Objective Uninterrupted oral warfarin strategy has become the standard protocol to prevent complications during catheter ablation (CA) for the treatment of atrial fibrillation (AF). However, little is known about the safety and efficacy of uninterrupted dabigatran therapy in patients undergoing CA for AF. Therefore, this study investigated the safety and efficacy of uninterrupted dabigatran therapy and compared the findings with those for uninterrupted warfarin therapy.
Methods Bleeding and thromboembolic events during the periprocedural period were evaluated in 363 consecutive patients who underwent CA for AF at Nagoya University Hospital, and received uninterrupted dabigatran (n=173) or uninterrupted warfarin (n=190) for periprocedural anticoagulation.
Results A total of 27 (7%) patients experienced either bleeding or thromboembolic complications. Major bleeding complications occurred in 2 (1%) patients in the dabigatran group (DG) and 2 (1%) patients in the warfarin group (WG). Eight (5%) patients in the DG and 9 (5%) patients in the WG experienced groin hematoma, a type of minor bleeding complication. Meanwhile, no patient in the DG and 1 (1%) in the WG developed cerebral ischemic stroke. Overall, there was no significant difference between the groups for any category. The activated partial thromboplastin time (APTT) independently predicted periprocedural complications in the DG.
Conclusion Uninterrupted dabigatran therapy in CA for AF thus may be a safe and effective anticoagulant therapy, and appears to be closely similar to continuous warfarin; however, it is essential to pay close attention to the APTT values when using dabigatran during CA.

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Objective The dominant frequency (DF) in frequency analyses is considered to represent the objective cycle length and complexity of activation under conditions of ventricular fibrillation (VF). However, knowledge regarding the mechanisms determining the DF in human VF is limited. We studied the characteristics of the DF of human VF and relationship between DF and the defibrillation threshold.
Methods Seventy-two implantable cardioverter-defibrillator patients and 211 VF were studied. Using defibrillation tests, we performed a frequency analysis with fast Fourier transformation. The correlations between DF and clinical characteristics, including the defibrillation threshold, were assessed.
Results The mean DF of all induced VFs was 5.2±0.8 Hz. The patients were divided into two groups according to DF: the low-DF (DF <5.2 Hz, n=32) and high-DF (DF ≥5.2 Hz, n=40) groups. The frequency of structural heart disease was significantly higher in the low-DF group. In addition, the QRS duration, QT interval and effective refractory period of the right ventricle (RV-ERP) were significantly longer in the low-DF group. A multivariate analysis showed RV-ERP to be the only independent predictor of DF. Excluding patients receiving group III anti-arrhythmic drugs, which are known to have potent defibrillation threshold effects, the defibrillation threshold was significantly lower in the low-DF group (p=0.026).
Conclusion We found that the DF of human VF is associated with underlying heart disease, the cardiac function, cardiac conduction, ventricular refractoriness and defibrillation threshold. Our findings may be useful for identifying and managing patients with a high defibrillation threshold.

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Objective There is a discrepancy in the intensity of breath sounds in chronic obstructive pulmonary disease (COPD) patients between subjective studies, which have reported a diminished intensity, and objective studies using airflow-standardized measurements, which have not demonstrated a diminished intensity. We herein evaluated the breath sound intensity in COPD patients during tidal breathing in order to obtain clinically relevant results.
Methods The subjects included 20 stable COPD patients and 20 normal controls. Microphones were attached to six sites on the chest wall, and breath sounds at the chest wall and airflow in the mouth were measured during resting tidal and deep tidal breathing. The octave-band power values of the breath sounds were subsequently calculated.
Results 1. During resting breathing, the intensity of breath sounds during both inspiration and expiration was significantly greater in the COPD group than in the control group; the difference was prominent at higher frequency bands (>400 Hz). In addition, the power of the high frequency bands tended to be positively correlated with the CT visual emphysema scores but not the forced expiratory volume in one second, The airflow during resting breathing did not differ between the two groups. 2. During deep breathing, the intensity of inspiratory breath sounds at the dominant frequency band (200-400 Hz) was diminished over the upper and middle lung fields in the COPD group compared to that observed in the control group, while the intensity during expiration was not. The airflow during deep breathing was lower in the COPD group than in the control group.
Conclusion In the present study, the breath sound intensity in the COPD patients was diminished during deep inspiration due to a reduced airflow and increased during both resting inspiration and expiration.

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Objective In subjects with chronic obstructive pulmonary disease (COPD), the effect of partial pressure of CO₂ (PaCO₂) alterations during long-term non-invasive ventilation (NIV) on continuance remains uncertain. We herein investigated the utility of PaCO₂ stability during long-term NIV as a prognostic outcome.
Methods We retrospectively assessed data from 54 subjects with COPD who received long-term NIV. The annual alteration in PaCO₂ during NIV was determined using a simple linear regression method for each subject who had at least two 6-month intervals of PaCO₂ data. Annual alterations in PaCO₂ during long-term NIV and probable confounders were examined, and long-term NIV discontinuation was the major outcome.
Results Data from 37 subjects who met the criteria were analyzed. PaCO₂ during long-term NIV increased slightly in 19 subjects (group 1, <2 mm Hg/y), and increased greatly in 18 subjects (group 2, >2 mmHg/y). In the multivariate modality model, smaller annual alterations in PaCO₂ (p=0.009) and lower PaCO₂ 6 months after the start of long-term NIV (6 m-PaCO₂) (p=0.03) were associated with a significantly higher probability of continuing NIV. The 2- and 5-year probabilities of continuing NIV were 89% and 66% for group 1 and 78% and 32% for group 2, respectively.
Conclusion A lower 6 m-PaCO₂ and a lower annual alteration of PaCO₂ during long-term NIV are significant predictive variables for patients with COPD.

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Objective To examine the safety of and immune response to the influenza A(H1N1)pdm09 vaccine in patients with Duchenne muscular dystrophy (DMD).
Methods Forty-four non-ambulatory patients with DMD hospitalized in a muscle disease ward and 41 healthy healthcare workers each received one dose of the influenza A(H1N1)pdm09 vaccine. Serum samples were collected before and four weeks after vaccination to measure the hemagglutinin inhibition antibody titers.
Results No severe adverse events were noted in any of the subjects. The immune responses of the patients were comparable to those of the healthcare workers. Among the patients, tube feeding and a lower total protein level in the serum were identified to be significantly associated with a lower immune response.
Conclusion A single dose of the vaccine was found to be safe and induced an optimal level of immunity in the DMD patients. The nutritional status may be associated with the immune response in patients with DMD.

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We herein describe a rare case of gastric cancer that was treated with chemotherapy during pregnancy. A woman in her thirty's at 23 weeks of gestation was admitted to the hospital with epigastralgia and left cervical lymph node swelling. She had been previously diagnosed with metastatic adenocarcinoma at another hospital. Findings from a CT scan and esophagogastroduodenoscopy revealed progressive gastric cancer, and the pathology indicated poorly differentiated adenocarcinoma. Chemotherapy was administered at 24 weeks of gestation, without the development of severe toxicity. At 32 weeks of gestation, a healthy baby girl (birth weight 1,442 g, Apgar score 7/8) was delivered by caesarean section. The child continued to show no abnormalities at 12 months after delivery.

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Patients with resolved hepatitis B virus (HBV) infection undergoing chemo- or immunosuppressive therapy are at potential risk for HBV reactivation. To determine whether the host immune response contributes to liver injury, we performed an immunological analysis of a patient with HBV reactivation. Consistent with the detection of HBV DNA in the sera, the number of polyclonal HBV-specific cytotoxic T lymphocytes (CTLs) gradually increased; however, the number of CD4⁺CD25⁺ regulatory T cells (Treg) decreased. The interaction between HBV-specific CTLs and CD4⁺CD25⁺ Treg is an important determinant of liver injury during HBV reactivation. Therefore, monitoring the number of these cells might be a useful modality for the diagnosis of acute hepatitis resulting from HBV reactivation.

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Liver dysfunction was identified in a 16-year-old boy hospitalized with high fever and abdominal pain and fullness. He had received pharmacotherapy for a headache 2 months previously and other drugs for a high fever 4 days prior to being admitted to our hospital. The patient's liver dysfunction was consistent with and fulfilled the criteria for drug induced liver injury, but the laboratory findings showed elevated procalcitonin levels, hyponatremia and leukocytosis. Moreover, we confirmed the presence of human herpesvirus 6 (HHV-6) DNA. The patient exhibited symptoms of high fever and abdominal pain and fullness but no exanthema. The clinical and laboratory findings did not satisfy the criteria for drug-induced hypersensitivity syndrome, and we speculate that the diversity of clinical and laboratory findings may have resulted from HHV-6 reactivation. To the best of our knowledge, this is the first case report on drug-induced liver injury with various findings due to HHV-6 reactivation. HHV-6 reactivation should be considered in patients with drug induced liver injury even in the absence of exanthema.

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A 41-year-old man developed sustained monomorphic ventricular tachycardia (VT) with a left bundle branch block and inferior axis pattern during treadmill exercise concomitantly with unmasking of the typical Brugada electrocardiography (ECG) pattern. The typical ECG phenotype was provoked by a class IC drug. VT was not inducible with programmed electrical stimulation, but premature ventricular beat and non-sustained VT with the same morphology increased in frequency with isoproterenol treatment. Additionally, atrioventricular nodal reentrant tachycardia (AVNRT) was induced by electrical stimulation and VT and AVNRT were treated by radiofrequency catheter ablation.

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It has been reported that hypereosinophilic syndrome may be induced by antituberculosis drugs. We herein report the case of a 43-year-old man who had been on antituberculosis drugs for two months to treat tuberculous meningitis. During therapy, he suffered from drug rash with eosinophilia and systemic symptoms (DRESS) presenting as acute eosinophilic myocarditis, as confirmed on a histopathologic examination. According to the patient's medication history, clinical features and accessory examination findings, the eosinophilic myocarditis was thought to be possibly induced by isoniazid. Although further investigations are needed to confirm causality, isoniazid may be added to the list of drugs with the potential to cause DRESS syndrome.

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We herein report the case of a 65-year-old man with pericardial involvement associated with autoimmune pancreatitis. Chest CT imaging showed pericardial thickening. The patient responded to corticosteroid therapy, and the pericardial thickening resolved. Multiple organs are involved in immunoglobulin G4 (IgG4)-related disease (IgG4-RD); however, only a few cases of IgG4-related chronic constrictive pericarditis have been reported. To our knowledge, this is the first reported case of IgG4-RD with pericardial involvement at an early stage. This case indicates that recognizing pericardial complications in autoimmune pancreatitis is important and that CT imaging may be useful for obtaining the diagnosis and providing follow-up of pericardial lesions in cases of IgG4-RD.

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Congenital contractural arachnodactyly (CCA) is a rare connective tissue disorder characterized by marfanoid habitus with camptodactyly. However, cardiac features have rarely been documented in adults. We herein report a sporadic case of CCA in a 20-year-old woman who developed decompensated dilated cardiomyopathy. The patient did not have any mutations in the FBN1 or FBN2 genes, which are most commonly associated with Marfan syndrome and CCA, respectively. Although whether these two diseases are caused by a mutation(s) in the same gene or two different genes remains unknown, this case provides new clinical insight into the cardiovascular management of CCA.

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Tolvaptan (TLV) is a new vasopressin type 2 receptor antagonist effective in patients with heart failure (HF). We herein describe the case of an 84-year-old woman who developed acute renal injury induced by hypersensitivity to TLV. The patient had received an implanted pacemaker and was diagnosed with exacerbation of chronic HF due to atrial fibrillation, mitral regurgitation, tricuspid regurgitation and left ventricular dyssynchrony. Treatment with tolvaptan increased the urine volume, improved the dyspnea and decreased the edema. However, the patient's renal function and hyperkalemia worsened, and the blood eosinophil count increased without signs of dehydration or hypotension. Positive findings on a drug-induced lymphocyte stimulation test for TLV were consistent with this diagnosis.

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A 50-year-old woman presented with a headache and nausea. A sellar and suprasellar mass was detected on MRI; the tumor was heterogeneously enhanced with gadolinium, and the pituitary stalk was slightly thickened. Laboratory tests revealed severe growth hormone, luteinizing hormone, follicle-stimulating hormone and thyroid-stimulating hormone deficiencies. A pathological examination of the tumor showed scattered granulomas with central necrosis and Langhans giant cells. Tuberculin skin and QuantiFERON TB-Gold tests (QFT-2G) were positive. Accordingly, we diagnosed the patient with pituitary tuberculoma presenting with pituitary dysfunction. Following treatment with antituberculous drugs, the pituitary hormone function normalized and the pituitary tuberculoma disappeared.

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IgG4-related kidney disease (IgG4RKD) is recognized as a fibroinflammatory disease characterized by storiform fibrosis, lymphoplasmacytic infiltration and a high serum IgG4 level. A renal biopsy is necessary to diagnose IgG4RKD in patients without any lesions in other organs. Nephrologists typically perform renal biopsies in patients with abnormal urinalysis, such as proteinuria or hematuria, or renal failure. However, we experienced a patient with IgG4RKD without abnormalities in the urinalysis, renal function or imaging, who had severe interstitial lesions. We therefore propose that renal biopsies should be considered if patients do not show abnormal urinalysis findings and are suspected to have IgG4RKD.

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Infective endocarditis can be a diagnostic challenge. Few cases have so far reported infective endocarditis presenting as renal failure. Pseudoaneurysms of the mitral-aortic intervalvular fibrosa and splenic abscess are rare complications of infective endocarditis. We herein report a case of an 80-year-old man admitted due to anorexia, malaise, edema of the legs and renal failure. A progressive degradation of the patient's renal function was documented and hemodialysis was started. Blood cultures revealed the presence of Enterococcus faecalis, and the patient was treated with ampicillin and gentamicin. The transesophageal echocardiogram findings showed pseudoaneurysms of the mitral-aortic intervalvular fibrosa and in the aortic root wall. Due to abdominal pain, an abdominal CT scan was performed and showed a cystic lesion compatible with splenic abscess. The patient received 6 weeks of antibiotic treatment. There was progressive clinical improvement and regression of the splenic abscess, but no recovery of the renal function. This report illustrates an unusual clinical presentation of infective endocarditis with unusual complications that were successfully treated with antibiotics.

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An 80-year-old man presented with a mildly decreased renal function and increased anti-double-stranded-DNA (anti-dsDNA) antibody levels, and met the diagnostic criteria of the American College of Rheumatology for systemic lupus erythematosus (SLE). However, the incremental increase in creatinine levels and the mild proteinuria were inconsistent with lupus nephritis. We performed a renal biopsy, which revealed interstitial nephritis and minor glomerular abnormalities. Further examinations determined that the renal lesion was due to Sjögren's syndrome secondary to SLE. Following treatment with oral prednisolone, the patient's renal function improved as his anti-dsDNA antibody levels decreased. This case report indicates that renal biopsy should be considered even in elderly individuals when it may assist in the diagnosis, treatment, and prognosis of the patient.

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A 78-year-old woman diagnosed with non-classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency had been under glucocorticoid replacement therapy since the age of 17 years. After several weeks of suffering from gastroenteritis with vomiting, she presented with disturbance of consciousness, hypotension, dehydration, and severe hyponatremia (108 mEq/L) and a markedly increased serum vasopressin concentration (45.5 pg/mL). She regained consciousness after correcting her body-fluid balance with hypertonic saline and intravenous hydrocortisone sodium therapy. Her hyponatremia was likely caused by extra-renal sodium loss and impaired water excretion induced by an increase of serum vasopressin due to volume depletion and glucocorticoid deficiency.

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Neuroendocrine carcinoma as a somatic-type malignancy (STM) arising in an extragonadal teratoma is extremely rare. The effect of chemotherapy has been poorly evaluated in such cases contrary to teratomas with other STMs. We herein demonstrate that systemic chemotherapy may be beneficial in a case of neuroendocrine carcinoma arising in a mediastinal teratoma with pulmonary metastasis. A 31-year-old man presented with mediastinal widening visualized on a chest radiograph. Computed tomography showed a huge mediastinal mass with two pulmonary nodules. Surgical resection confirmed the presence of a neuroendocrine carcinoma arising in a mediastinal teratoma and pulmonary metastasis. The patient subsequently received chemotherapy and has had no recurrence during the 28-month follow-up.

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A 59-year-old woman was admitted to our hospital for an evaluation of a 10-day history of progressive pain and hypoesthesia of the right lower back associated with fever and constipation. Sarcoidosis was confirmed on mediastinal lymph node and skin biopsies. Although the neurological symptoms were suspected due to sarcoidosis-induced nerve dysfunction, nerve conduction studies and other routine examinations did not show any abnormalities. The intraepidermal nerve fiber density assessed on a skin biopsy was significantly reduced, suggesting small-fiber neuropathy (SFN). The patient was finally diagnosed with sarcoidosis-induced SFN, and her neurological symptoms were effectively relieved with high-dose steroid therapy.

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Hairy cell leukemia typically presents with pancytopenia and often mimics aplastic anemia. Making an accurate diagnosis is crucial, as treatment with the purine analogues cladribine and pentostatin brings about durable complete remission in the majority of patients. Surface kappa and lambda flow cytometric analyses of peripheral blood or bone marrow are a powerful screening tool, although routine gating of the entire lymphocyte region may fail to show light chain restriction due to a low tumor burden. We herein demonstrate that accurate subgating of the large lymphocyte region is essential and recommend the application of this method in all cases of pancytopenia of unknown etiology.

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A 76-year-old woman treated with meropenem developed intravascular hemolytic attacks. A direct antiglobulin test was positive for C3d and IgG, and drug-dependent antibody testing indicated that the antibodies were indeed drug-dependent and reacted with drug-treated RBCs and RBCs in the presence of the drug. To our knowledge, this is the first reported case in which the causative antibodies related to meropenem were identified. This case highlights the importance of maintaining a high level of suspicion for drug-induced immune hemolytic anemia in patients with explained hemolysis as well as conducting specialized serologic testing.

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Choroid plexus metastasis (CPM) is extremely rare and originates most frequently from renal cell carcinoma (RCC). We herein report the case of a 58-year-old man who developed a solitary CPM lesion derived from follicular thyroid carcinoma in addition to intraventricular hemorrhage. Computed tomography revealed acute hydrocephalus as a result of the hemorrhage, and we planned endoscopic hematoma evacuation. Since it was too difficult to reach the hematoma, we considered the possibility of a neoplasm and performed a biopsy of the lesion, the results of which led to an accurate diagnosis of CPM in this case. We also review previous reports of CPM originating from thyroid carcinoma compared with RCC.

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We herein report a rare case of microscopic polyangiitis with primary biliary cirrhosis (PBC) and a literature review of six previously reported cases of PBC complicated by anti-neutrophil cytoplasmic antibody-associated vasculitis. Due to the scarcity of similar reports, it was not possible to establish a true overlap syndrome or casual association. When the biliary enzyme levels are elevated in patients with vasculitis, physicians should thus be mindful of the possible coexistence of these diseases.

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Although biological agents are of considerable benefit to patients with rheumatoid arthritis (RA), the potential for opportunistic infections is a critical issue. It is therefore important to achieve a balance between treatment efficacy and controlling opportunistic infection. We herein report the successfully managed case of a 53-year-old patient with RA who developed pulmonary Mycobacterium abscessus infection during treatment with tocilizumab and methotrexate.

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