could have induced the peritubular capillaritis.

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We herein report the case of an elderly patient with light-chain deposition disease (LCDD) successfully treated with bortezomib. An 83-year-old woman was admitted because of nephrotic syndrome. She was diagnosed to have monoclonal gammopathy of undetermined significance (IgG-κ type) and LCDD, on the basis of serum and urinary immunoelectrophoresis and renal biopsy. She responded to a modified regimen of bortezomib-based chemotherapy with disappearance of proteinuria without any adverse effects. According to a literature review of 16 cases, including the present case, bortezomib-based chemotherapy appears to be a convincing strategy for the treatment of LCDD even in elderly patients.

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A 31-year-old woman with no significant past medical or family history was admitted with complaints of general weakness. Laboratory tests revealed: serum potassium 3.0 mEq/L, arterial blood pH 7.28, serum bicarbonate 17.8 mEq/L and urinary pH 7.0. Double-labeling confocal fluorescence microscopy using H⁺-ATPase and pendrin antibodies demonstrated a decreased expression of these proteins in the patient's renal collecting duct compared to normal controls. Anti-Sjögren's-syndrome-related antigen A (Anti-Ro/SS-A) and anti-Sjögren's syndrome type B (anti-La/SS-B) antibodies were strongly positive with very high titers, consistent with Sjögren's syndrome. We present a case of distal renal tubular acidosis-associated Sjögren's syndrome with a defect in H⁺-ATPase and pendrin in the renal collecting duct.

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A 55-year-old woman was diagnosed with a tyrosine kinase inhibitor of anaplastic lymphoma kinase (ALK) rearrangement-positive lung adenocarcinoma and treated with chemotherapy consisting of crizotinib, a tyrosine kinase inhibitor of ALK, as second-line chemotherapy. However, the size of the metastatic adrenal lesion increased, and the patient died due to multiple organ failure. An autopsy report revealed that the metastatic lesion of the adrenal tumor was ALK rearrangement-positive pleomorphic carcinoma. The epithelial-mesenchymal transition (EMT) marker vimentin was immunohistochemically positive in both the lung and adrenal lesions. The present case report suggests the possibility of transformation into pleomorphic carcinoma as a result of EMT in patients with ALK rearrangement-positive lung cancer.

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It has recently been shown that nanoparticle albumin-bound paclitaxel (nab-PAC)+carboplatin (CBDCA) provides a favorable overall response rate in non-small cell lung cancer. This is the first case report of nab-PAC+CBDCA therapy in small cell lung cancer (SCLC). Our patient was a 72-year-old man with stage IV SCLC combined with squamous cell carcinoma and interstitial lung disease (ILD). We administered nab-PAC+CBDCA as a second-line chemotherapy. A partial response was evident after two cycles of chemotherapy, and no serious side effects occurred. The progression-free survival was 15 weeks. Second-line chemotherapy using nab-PAC+CBDCA was effective and well tolerated in an SCLC patient with ILD.

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Deciduoid mesothelioma is a rare variant of epithelioid mesothelioma. We experienced the case of a 73-year-old man with asbestos exposure who was diagnosed with malignant pleural mesothelioma with deciduoid features. He received chemotherapy containing six cycles of cisplatin and pemetrexed and survived for twenty-five months after the diagnosis. At autopsy, the final diagnosis was biphasic pleural mesothelioma. Cells with deciduoid features had mostly disappeared, and spindle cells markedly proliferated. To the best of our knowledge, this is the first autopsy case of malignant pleural mesothelioma with deciduoid features that exhibited a response to chemotherapy.

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Adult T-cell leukemia/lymphoma (ATL) sometimes causes opportunistic infections. A 53-year-old woman with systemic lymphadenopathies was diagnosed with ATL by inguinal lymph node biopsies and underwent oral chemotherapy. Two months later, high grade fever, lower abdominal pain and lymphadenopathy recurred. Computed tomography revealed the presence of lymphadenopathy with marked gas formation in the pelvic lesion. Blood cultures were suggestive of septic lymphadenitis by Bacteroides fragilis (BF). This represents the first demonstration of giant lymphadenitis with gas formation caused by BF in a patient with ATL. Notably, septic lymphadenitis is pivotal in the differential diagnosis of systemic lymphadenopathy in ATL.

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Catastrophic antiphospholipid syndrome (CAPS) survivors rarely relapse. We herein report a case of a second CAPS episode with an unusual subacute course and no microangiopathic hemolytic anemia (MAHA), a common CAPS symptom. During the first episode, the 69-year-old woman responded well to high-dose glucocorticoids and plasma exchange. On relapse, these treatments plus rituximab were ineffective and she died of multi-organ failure and bacterial cholangitis. The absence of MAHA and a subacute course do not exclude a CAPS recurrence.

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A 63-year-old Japanese woman with a 30-year history of systemic lupus erythematosus developed macrohematuria and massive proteinuria after seroconversion of myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA). A renal biopsy indicated focal proliferative lupus nephritis (class III A/C) with a fibrous crescent formation. Methylprednisolone pulse therapy (500 mg, 3 successive days) was administered because of progressive proteinuria. Steroid therapy did not suppress the progressive proteinuria; therefore, tacrolimus was added as an alternative immunosuppressive therapy, resulting in the improvement of proteinuria and renal impairment. This case report suggests that MPO-ANCA might play a pathogenic role in the exacerbation of immune-complex-type lupus nephritis.

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Among patients with bacterial meningitis, a cerebral vasospasm typically occurs during the acute phase. We experienced a case of delayed cerebral vasospasm with infarction that was secondary to Listeria monocytogenes meningitis. An 82-year-old woman with Listeria monocytogenes meningitis, whose symptoms had been improving after the initiation of antibacterial therapy, fell into a coma on day 15 and developed generalized seizure. Magnetic resonance imaging (MRI) and MR angiography (MRA) indicated a cerebral vasospasm with multiple infarctions. The risk of vascular complications following acute bacterial meningitis requires close follow-up to identify neurological changes and a low threshold for vascular evaluation. In such cases, MRI and MRA have diagnostic utility.

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We herein report a case of Klebsiella oxytoca-producing IMP-1 that was detected as a first isolate of carbapenem-resistant Enterobacteriaceae (CRE) at our facility. Since K. oxytoca is an uncommon strain for CRE, we speculated that the resistant organism had already spread out inside the hospital. Metallo-β-lactamases promotes antibiotic resistance in Enterobacteriaceae, which potentially yields problematic issues in clinical settings. Active surveillance of antibiotic resistant strains is important and should be repeatedly highlighted. Furthermore, appropriate methods should be established to detect highly resistant strains.

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Leptospirosis is recognized as a zoonotic disease that is emerging worldwide. Severe manifestations are associated with high morbidity and mortality rates and may therefore pose an important risk to public health, especially in certain high prevalence areas like Taiwan. The severe pulmonary form of leptospirosis is a lesser known entity and is characterized by intra-alveolar hemorrhage and can lead to acute respiratory failure with resistant hypoxemia, which leads to high mortality rates despite maximally invasive mechanical ventilation and adequate treatment. We herein present a case of severe leptospirosis complicated by massive pulmonary hemorrhage, which was successfully managed by extra corporeal membrane oxygenation.

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