Objective Papaverine is used for the evaluation of functional status of the coronary arteries but it may provoke severe ventricular tachyarrhythmias (VTAs). This study compared the clinical and ECG characteristic of patients with papaverine-induced VTAs.
Materials and Methods The study involved 25 patients who underwent a fractional flow reserve (FFR) study. FFR was determined as the ratio of blood pressure at the distal and the proximal site of stenosis after intracoronary papaverine administration at 12 mg into the left and 8 mg into the right coronary artery. The QT and QTU intervals were measured manually in the limb leads and in the precordial leads, respectively and corrected by the R-R interval to obtain QTc and QTUc. The clinical and ECG data were compared between the patient groups with and without VTAs.
Results After papaverine administration into the left (20), right (3) or both coronary arteries (2), the RR interval shortened, but non-significantly however, the QT interval (and QTc) and the QTU interval (and QTUc) were significantly prolonged. VTAs developed in four women: torsade de pointes in 3 followed by ventricular fibrillation and ventricular premature beats in 1 patient. After papaverine administration, QTU and QTUc were more prolonged in women than men and in patients with VTAs compared to those without. Just prior to VTAs, giant T-U waves were observed.
Conclusion Intracoronary papaverine was used to determine FFR which may induce VTAs. VTAs developed only in women and they were closely related to prolongation of the QTU intervals with prominent T-U waves.

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Objectives The present study evaluated the clinical benefits of a new therapeutic method of radiofrequency catheter ablation (RFCA) using an EnSite system for inappropriate sinus tachycardia (IST).
Materials and Methods Six patients with debilitating IST underwent RFCA using EnSite. Using the beta-adrenergic blocker and agonist, the heart rate was controlled between 70 to 150 bpm before and after the RFCA. The areas of the breakout sites (BOSs) were clearly distinguished between those from the normal P-wave zones during rates of less than 100 bpm and those from more upper rate sites during rates of more than 100 bpm using the EnSite system, in accordance with the appearance of tall P-waves (tall P-wave zone) in the IST patients. This was selected as the target for ablation.
Results After the RFCA, the BOSs observed during heart rates of more than 100 bpm moved completely from the tall P-wave zone to the normal P-wave zone in the IST patients. The total number of heart beats and average heart beat on the 24-h Holter monitoring decreased statistically from that before the RFCA to that after, and no adverse heart rate responses was observed after the RFCA. Before the RFCA, the brain natriuretic peptide was elevated, New York Heart Association functional class was worse, and there was an impaired exercise tolerance observed with exercise electrocardiogram testing. The RFCA for the IST significantly improved those parameters.
Conclusion This new therapeutic method for IST using EnSite is effective and produces clinical benefits.

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Objective To evaluate the impact of oxidative stress and cellular adhesion molecules on ischemic reactive hyperemia (IRH) in patients with OSA.
Materials and Methods Consecutive patients treated at a sleep laboratory and whose polysomnography showed an apnea hypopnea index (AHI) ≥5 were included in the study. Patients with acute illness receiving vasoactive medications were excluded. Based on their oxygen desaturation index (ODI), subjects were assigned to the mild-moderate (ODI ≤30) or the severe desaturation group (ODI >30). Then IRH and oxidative stress markers [malondialdehyde (MDA)] and proinflammatory markers (ICAM-1 and P-selectin) were measured.
Results Sixty-eight subjects with OSA were included, 31 in the mild-moderate desaturation group and 37 in the severe group. No differences by age, gender and body mass index were observed. The severe desaturation group showed significantly higher values in the AHI, MDA, ICAM-1 and P-selectin (p<0.005), as well as a worsening of IRH (p=0.001). Only ICAM-1 (p=0.019) and P-selectin (p=0.033) were independently associated with IRH in a multiple-linear regression model.
Conclusion Patients with OSA and greater intermittent hypoxia showed worse endothelial function, and higher levels of MDA, ICAM-1 and P-selectin. Nevertheless, ICAM-1 and P-selectin rather than MDA were independently associated with IRH.

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Objective The quantification of physical activity is useful for the management of chronic obstructive pulmonary disease (COPD) but has not been fully established yet. The DynaPort Activity Monitor^® (DAM), a triaxial accelerometer is the only well validated accelerometer in Caucasians but it has not been validated in Japanese COPD patients. We initially evaluated the reproducibility of the DAM in Japanese healthy subjects. Next, the within-subject repeatability and the determinants of physical activity were investigated in Japanese COPD patients.
Materials and Methods The durations of locomotion, standing, and sitting measured by the DAM were compared to those of the self-records (Study 1). COPD patients wore the DAM for 3 days and the durations of each activity of 2 selected days were compared to assess the repeatability (Study 2). The relationship between the duration of locomotion and the physiological properties were examined (Study 3).
Results The activities measured by the DAM were significantly associated with those of the self-records (p<0.001). The values of the intra-class correlation coefficient (ICC) for the reproducibility were over 0.99, and the agreement with the self-records was observed for the DAM. Similarly, the values of ICC for repeatability were over 0.84 in all activities, and there was no systematic bias in the COPD patients. The duration of locomotion was negatively correlated with the total lung capacity (TLC) and closing capacity/TLC, but not with other pulmonary functions, exercise capacity, muscle force, dyspnea, or modified BODE index.
Conclusion The triaxial accelerometer is reliable for evaluating the physical activity of Japanese COPD patients.

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A 27-year-old woman visited our hospital because of high fever. She had been diagnosed as 22q11.2 deletion syndrome (22q11.2DS) due to her cardiac history (tetralogy of Fallot), thymic hypoplasia and 22q11.2 deletion. She had a normal CD4/CD8 ratio, a slightly decreased lymphocyte count and normal serum immunoglobulin levels. Blood cultures were positive for Staphylococcus lugdunensis (S. lugdunensis). Infection route of S. lugdunensis in this case was unclear. The patient was successfully treated with several intravenous antibiotics. Infection should be considered when managing patients with 22q.11.2DS. regardless of whether their immune system is impaired.

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Metastasis to lymph nodes in patients with hepatocellular carcinoma (HCC) is generally observed to occur in regional chains of involvement. We encountered a HCC patient who had hepatitis C virus-related liver cirrhosis and portal vein tumor thrombosis, accompanied by metastasis to only a single cervical lymph node, skipping the common intermediate routes of involvement. It is noteworthy, so as not to miss the clinical diagnosis, to remark that metastasis to the cervical lymph node is rarely observed in patients with HCC, and that the metastasis can skip to the regional lymph nodes.

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A 70-year-old man was diagnosed with mid-ventricular obstructive hypertrophic cardiomyopathy (MVOHCM) with apical aneurysm and paradoxic jet flow. At cardiac catheterization, pressure study showed that there was a markedly high pressure-gradient of 90 mmHg between the apex and the base in systole. Apical pressure was 350 mmHg after premature ventricular contraction. The apical aneurysm was already dilated and spherical in late systole; the absence of active relaxation was considered to be the cause of the paradoxic jet flow. In this report, we suggest the pathogenesis of left ventricular apical aneurysm and paradoxic jet flow in MVOHCM.

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Echinococcus granulosus is a common infestation in sheep and cattle raising countries. Although it is typically encountered in liver and lung, rare cardiac involvement of this infestation has very important clinical complications such as heart failure, valve regurgitation, pericardial effusion-tamponade and arrhythmia. In addition, pericardial infestation is an extremely rare condition of Echinococcus granulosus. Here, we report a case of recurrent pericardial hydatid cyst presenting exertional dyspnea, palpitation and presyncope attacks in a 72-year-old man. The diagnosis of recurrent pericardial hydatid cyst was made by transthoracic echocardiography, computed tomography and surgical history

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A 36-year-old woman with Cushing syndrome was evaluated for coexisting hyperaldosteronism, which was suggested by an abnormal response of the aldosterone-to-cortisol ratio in peripheral blood to the ACTH-administration despite a low basal aldosterone-to-renin ratio. Computed tomography revealed two independent tumors in the left adrenal gland, and adrenal venous sampling indicated hyperaldosteronism in addition to hypercortisolism in the same side. Postsurgical study including immunohistochemical analysis of steroidogenic enzymes suggested one adenoma to be cortisol-producing and the other, aldosterone-producing. The comorbidity of these different hormone-producing adenomas is not rare and careful pre-surgical evaluation is necessary to avoid post-surgical exacerbation of latent hyperaldosteronism.

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Endobronchial metastasis from thyroid follicular carcinoma is a rare manifestation. We describe a case of 62-year-old woman who underwent total thyroidectomy due to thyroid follicular carcinoma 19 years previously. Computerized tomography and bronchoscopy suggested an endobronchial enhancing nodule in the right bronchus intermedius, resulting in right middle lobe (RML) and right lower lobe (RLL) collapse. A biopsy specimen showed thyroid follicular carcinoma identical to that taken from a specimen previously. She underwent metastectomy and high-dose radioactive iodine ablation therapy. To our knowledge, this patient represents the first case of endobronchial metastasis with a long past history of thyroid follicular carcinoma.

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Disseminated adenovirus disease after allogeneic hematopoietic stem cell transplantation (HSCT) is lethal in most cases, especially when it develops as fulminant hepatic failure. We encountered a patient who developed fulminant hepatic failure caused by adenovirus infection. She did not show manifestations of graft-versus-host disease and the results of serum tests for viral infection were all negative. Abdominal computed tomography (CT) findings were consistent with peliosis hepatitis. She died of fulminant hepatic failure, however, and pathological examinations of the liver specimen obtained after her death revealed adenovirus infection. In this report, we review the clinical characteristics and imaging findings of fulminant hepatic failure caused by adenovirus infection.

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A 19-year-old woman suffered from severe excessive daytime sleepiness accompanied with long sleep episodes both in the daytime and nighttime and frequent episodes of cataplexy shortly after the removal of craniopharyngioma in the intrasellar space. Multiple sleep latency test showed a typical finding of narcolepsy, and cerebrospinal fluid orexin concentration was below the narcolepsy cut-off value. MRI-tractography showed a clear lack of neuronal fiber connections from the hypothalamus to the frontal lobe. SPECT using ¹²³I-IMP showed frontal hypoperfusion. These connection damages could have been responsible for the occurrence of narcolepsy-like symptoms and long daytime sleep episodes in this case.

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A 66-year-old Japanese man with an 11-year history of Mikulicz's disease (MD) received continuous corticosteroid administration. At age 58, a left renal pelvic mass was identified and diagnosed as an IgG4-related inflammatory pseudotumor. The patient underwent an operation to remove the tumor. Subsequently, he contracted repeated pulmonary infections and eventually died of severe gastrointestinal bleeding. Autopsy revealed systemic lymph node swelling and infiltration in some organs, and diffuse large B-cell lymphoma (DLBCL) was diagnosed. These findings suggest that an IgG4-related disease can be causally related to the development of malignant lymphoma through the occurrence of mucosa-associated lymphoid tissue lymphoma.

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Hypogammaglobulinemia is a reduction or absence of immunoglobulin, which may be congenital or associated with immunosuppressive therapy. In addition to infectious diseases, autoimmune diseases have also been reported in patients with hypogammaglobulinemia. A 26-year-old man with hypogammaglobulinemia had multiple joint pain and swelling with erosive changes in the proximal interphalangeal joint of the right middle finger on X-ray film, mimicking rheumatoid arthritis (RA). As polyarthritis remained after immunoglobulin replacement therapy and there was no finding indicating any infection at that time, a diagnosis of RA was made. Prednisolone and etanercept were started. However, his polyarthritis did not improve and he developed meningitis and massive brain ischemia. Finally, a diagnosis of disseminated Mycoplasma hominis infection was made. The differential diagnosis of polyarthritis in patients with hypogammaglobulinemia should strictly exclude Mycoplasma infection by culture with special media or longer anaerobic culture, and molecular methods for mycoplasma.

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Gefitinib and erlotinib are first-generation small molecular inhibitors of EGFR tyrosine kinase activity. To the best of our knowledge, to date, two reports have stated that patients with NSCLC who develop severe hepatotoxicity secondary to gefitinib treatment can be safely switched to erlotinib. However, the reverse situation has not been reported. Here, we present the first case with non-small cell lung cancer harboring EGFR mutation who developed grade 3/4 hepatotoxicity after initiation of erlotinib, which resolved when therapy was changed to gefitinib. As far as we know, this is the first report showing the efficacy of gefitinib for a non-small cell lung cancer patient who developed severe hepatotoxicity while under erlotinib therapy.

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A 37-year-old woman was evaluated at our hospital for a treatment-resistant proctosigmoiditis. Fifteen years previously, ulcerative colitis was suspected and she was cured by steroid therapy. Colonoscopy revealed multiple sessile polyps covered with white exudate from the rectum to the sigmoid colon. Histopathological examination revealed a mucosal surface cover of granulation tissue. Based on these findings, cap polyposis was diagnosed. She underwent Helicobacter pylori eradication therapy, and at 1 month after therapy, her symptoms had improved. We present a case of cap polyposis relapsing 15 years after remission with steroid therapy, and that was improved by H. pylori eradication therapy.

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