Objective Local recurrence after radiofrequency ablation (RFA) is a major problem that needs to resolved to increase the survival rate of hepatocellular carcinoma (HCC). CE-US with Sonazoid^®, the second-generation contrast media, can detect smaller HCC lesions and the detection rate of ultrasonically unrecognized hypervascular HCC was improved by CE-US. The aim of the present study was to evaluate the role of CE-US with Sonazoid^® in improving radicality and reducing local recurrence after RFA for HCC.
Patients and Methods A total of 102 nodules treated by RFA at our hospital from January 2006 to October 2009 were enrolled: 31 nodules were treated without CE-US, since CE-US was not yet available (Group A), and 71 nodules were treated with a combination of RFA and CE-US with Sonazoid^® (Group B).
Results The clinical characteristics (sex, virus marker, Child-Pugh grade, with or without transcatheter arterial infusion chemotherapy with lipiodol, and T factor) did not differ significantly between group A and group B. Mean age was significantly older and tumor size was significantly larger in group B. Group B had significantly better radicality compared with group A. The non-local recurrence rate was significantly higher in group B as compared with group A.
Conclusion CE-US with Sonazoid^® greatly helps to improve RFA efficacy in HCC treatment. We suggest that the ability of CE-US with Sonazoid^® to detect an accurate area of HCC before RFA and to immediately detect a residual tumor during RFA might contribute to an increase of the radicality and reduction of local recurrence after RFA.

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Objective A multicenter open trial was performed to clarify the optimal duration of combined pegylated interferon (Peg-IFN) plus ribavirin therapy in patients with chronic hepatitis caused by HCV genotype 2.
Methods A total of 100 patients seen between 2005 and 2007 received the combination therapy for 4 to 52 weeks. The cutoff value of the HCV-RNA-negative (titers under 1.7 Log IU/mL) period during the therapy to predict sustained virological response (SVR) was determined by ROC curve and multivariate logistic regression analyses. The result was validated in 48 patients between 2008 and 2009.
Results SVR was achieved in 78 patients. Serum HCV-RNA titers decreased to less than 1.7 Log IU/mL at 4 weeks of the therapy in 60 patients. The SVR rate in these patients was 85%, which was significantly higher than that of remaining 40 patients with a SVR rate of 68%. An HCV-RNA-negative period of ≥17 weeks was selected as the cutoff value, which showed a significant odds ratio of 4.77 for SVR. Among the 35 patients who showed a decrease of the serum HCV-RNA of less than 1.7 Log IU/mL between 8 and 16 weeks of therapy, the SVR rate was significantly higher in 16 patients with a serum HCV-RNA-negative period of ≥17 weeks (94%) than in 19 patients in whom the period was less than 17 weeks (63%). Similar results were obtained in the subsequent validation study.
Conclusion Prolonged combined Peg-IFN plus ribavirin therapy, with an HCV-RNA-negative period of ≥17 weeks, yielded good therapeutic outcomes in patients with chronic HCV genotype 2 hepatitis.

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Background Systemic lupus erythematosus (SLE) has been reported to be associated with an increased risk of cardiovascular disease. However, most studies have been criticized for either a small sample size or the lack of a prospective control. Our study investigated the relationship of SLE and the subsequent development of ischemic stroke using a nationwide, population-based database in an Asian population.
Methods From 2000 to 2007, we identified a study cohort consisting of a total of 11,637 newly diagnosed SLE patients using the National Health Insurance Research Database in Taiwan. A control cohort of 58,185 subjects without SLE, matched for age, gender, and comorbidities were selected for comparison to observe the occurrence of ischemic stroke in these two groups.
Results During a follow-up period of up to 7 years, ischemic stroke developed in 258 (2.22%) of the patients with SLE and in 873 (1.5%) of patients in the comparison cohort. Kaplan-Meier analysis also revealed a tendency of SLE patients toward ischemic stroke development (log rank test, p = 0.001). After Cox model adjustment for patients' demographic characteristics and selected comorbidities, patients with SLE were found to have a 1.67-fold (95% CI, 1.45 to 1.91) higher risk of developing ischemic stroke.
Conclusion Patients with SLE have an increased risk of stroke.

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Objective The present study was undertaken to investigate the role of neck circumference (NC) in predicting prehypertension in children and adolescents.
Methods We conducted a cross-sectional study including 6,802 children and adolescents (3,631 boys and 3,171 girls) aged 5-18 years. Main anthropometric data and related information were collected.
Results The prevalence of prehypertension was 14.4%, 16.4% and 17.1% in normal weight, overweight and obese group respectively. Among the participants with normal body mass index (BMI), higher NC was significantly associated with increased odds of prehypertension (OR: 1.439; 95% CI: 1.118 to 1.853) after adjustment for age, gender, BMI and WC. Significant associations between NC and either systolic blood pressure (SBP) (β=0.581 mmHg) or diastolic blood pressure (DBP) (β=0.236 mmHg) were found in the linear regression. No significant ORs were observed either in overweight or in obese categories.
Conclusion We concluded that additional measure of NC might expand the ability of BMI for the identification of prehypertension in normal weight children and adolescents.

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Objective A strong positive correlation between estimated glomerular filtration rate (eGFR) multiplied by follow-up time and memory function has been demonstrated previously. The purpose of this study was to investigate the factors affecting progression of cognitive dysfunction in the elderly with different levels of kidney function.
Methods In April 2002, a prospective study on progression of cognitive function was conducted on 356 elderly people. All participants received a comprehensive geriatric assessment, serum biochemical test, homeostasis model assessment (HOMA), and a genetic polymorphism study, including polymorphism of apolipoprotein E (APOE), Cystatin, C-reactive protein (CRP) and tumor necrosis factor (TNF). Lean body mass-adjusted eGFR was used to evaluate severity of chronic kidney disease (CKD), and a clinical dementia rating scale was used to assess cognitive dysfunction. Patients were stratified according to eGFR (≥49 mL/min and <49 mL/min).
Results Using multivariate logistic regression models, older age (OR=1.27, 95% CI=1.00-1.61) was associated with a high risk for progression of cognitive function in patients with a relatively low eGFR. In patients with lower eGFR, higher mini-mental state examination (MMSE) scores (OR=0.23, 95% CI=0.10-0.54) were correlated with a low risk for progression of cognition, while in patients with higher eGFR, higher MMSE scores (OR=0.39, 95% CI=0.23-0.65) were less correlated with cognitive dysfunction. However, elevated serum plasminogen activator inhibitor-1 (PA1-1) was associated with a high risk for cognitive dysfunction (OR=1.06, 95% CI=1.01-1.11) in patients with a better eGFR.
Conclusion Age, MMSE score and serum PAI-1 were found to be factors that predicted cognitive dysfunction at different functional levels of CKD.

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Objective Streptococcus pneumoniae is an important and common pathogen of acute pneumonia. The urinary pneumococcal antigen test has been increasingly used for the diagnosis of pneumococcal pneumonia, but there have been few studies on pneumococcal pneumonia after this test became prevalent. The present study was conducted to characterize the clinical features of pneumococcal pneumonia after the introduction of the urinary antigen test.
Methods We retrospectively analyzed 111 cases from 105 patients with pneumococcal pneumonia in our hospital between 2007 and 2010, and collected data regarding background characteristics, laboratory data, isolated bacteria, and clinical courses.
Results The cases analysed included 77 of community-acquired pneumonia (CAP), 21 of healthcare-associated pneumonia (HCAP), and 13 of hospital-acquired pneumonia (HAP). Penicillin-resistant Streptococcus pneumoniae was isolated in 12.5 and 55.6% of non-HAP and HAP cases, respectively. When the cases were divided into 5 groups according to A-DROP scores (0, 1, 2, 3 and 4 points), critical events occurred at 0, 0, 8.7, 15.4, and 75.0% in groups of cases of 0, 1, 2, 3, and 4 points, respectively. Similarly, critical events occurred at 66.7, 4.7, 3.8, and 9.1% in groups of cases according to WBC counts of <4,000, 4,000-9,999, 10,000-19,999, and ≥20,000 cells/μL, respectively.
Conclusion Most of the pneumococcal pneumonia cases occurred as CAP and were treated successfully. HAP cases were frequently penicillin resistant. Elevated A-DROP scores and decreased WBC counts were found to be predictive of critical events.

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Objective It remains unclear which patients with acute stroke need percutaneous endoscopic gastrostomy (PEG) placement and when it should be performed. The aim of this study was to identify the predictors for oral intake 6 months after onset in stroke patients.
Methods Data were obtained from a prospectively registered database of 738 acute ischemic stroke patients both with and without a history of stroke. Patients who survived for 6 months and replied to a questionnaire were divided into two groups: those with oral intake 6 months after onset and those without. Predictors for oral intake 6 months after onset were analyzed.
Results Forty-five patients died during their hospital stay, 145 did not reply to the questionnaire, and 23 died during follow-up. Of the residual 525 patients, 485 (92.4%) had oral intake after 6 months. On multivariate analysis, modified Rankin Scale score 0 before admission (OR 2.70, 95%CI 1.10-6.61) and National Institutes of Health Stroke Scale (NIHSS) score ≤9 on day 10 (OR 21.12, 95%CI 5.04-88.39) were independent predictors for oral intake after 6 months, while NIHSS score on admission was not.
Conclusion Clinicians should be cautious about PEG placement for stroke patients who were independent prior to their stroke and whose stroke severity decreases 10 days after admission, because their swallowing dysfunction may improve within a brief period.

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Objective Acinetobacter baumannii is a worldwide nosocomial pathogen that has become increasingly common over the past few decades, and strains of multidrug-resistant A. baumannii have been increasing. The aim of this study was to assess the clinical characteristics of A. calcoaceticus-A. baumannii complex (Acb complex) strains and to determine the risk factors of this infection.
Methods The medical records of 121 patients at Nagasaki University Hospital from whom Acb complex had been isolated between January 2007 and December 2009 were retrospectively reviewed. Patient backgrounds, sensitivity to antibiotics, risk factors for infection, and prognosis were evaluated.
Results Lower respiratory isolates accounted for 73% (147 strains) of all 201 isolates. Most of the isolates were sensitive to carbapenems. Of the 121 patients (74 males and 47 females; mean age: 62.1 years), 48 (39.7%) had malignancy and 75 (62.0%) were treated with antibiotics prior to isolation. Thirty-seven of the patients in this study (30.6%) were infected by Acb complex and the most frequent clinical manifestation was pneumonia (18 cases; 48.6%). Approximately 60% of infected patients were treated with β-lactam agent in combination with β-lactamase inhibitors or carbapenems. The mortality rate of infected patients was significantly higher than that of colonized patients (infected: 24.3%, colonized: 6.0%, p<0.05). Risk factors for Acb complex infection include being over 60 years of age, chronic liver disease, and the use of first-generation cephalosporins prior to isolation.
Conclusion Acb complex was relatively sensitive to antibiotics. The appropriate usage of antibiotics should be continued for the prevention of drug resistance in Acb complex.

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Objective The mortality rates for bacteremia due to Pseudomonas aeruginosa remain high. In our hospital, we performed retrospective analyses to determine risk factors for mortality among patients with bacteremia caused by P. aeruginosa.
Materials and Methods This retrospective cohort study was conducted among adult patients with bacteremia due to P. aeruginosa at Jikei University Hospital. We analyzed factors, such as age, gender, underlying disease, initial antimicrobial treatment, and primary site of infection to determine which of these were predictive of mortality in patients with P. aeruginosa bacteremia.
Results One hundred and thirty-four patients with P. aeruginosa bacteremia were identified between April 2003 and March 2010. The 30-day mortality rate among all patients with P. aeruginosa bacteremia was 20.9%. The most common underlying disease was leukemia (20.9%), and the most common primary site of infection was the urinary tract (24.6%). Seventy-one patients (65.7%) were treated with an appropriate initial antimicrobial regimen for P. aeruginosa bacteremia. However, these patients had similar 30-day mortality to that observed in patients not administered appropriate antibiotics. This study revealed that risk factors for the 30-day mortality were thrombocytopenia and polymicrobial P. aeruginosa bacteremia (p<0.01).
Conclusion Thrombocytopenia and polymicrobial bacteremia were associated with a greater incidence of 30-day mortality among patients with P. aeruginosa bacteremia. On the other hand, age, underlying disease, and inappropriate initial empirical antimicrobial treatment did not affect mortality.

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Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. We report an unusual case of sarcoidosis in a woman presenting with cardiac sarcoidosis and massive splenomegaly with a familial history of cardiac sarcoidosis. Cardiac sarcoidosis was diagnosed based on electrocardiogram, echocardiogram, ¹⁸F-fluoro-2-deoxyglucose positron emission tomography (¹⁸F-FDG-PET) and skin histological findings. We performed splenectomy to rule out malignant lymphoma, and histological findings confirmed sarcoidosis. After splenectomy, we initiated prednisolone therapy. After 20 months of diagnosis, she was symptom free. Echocardiography and ¹⁸F-FDG-PET may be a key diagnostic tool and prednisolone therapy may be safe, effective, and feasible for cardiac sarcoidosis.

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Congenital absence of the left circumflex artery is a rare anomaly of the coronary arteries. A 52-year-old man who developed acute inferior myocardial infarction underwent coronary angiography which revealed the absence of the left circumflex artery and that the surrounding structures were supplied by the infarct-related super-dominant right coronary artery. Two stents were implanted into the right coronary artery and one stent into the mid portion of the left anterior descending artery. Follow-up coronary angiography at 67 months showed no detectable restenosis, and 64-slice multidetector computed tomography confirmed the absence of the left circumflex artery. The circumflex artery as a terminal extension of a culprit right coronary artery has not been previously reported.

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We report a 53-year-old man who presented with dyspnea and low-grade fever. Cardiac ultrasound showed pulmonary hypertension and an ill-defined echogenic mass within the pulmonary trunk. Computed tomography scan revealed an inhomogeneous mass which filled the main pulmonary trunk with near-total occlusion, and extended into both pulmonary arteries. Anticoagulant therapy was administered based on a presumptive diagnosis of pulmonary thromboembolism. Positron-emission tomography scan was useful for differentiating the mass, which was determined as a pulmonary artery sarcoma by surgical resection. Although complete resection was impossible, the patient survived for 20 months with adjuvant chemotherapy and medical treatment.

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A 73-year-old woman had previously been diagnosed with CREST syndrome, PBC and diabetes. Hepatic fibrosis was not evident, in spite of the transudative ascites and active esophageal varices. ACA were positive, whereas AMA and anti-gp210 antibodies were negative. She showed low urinary excretion of C-peptide and was weakly positive for anti-GAD antibody. She was diagnosed with a form of PBC that progresses via portal hypertension rather than liver failure and with SPIDDM. Her HLA type did not contain risk allele for IDDM or PBC. SPIDDM should be considered when patients with PBC with portal hypertension-type progression develop diabetes.

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A 47-year-old woman presented with hypokalemia (2.4 mmol/L). She also had hypomagnesemia, hypocalciuria, and hyperreninemic hyperaldosteronism. Sequence analysis revealed a compound heterozygous mutation, R655C and R955Q, in the SLC12A3 gene. These findings were compatible with Gitelman's syndrome (GS). Eplerenone, a selective aldosterone blocker, in combination with oral potassium chloride improved serum potassium level (3.6 mmol/L) with no apparent adverse effect. Although eplerenone has an advantage over spironolactone for its selective affinity for the aldosterone receptor, the efficacy and safety of eplerenone for GS is little understood. Our observation suggests that eplerenone is a useful treatment option for GS.

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Adenoid cystic carcinoma (ACC) is a malignant neoplasm that commonly arises in the major or minor salivary gland and usually forms mass lesions. Here, we report a case of ACC involving a 56-year-old man, who displayed right multiple cranial nerve palsies with ipsilateral severe facial pain but not any mass formation. Right submaxillary gland biopsy after repeated challenges at last revealed the primary focus of ACC with perineural invasion and without lymph node metastasis. The neurological manifestations were considered to be attributed to the perineural spread of ACC. It is extremely rare for ACC to show Garcin's syndrome without mass formation.

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Oculodentodigital dysplasia (ODDD) is a rare autosomal dominant inherited disorder mainly affecting the development of the face, eyes, dentition, limbs, hair and heart. GJA1 (the gap junction protein α-1) has been determined to be a causative gene of ODDD, mapped to chromosome 6q22-24 identified as the connexin 43 gene (Cx43). We found a novel GJA1 mutation (W25C) as the possible causative gene in this sporadic ODDD patient with neurological features of motor deficits by pyramidal tract signs, and sensory deficits due to peripheral nerve disturbance. It is also notable that the MRI of this patient demonstrated widespread aberrant signal lesions in the brain and brainstem.

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Writer's cramp is a rare movement disorder of unknown etiology, in which a cramp is elicited primarily or exclusively with writing. We describe a patient with primary writer's cramp that was completely improved by drinking a small amount of alcohol. Although it is unclear how "alcohol" ameliorated the dystonia, this case suggests that alcohol might reverse the pathophysiologic changes in the entire basal ganglia circuit. In addition, we cannot rule out the possibility that the anxiolytic influence of alcohol may contribute to the beneficial effects on dystonia.

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We report a case of elderly-onset neuromyelitis optica (NMO) positive for the anti-aquaporin-4 (AQP-4) antibody; symptoms developed after the diagnosis of prostate adenocarcinoma and relapsed after a 23-valent pneumococcal polysaccharide vaccination. We suggest that activation of CD4-positive T cells and secretion of interferon-gamma induced by adenocarcinoma and complement activation induced by vaccination are responsible for the onset and relapse of NMO, even if a patient is positive for the anti-AQP-4 antibody. This case supports the previous experimental finding that the anti-AQP-4 antibody does not cause NMO-like lesions when injected alone, but does so after the induction of T cell-mediated experimental autoimmune encephalomyelitis or when co-injected with human complement.

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We present a case of Churg-Strauss syndrome complicated by chronic periaortitis. A 68-year-old man presented with wheezing, dyspnea, purpurae, and numbness of the extremities. Antineutrophil cytoplasmic antibodies were absent; however, eosinophilia, a pulmonary infiltrative shadow on chest X-ray, eosinophilic vasculitis on histologic examination of skin and kidney, and mononeuritis multiplex were detected. Churg-Strauss syndrome was diagnosed. Contrast-enhanced abdominal computed tomography revealed a periaortic soft tissue mass extending from the subphrenic abdominal aorta to the proximal area of the bilateral iliac arteries. This indicated chronic periaortitis, probably caused by vasculitic activities. Both disorders improved with steroid therapy.

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Chlamydia trachomatis and Chlamydophila pneumoniae are human pathogens; the former being the etiologic agent for trachoma as well as a prevalent sexually transmitted bacterium, while C. pneumoniae is a respiratory pathogen responsible for community-acquired pneumonia. Patients with reactive arthritis show evidence of present or past Chlamydial infection. Chlamydia spp., has been strongly implicated as a triggering factor for reactive arthritis. We describe the simultaneous occurrence of C. pneumoniae and C. trachomatis infections in a subject with reactive arthritis. We suggest treatment for a patient with Chlamydia-associated arthritis to define a means by which persistent organisms can be induced to return to the active developmental cycle, thereby making them more accessible to antibiotic activity.

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