Objective Peripheral arterial disease (PAD) is a common clinical manifestation of the systemic atherosclerotic process, and the ankle-brachial index (ABI) is an ideal tool to diagnose PAD. Currently, there have been few long-term follow-up studies focused on the associations of the ABI with all-cause mortality and cardiovascular disease (CVD) mortality in Chinese MetS patients. The aim of this study was to evaluate the usefulness of ABI to predict the prognosis of CVD in hospitalized Chinese patients with metabolic syndrome (MetS).
Methods Participants from multi-center departments were followed up from November 2004 to January 2011. The study sample actually comprised 1,266 valid participants whose age was ≥35 years. Patients were separated into four groups, with an ABI ≤0.4, 0.41-0.7, 0.71-0.9 and 0.91-1.4. An ABI ≤0.9 was defined as PAD, and subjects with an ABI >1.4 were excluded because of the false negative rate. Factors related to all-cause and cardiovascular mortality were observed by Cox models and the log rank test. Potential confounding variables with values of p<0.10 were adjusted for the multivariate analysis.
Results An abnormal ABI value was strongly, independently, and inversely correlated with the all-cause and cardiovascular mortality. After adjusting for age and other covariates, Cox models revealed that an abnormal ABI value was still correlated with the all-cause mortality (relative risk/RR/=1.82, 95% confidence interval/CI/=1.45-2.34 p<0.01), and CVD mortality (RR=1.88, 95% CI=1.51-2.90 p<0.01).
Conclusion An abnormal ABI value was not only a significant and independent risk factor for CVD, but also for the survival rate in Chinese MetS patients. Routine ABI evaluation could therefore be helpful for identifying high risk patients, especially MetS patients.

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Objective To compare the acute hemodynamic effects of aerosolized iloprost and inhaled nitric oxide (NO) in adult congenital heart disease (CHD) patients with severe pulmonary arterial hypertension (PAH).
Methods One hundred and eighty five adult CHDs with severe PAH were nonrandomized into two groups (iloprost, n=127; NO, n=58). Various hemodynamic parameters were measured before and after iloprost or NO inhalation.
Results Iloprost and NO inhalation resulted in significant reductions in pulmonary arterial pressure (from 110.6±21.8 mmHg to 105.5±22.3 mmHg, p<0.05; from 113.1±18.7 mmHg to 107.2±19.9 mmHg, p<0.05, respectively) and pulmonary vascular resistance (PVR) (from 13.4±8.3 Wood units to 9.6±6.4 Wood units, p<0.01; from 13.7±7.1 Wood units to 9.3±4.9 Wood units, p<0.01, respectively) and increases in pulmonary blood flow (from 6.7±3.3 L/min to 9.4±5.8 L/min, p<0.05; from 6.6±3.1 L/min to 9.6±5.9 L/min, p<0.01, respectively) and the Qp/Qs ratio (from 1.5±0.8 to 2.1±1.4, p<0.01; from 1.5±0.8 to 2.0±1.3, p<0.01, respectively). When the effects of inhaled iloprost and NO were compared, similar reductions in pulmonary arterial pressure and pulmonary vascular resistance were observed. Aerosolized iloprost and inhaled nitric oxide (iNO) were generally well tolerated and no patient experienced any side effects during inhalation.
Conclusion Aerosolized iloprost can be effectively and safely used and might be an alternative to NO for testing pulmonary vascular reactivity and treating severe PAH in adult CHD patients.

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Objective Abnormal left ventricular (LV) geometry, especially concentric hypertrophy, is associated with cardiovascular morbidity and mortality. The aim of this study was to evaluate the impact of obstructive sleep apnea syndrome (OSAS) and the effects of nasal continuous positive airway pressure (CPAP) on the LV geometric patterns.
Methods The LV morphological parameters, including the LV mass index (LVMI) and the relative wall thickness (RWT), were evaluated using echocardiography in 37 patients with OSAS diagnosed on polysomnography and 34 control subjects. Based on the values of LVMI and RWT, the LV geometry was classified as normal, concentric remodeling, concentric hypertrophy or eccentric hypertrophy. The echocardiographic parameters were reassessed after three months of CPAP treatment.
Results Compared with the controls, the OSAS patients had a higher proportion of concentric hypertrophy patterns (54% vs. 0%, p<0.001) and a lower proportion of normal geometric patterns (5% vs. 62%, p<0.001). A univariate logistic regression analysis showed the apnea-hypopnea index, lowest oxygen saturation, hypoxemia index and body mass index to each be significantly associated with the presence of concentric hypertrophy. In a multivariate analysis of these factors, the apnea-hypopnea index was found to be a significant independent factor associated with the presence of concentric hypertrophy (odds ratio: 1.06, p=0.008). Three months of CPAP treatment resulted in significant decreases in LVMI and the proportion of cases with concentric hypertrophy (both p=0.025).
Conclusion In our limited study population, OSAS patients were found to be associated with a high prevalence of concentric LV hypertrophy patterns that were able to be reversed with three months of CPAP treatment.

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Objective Pneumocystis jiroveci pneumonia (PJP) infection is a rare but lethal complication in immunocompromised hosts. However, risk factors for PJP infection in glomerulonephritis (GN) patients receiving immunosuppressants are unknown.
Methods From August 2009 to July 2010, we encountered a cluster occurrence of PJP infection in our renal biopsy patients. Seven of 73 GN patients under immunosuppressant agents developed PJP infection, which were diagnosed by the Giemsa and Gomori's methenamine silver stains of the bronchoalveolar lavage specimen.
Results The average time of PJP onset was 2.4 months after immunosuppressant initiation. We found that the immunosuppressant regimens were equal between patients with and without the development of PJP infection regarding the daily dose per body weight, treatment duration, and accumulative dose per body weight. Logistic regression analysis indicated that high serum creatinine, low hemoglobin, and low absolute lymphocyte count at immunosuppressant initiation, and high chronicity indices of kidney pathology were predictors of PJP infection. In addition, patients with PJP infection had persistently worse renal function, more severe anemia, and more severe lymphocytopenia as compared to those without.
Conclusion Prophylactic therapy for P. jiroveci and immunosuppressant dose reduction should be considered in GN patients with high chronicity of their kidney diseases and/or persistent lymphocytopenia.

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Objective Continuous positive airway pressure (CPAP) is an effective treatment for obstructive sleep apnea (OSA). After performing an initial titration study, most physicians do not have the CPAP equipment retitrated unless the patient complains about the CPAP use. Several automated CPAP devices are used clinically that can detect upper airway obstructive events and provide information about residual events while patients are on CPAP. The aim of this study was to compare the apnea-hypopnea index (AHI) determined by automated CPAP devices to that obtained from polysomnography.
Methods Patients with OSA underwent polysomnography for CPAP titration using the REMstar Auto M-series. The initial two hours of CPAP titration were spent at a subtherapeutic pressure of 4 cmH₂O so that more breathing events could be observed. The correlations between the simultaneous determination of the AHI with polysomnography (AHI-PSG) and the automated device (AHI-RAM) during the subtherapeutic, therapeutic and overall phases were evaluated. In addition, the apnea index (AI) and the hypopnea index (HI) were each evaluated separately.
Results Sixty patients were enrolled. The mean AHI on diagnostic PSG was 35.2±2.6 events/hour. Strong correlations were observed between the AHI-PSG and the AHI-RAM (subtherapeutic: r=0.958, p<0.001; therapeutic: r=0.824, p<0.001; overall: r=0.927, p<0.001). A slightly stronger correlation was observed between the AI values, whereas a weaker correlation was observed between the HI values in all three phases.
Conclusion Strong correlations between the AHI-PSG and the AHI-RAM were observed. The correlations were weakened when the analysis was limited to the HI and the therapeutic phase.

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Objective Determining the prognoses of patients with acute ischemic stroke is difficult. Therefore, the aim of this study was to evaluate whether the combined assessment of plasma N-terminal pro-brain natriuretic peptide (NT-pro-BNP) and the National Institutes of Health Stroke Scale (NIHSS) variables is relevant to the prognosis of patients with acute cerebral ischemic infarction in-hospital.
Methods We enrolled 122 patients who were within three days of onset of acute ischemic stroke. We measured the plasma NT-pro-BNP level of each patient within 72 hours and recorded the NIHSS score on admission. The factors associated with death were investigated using a multivariate logistic regression analysis.
Results Twenty-three patients (18.85%) died during hospitalization. The frequency of atrial fibrillation (AF), the NIHSS score on admission (8.69±4.87 in the survival group vs. 14.48±2.54 in the deceased group, p<0.001) and the plasma NT-pro-BNP level (median: 926.30 pg/mL in the survival group vs. 3,280 pg/mL in the deceased group, p<0.001; Lg NT-pro-BNP 2.82±0.66 in the survival group vs. 3.46±0.52 in the deceased group, p<0.001) were each significantly higher in the deceased group than in the survival group. The optimal cut-off levels for the NT-pro-BNP level and NIHSS score to distinguish the deceased group from the survival group were 1,583.50 pg/mL and 12.5, respectively. Patients with both elevated NT-pro-BNP levels (>1,583.50 pg/mL) and NIHSS scores on admission (NIHSS >12.5) had a substantially higher mortality rate than those without elevated NT-pro-BNP levels and NIHSS scores (89.47% vs. 9.84%, p<0.001). A multivariate logistic regression analysis demonstrated that a NT-pro-BNP level >1,583.50 pg/mL (OR, 5.001; 95% CI, 1.233 to 20.287, p=0.024) and a NIHSS score >12.5 (OR, 1.465; 95% CI, 1.191 to 1.801, p<0.001) were each independent factors associated with in-hospital death.
Conclusion The plasma NT-pro-BNP level and the NIHSS score added independent and incremental contributions to the prognostic stratification of patients with acute ischemic stroke.

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A 69-year-old man presented with multiple nodules on the spleen and liver that had been detected by computed tomography (CT). A liver tumor biopsy was performed, and the patient was diagnosed to have histiocytic sarcoma (HS). Splenectomy was performed, and the chemosensitivity of the spleen tumor was measured using the collagen gel droplet-embedded culture drug sensitivity test (CD-DST). Hepatic arterial infusion chemotherapy was administered, based on the results of the CD-DST. The patient achieved complete remission (CR) after this therapy and remained alive without recurrence at the final follow-up. This is the first known case of CR in response to chemotherapy for advanced splenic HS.

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A 69-year old man came to our hospital complaining of abdominal pain. Contrast-enhanced computed tomography (CT) showed a 65-mm ruptured mass in Couinaud segment 5 of the liver. The mass was treated with emergent transcatheter arterial embolization (TAE), followed by partial hepatectomy. Microscopically, the mass was determined to be an angiosarcoma. Six months previously, enhanced CT had shown a 15-mm mass diagnosed as a cavernous hemangioma in the same region of the liver. Even when the enhancement pattern of a small hepatic mass resembles that of hemangioma, the mass should be reassessed within several months to exclude a diagnosis of hepatic angiosarcoma.

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Primary cardiac angiosarcoma is extremely rare, but it is the most common primary malignant cardiac tumor. We herein present the case of a 51-year-old man who presented with symptoms of acute right heart failure, secondary to pericardial tamponade. Pericardiocentesis showed bloody fluid with negative pathology. Repeat 2-D echocardiography and a trans-esophageal echocardiogram showed a right atrial mass. The patient underwent surgery and adjuvant chemotherapy, but died seven months after the diagnosis. Despite being rare, cardiac angiosarcoma should be included in the differential diagnosis of bloody pericardial effusions, even with negative early investigations. The prognosis of the disease is usually poor. Treatment is mainly surgical resection if the cancer is localized, and can include neadjuvant and adjuvant chemotherapy, radiation treatment, and immunotherapy.

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A 67-year-old woman was admitted for severe abdominal pain (stomach ache). Computed tomography (CT) revealed gas along the abdominal aortic wall. A blood culture was positive for Salmonella dublin, a gram-negative bacillus that is rare in humans. Treatment with an antibiotic improved the inflammatory signs; however, on the 11th hospital day, the patient complained of sudden severe abdominal pain. Enhanced CT revealed a pseudoaneurysm surrounded by a periaortic abscess. The infected aortic wall, including the aneurysm, was resected and an extra-anatomic bypass was constructed between the axillary artery and the external iliac arteries. The patient recovered fully and her course has been uneventful for the past two years since her discharge.

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A 77-year-old man previously treated with maintenance hemodialysis was admitted due to appetite loss, nausea and shortness of breath. He showed progressive heart failure and eosinophilia without any basal disorders and was diagnosed with idiopathic hypereosinophilic syndrome (HES) accompanied by eosinophilic myocarditis. Laboratory data revealed hypercalcemia, a low serum parathyroid hormone level and a high 1,25(OH)₂D concentration in spite of renal failure and no causal medications. Steroid therapy resulted in the patient's rapid recovery from heart failure, hypereosinophilia and hypercalcemia. Since the serum 1,25(OH)₂D level promptly and markedly decreased, the hypercalcemia complicated with HES was most likely caused by extrarenal production of 1,25(OH)₂D.

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A 68-year-old woman with Alzheimer's disease developed renal dysfunction after starting carbamazepine for epilepsy. Although Ga-67 citrate scintigraphy strongly suggested interstitial nephritis, renal biopsy was not possible due to her overall state. At 61 days after admission, she died of unexplained shock. At autopsy, severe infiltration of T lymphocytes was noted, not only in the renal interstitium but also in the liver, lungs, and adrenal glands. Adrenal failure was a possible cause of shock. In carbamazepine-induced interstitial nephritis, multiple organ involvement including fatal adrenalitis should be considered.

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A 26-year-old man presented at our hospital in 2008 to undergo detailed investigations as part of a routine health examination. Chest computed tomography (CT) showed linear and reticular opacities with, in part, diffuse calcification in the lung fields bilaterally. A surgical lung biopsy was performed and the histological findings were compatible with a diagnosis of diffuse pulmonary ossification (DPO) of the dendriform type. DPO usually occurs as a secondary disease. As the histological changes in interstitial fibrosis were minimal rather than diffuse and not significant enough to be regarded as interstitial pneumonia, we considered this to be an idiopathic case. However, the findings appear to suggest that inflammation and fibrosis were associated with ossification.

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We herein present the first case of pulmonary actinomycosis caused by Actinomyces cardiffensis (A. cardiffensis). A computed tomography (CT) examination revealed a nodule with cavitation in the left upper lobe of the lung. One month later, the lesion had almost disappeared, but a new nodule with peripheral consolidation had appeared in the right middle lobe. Because organizing pneumonia was suspected, prednisolone was begun and improvement was seen. However, two months after the initiation of corticosteroid administration, a chest CT scan showed a lung abscess. The patient underwent surgical resection of the abscess. A. cardiffensis was identified by an amplified 16S ribosomal DNA restriction analysis of a pus sample.

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We herein report a case of cytomegalovirus (CMV) bronchitis in a 62-year-old woman with polymyositis. She presented with respiratory symptoms and CMV antigenemia while undergoing immunosuppressive therapy with methotrexate (MTX) and prednisolone (PSL). Bronchoscopy was performed, which revealed an ulceration of the left main bronchus. A mucosal biopsy confirmed CMV infection, and the patient was diagnosed with CMV ulcerating bronchitis. The administration of ganciclovir improved the lesion, and the CMV antigenemia disappeared. Endobronchial ulceration should be considered in the differential diagnosis of CMV disease.

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The prognosis of patients who relapse with acute myeloid leukemia (AML) after undergoing stem cell transplantation (SCT) is poor. There exist some treatments for relapsed AML; however, almost all treatments are associated with a high level of regimen-related toxicities (RRTs). The RRT of donor lymphocyte infusion is lower than that of other treatments; however, the efficacy of this treatment in treating patients with relapsed AML is lower than that observed in patients with chronic myelomonocytic leukemia. We herein report a case of relapsed AML after SCT in a 65-year-old man. We performed donor lymphocyte infusion; however, it was not effective. We then administered chemotherapy with cytosine arabinoside and macrophage colony-stimulating factor/granulocyte colony-stimulating factor and complete remission was achieved. Since graft-versus-host disease occurred after the administration of low-dose chemotherapy in this case, we speculated that the chemotherapy induced a graft-versus-leukemia effect.

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A 22-year-old man with acute lymphoblastic leukemia underwent allogeneic stem cell transplantation (allo-SCT) twice, then underwent allo-SCT a third time due to relapse. On day 27, he developed acute respiratory distress, and bilateral interstitial infiltrates were noted on CT images. Despite receiving intensive treatment, the patient died on day 32 from progressive respiratory failure. An autopsy revealed evidence of diffuse alveolar damage caused by the genus Toxoplasma. At present, toxoplasmosis is considered to be a rare infectious complication in Japan. However, the actual incidence of toxoplasmosis may be higher than currently believed due to a lack of suspicion of the diagnosis in patients, difficulty in making a diagnosis and low autopsy rates.

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A 76-year-old man presented with a two-month history of angina pectoris. Computed tomography (CT) revealed a serial enlargement of the supraclavicular and mediastinal lymph nodes compressing the heart, pulmonary artery and aorta. CT angiography (CTA) showed stenosis of the coronary arteries as a result of compression by the enlarged lymph nodes. First-pass contrast-enhanced cardiac magnetic resonance imaging (MRI) at rest revealed a perfusion defect, thus indicating myocardial ischemia. Diffuse large B-cell lymphoma was diagnosed and multidrug combination chemotherapy led to prompt improvement of the symptoms. Relief of the stenosis in the coronary arteries and improvements in myocardial perfusion were noted on follow-up CTA and MRI.

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Spinocerebellar ataxia type 7 (SCA7) is a neurodegenerative disorder characterized by degeneration of the cerebellum, brainstem and retina. We herein describe a family from southern Italy whose proband was a 49-year-old man presenting with ataxia with progressive gait disturbances, clumsiness and visual impairment. A molecular analysis identified 38 cytosine-adenine-guanine (CAG) repeat expansions within the SCA7 gene. Our study confirms the marked anticipation previously observed in SCA7 and extends the small number of patients studied thus far. In this family, the disease is most likely caused by a de novo expansion of a premutated intermediate allele carried by one parent.

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A 23-year-old woman was admitted with complaints of swelling and pain in the left breast, fever, polyarthralgia and erythema nodosum. A fine-needle biopsy of the mass in the left breast revealed non-caseous granulomatous lobulitis. A diagnosis of granulomatous mastitis was thus made. The administration of prednisolone 40 mg/day resulted in the resolution of the patient's symptoms, and the breast mass thereafter decreased in size. The mass relapsed during the subsequent prednisolone taper. Additional therapy with methotrexate resulted in complete remission. Granulomatous mastitis should therefore be included in the differential diagnosis of polyarthralgia.

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Multifocal skeletal tuberculosis is a very rare manifestation of tuberculous infection. The multiple bone lesions of multifocal skeletal tuberculosis are difficult to differentiate from metastasis, even when performing 2-[fluorine-18]-fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (FDG PET/CT) or magnetic resonance imaging (MRI). A 25-year-old man presented with an abnormal chest X-ray. Radiologically, there were extensive osteolytic lesions on the skull, along the entire spine and on the ribs and both iliac bones, suggesting a diagnosis of bone metastasis. On FDG PET/CT, intensely increased F-18 FDG lesions were observed. A bone biopsy with a microbiologic study revealed a tuberculous infection. Follow-up PET/CT performed after treatment showed marked improvement in the extensive FDG uptake lesions.

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Temozolomide (TMZ) is the standard chemotherapy treatment for glioblastoma. Lymphocytopenia is reported to be the most frequent and severe adverse effect of TMZ and leads to opportunistic infections. Few cases of TMZ-induced cytomegalovirus (CMV) reactivation have so far been reported, and there are no guidelines regarding the use of chemotherapy after recovery from CMV reactivation. We herein report the case of a 45-year-old man with glioblastoma who developed CMV hepatitis following surgery and chemoradiotherapy with concomitant TMZ and steroids. After successful treatment of the CMV infection with an antiviral agent and recovery from the lymphocytopenia were achieved, the patient resumed maintenance therapy with TMZ under careful monitoring of his lymphocyte count and CMV pp65 antigenemia level.

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A 21-year-old Japanese man with a history of marked body weight loss over a short period of time died of necrotizing duodenitis caused by Clostridium perfringens (C. perfringens) type A. C. perfringens type A is considered to usually cause self-limiting gastroenteritis. Necrotizing enteritis sometimes occurs due to C. perfringens in developing countries; however, it is primarily caused by the type C strain and its site of onset is typically the jejunum or ileum. This is a rare case of necrotizing duodenitis caused by C. perfringens type A in a Japanese young man. Physicians need to be more aware of this emerging fatal disease in developed countries.

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A 60-year-old woman was admitted to our hospital with a two day history of truncal ataxia and diplopia. Three days after admission, complete paresis of eye movements, left ptosis, taste impairment and absence of deep tendon reflexes appeared. The patient displayed normal facial movements; however, she reported decreased sensations of sweet and salty tastes. Anti-GQ1b antibodies were detected in the serum, and Fisher syndrome was therefore diagnosed. Intravenous immunoglobulin was administered starting five days after admission, with limitations of eye movements, areflexia and taste impairment showing improvements by 12 days after onset. Taste disturbance is rare in patients with Fisher syndrome. In this case, we hypothesize that autoantibodies may have targeted antigens in the chorda tympani, glossopharyngeal nerve or taste buds.

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