Objective Patients with hepatitis C virus (HCV) cirrhosis and thrombocytopenia are often excluded from receiving interferon therapy because the treatment results in severe platelet depletion. Surgical splenectomy or partial splenic embolization (PSE) is a promising procedure for increasing the platelet count before interferon therapy. We performed PSE and evaluated the long-term clinical course in HCV cirrhotic patients.
Methods Patients with HCV cirrhosis and thrombocytopenia were included (n=108) in this study. The straight-coiled PSE procedure (Takatsuka method) was performed. The platelet count, hemodynamic changes, rate of a sustained virological response (SVR) and prevalence of hepatocellular carcinoma (HCC) were evaluated.
Results PSE resulted in a significant increase in the platelet count (before PSE: 7.9±2.3×10⁴/μL, two weeks after PSE: 16.7±6.6×10⁴/μL (p<0.001). Therefore, all participants were started on regular-dose interferon therapy. The SVR rate was 24% for serotype 1 and 62% for serotype 2. In the biochemical responders (BR) with SVR, the overall survival rate was 94.6% over five years and 89.3% over 10 years. In the non-responders (NR), the overall survival rate was 78.7% over five years and 62.2% over 10 years. The overall survival rate of the patients with SVR+BR was significantly higher than that observed in the patients with NR (p=0.0082). There were no differences in the prevalence of HCC between the patients with SVR+BR and NR.
Conclusion PSE enabled the induction of regular-dose interferon therapy in patients with HCV cirrhosis and thrombocytopenia. Although the prevalence of HCC did not differ between the SVR+BR and NR patients, there was a significant survival benefit in the patients with SVR+BR.

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Objective The predictors of in-hospital outcomes after primary percutaneous coronary intervention (PCI) for acute myocardial infarction (AMI) complicated with heart failure or cardiogenic shock at presentation remain unclear.
Methods Using the AMI-Kyoto Multi-Center Risk Study database, the clinical background characteristics, angiographic findings, primary PCI results, and in-hospital prognoses were retrospectively compared between primary PCI-treated AMI patients with a Killip class status of ≥2 (Killip 2-4 patients, n=390) and those with a Killip class 1 status (Killip 1 patients, n=1,057).
Results The Killip 2-4 patients were more likely to have a higher age and proportion of women and exhibited a higher prevalence of previous myocardial infarction, diabetes mellitus and chronic kidney disease or anemia on admission, lower systolic blood pressure (SBP) values on admission, a higher rate of multivessels or left main trunk as the culprit artery, a larger number of diseased vessels, a lower Thrombolysis In Myocardial Infarction (TIMI) grade in the infarct-related artery (IRA) before/after primary PCI and a significantly higher in-hospital mortality rate than the Killip 1 patients. According to a multivariate analysis, age was found to be an independent positive predictor of in-hospital mortality, while admission SBP was an independent positive predictor of in-hospital survival in both groups. In contrast, anemia on admission was found to be an independent predictor of in-hospital death, while the TIMI 3 flow in the IRA after PCI was found to be an independent factor for survival in the Killip 2-4 patients, but not the Killip 1 patients.
Conclusion Anemia on admission and the final TIMI 3 flow in the IRA are critical determinants of in-hospital death in AMI patients with a Killip class status of ≥2 undergoing primary PCI.

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Objective To examine whether aging itself affects insulin resistance (IR) and insulin secretion in the general Japanese population.
Methods This study included data for 2,324 men and 1,472 women 30-79 years of age without diabetes who participated in a general health checkup program. The effects of age on homeostasis model assessment of insulin resistance (HOMA-IR) and homeostasis model assessment of β-cell function (HOMA-B) were examined using a linear regression model. Logistic regression models were used to identify clinical characteristics related to age. Multiple regression analyses using a stepwise form were performed to examine factors, including age, possibly affecting HOMA-IR and HOMA-B.
Results The fasting plasma glucose (FPG) levels increased with age in both sexes, whereas the body mass index (BMI) and HOMA-B decreased with increasing age in men and the BMI increased with age in women. The HOMA-IR increased with increasing age in postmenopausal women. The proportion of physically active subjects increased in both sexes, and the proportion of current smokers decreased with age in men and postmenopausal women. The proportion of regular drinkers increased, and the proportion of subjects with a family history of diabetes decreased with age in men. The stepwise multiple regression analysis showed that age was inversely associated with the HOMA-B in both sexes and positively associated with the HOMA-IR in men and postmenopausal women. However, the BMI had the largest impact on the HOMA-IR and HOMA-B in both sexes.
Conclusion Age-related increases in the glucose levels in Japanese men and women may be associated with impaired insulin secretion. As a result, a sex difference was observed regarding the impact of aging on IR.

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Objective Kakkonto, a Japanese herbal medicine, is frequently used to treat the common cold not only with a physician's prescription, but also in self-medication situations. This study aimed to examine whether Kakkonto prevents the aggravation of cold symptoms if taken at an early stage of illness compared with a well-selected Western-style multiple cold medicine.
Methods This study was a multicenter, active drug-controlled, randomized trial. Adults 18 to 65 years of age who felt a touch of cold symptoms and visited 15 outpatient healthcare facilities within 48 hours of symptoms onset were enrolled. The participants were randomly assigned to two groups: one treated with Kakkonto (Kakkonto Extract-A, 6 g/day) (n=209) and one treated with a Western-style multiple cold medicine (Pabron Gold-A, 3.6 g/day) (n=198) for at most four days. The primary outcome of this study was the aggravation of cold, nasal, throat or bronchial symptoms, scored as moderate or severe and lasting for at least two days within five days after entry into the study.
Results Among the 410 enrollees, 340 (168 in the Kakkonto group and 172 in the Pabron group) were included in the analyses. The proportion of participants whose colds were aggravated was 22.6% in the Kakkonto group and 25.0% in the Pabron group (p=0.66). The overall severity of the cold symptoms was not significantly different between the groups. No harmful adverse events occurred in either group.
Conclusion Kakkonto did not significantly prevent the progression of cold symptoms, even when prescribed at an early stage of the disease.

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In Japan, which ranks first among developed countries in the incidence of tuberculosis, intestinal tuberculosis should be regarded as an important disease. However, few studies of this condition have recently been reported. We analyzed nine patients treated at our hospital during and after 2000. Our results differ from those of previous studies in that many of the patients were healthy, not elderly, had primary intestinal tuberculosis and presented with non-gastrointestinal symptoms. All seven patients tested were found to be whole blood interferon gamma release assay (IGRA)-positive. These results suggest that IGRA is useful as an adjunct method for diagnosing intestinal tuberculosis.

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A 41-year-old man was admitted with decompensated heart failure. Mechanical ventilation was maintained with a large dose of propofol. On day 4, significant ST elevation with complete atrioventricular block was noted, which subsequently induced cardiopulmonary arrest. Treatment with percutaneous cardiopulmonary support and therapeutic hypothermia was initiated. Emergent cardiac angiography showed simultaneous multivessel coronary spasms. Although nitroglycerin and nicorandil were ineffective, the intracoronary administration of fasudil, a Rho-kinase inhibitor, successfully resolved the vasospasms. However, during rewarming, the coronary vasospasms recurred, and the patient died of cardiogenic shock. In addition to hypertrophy, the autopsied heart demonstrated the accumulation of inflammatory cells in the pericardium and adventitia of the coronary arteries.

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We herein report a case of infective endocarditis associated with mitral valve prolapse (MVP) in a 34-year-old man with Klinefelter syndrome. The patient was admitted with a fever and headache that had persisted for three weeks. Repeated blood cultures showed growth of Streptococcus oralis. Echocardiography demonstrated severe mitral regurgitation with a large vegetation attached to the prolapsed anterior leaflet. Surgical plasty of the mitral valve was performed because the vegetation measured over 10 mm in diameter and there was a risk of recurrence of embolic complications. This case demonstrates the link between MVP and Klinefelter syndrome and highlights the importance of performing cardiovascular screening and preventing endocarditis.

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A 20-year-old Japanese woman with systemic sclerosis was evaluated for the progressive aggravation of chest pain, palpitations and dyspnea. Cardiac magnetic resonance imaging revealed diffuse wall thinning, segmental dyskinesis and late gadolinium enhancement of the right ventricular (RV) myocardium. Cardiac catheterization demonstrated no pulmonary hypertension. 24-hour electrocardiography monitoring showed non-sustained ventricular tachycardia (VT). Pulseless VT was induced via programmed ventricular stimulation. An endomyocardial biopsy of the right side of the interventricular septum was performed, the histological specimen of which demonstrated massive myocardial atrophy and fibro-fatty replacement with predominant fibrotic changes. The patient was ultimately diagnosed with arrhythmogenic RV cardiomyopathy.

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A 22-year-old woman presented to us with seizures of a few minutes duration. She had clinical features of Albright hereditary osteodystrophy (AHO), including hypocalcemia, hyperphosphatemia and resistance to parathyroid hormone. Genetic testing revealed a sporadic form of pseudohypoparathyroidism type Ib (PHP-Ib). This is the first Japanese case involving overlap between pseudohypoparathyroidism type Ia (PHP Ia) associated with AHO and PHP Ib. It is important to perform both DNA sequencing and methylation status analyses in cases of suspected PHP in patients with signs of AHO.

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We herein report two cases involving a mother and daughter who presented with clinical features of Cushing's syndrome (CS) at 50 and 29 years of age, respectively, and were both found to have adrenocorticotropic hormone-independent adrenal adenoma. Furthermore, a new adenoma was detected in the contralateral adrenal gland in the mother 10 years after surgical treatment, when she presented with subclinical CS. The pathogenesis of this disorder, including the presence of unknown genetic abnormalities causing hereditary CS, is currently poorly understood. In this report, we describe our experience with and consider the pathophysiological implications of two rare and very interesting cases of familial CS.

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Thyroid dysfunction is related to many kidney diseases. We herein present the case of a 39-year-old woman who exhibited obesity-related glomerulopathy-like pathologic features in combination with hyperthyroidism. She displayed hyperthyroidism in spite of receiving anti-thyroid drug treatment, with massive proteinuria (4.5 g/gCr). A renal biopsy demonstrated glomerular hypertrophy (average diameter, 280 μm) and an increased number of capillary vessels, both of which are compatible with a diagnosis of obesity-related glomerulopathy. Following thyroidectomy, the proteinuria gradually decreased in association with an improvement in hyperphagia and normalization of the thyroid function. Obesity-related nephropathy associated with hyperthyroidism is very rare. In this report, we discuss the relationship between hyperthyroidism and obesity-related glomerulopathy-like pathologic features.

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A 57-year-old woman, who had undergone hysterectomy for uterine myoma 11 years earlier presented with cystic, nodular and cavitary lesions simultaneously visible on computed tomography images of the chest. Histological examinations of both the resected lung and past "myoma" specimens demonstrated that the original uterine tumor was a low-grade endometrial stromal sarcoma (ESS) that had metastasized to the lungs. No previous reports have described the coexistence of cystic, nodular and cavitary lesions with pulmonary metastasis of ESS; however, we successfully correlated the radiologic appearance with the corresponding pathologic findings. Medroxyprogesterone acetate therapy has effectively kept the patient asymptomatic for approximately five years.

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We herein report an 81-year-old woman with Alzheimer's disease (AD) in who donepezil, a cholinesterase inhibitor (ChEI), caused cervical dystonia. The patient had a two-year history of progressive memory disturbance fulfilling the NINCDS-ADRDA criteria for probable AD. Mini-Mental State Examination score was 19/30. The remaining examination was normal. After a single administration of donepezil (5 mg/day) for 10 months, she complained of dropped head. Neurological examination and electrophysiological studies supported a diagnosis of cervical dystonia. Antecollis disappeared completely at 6 weeks after cessation of donepezil. Dystonic posture can occur at various timings of ChEI use. Physicians should pay more attention to rapidly progressive cervical dystonia in ChEI-treated AD patients.

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A 25-year-old woman presented with a fever, headache, vomiting and somnolence. Cranial magnetic resonance imaging (MRI) showed multiple lesions in the cerebellum, brainstem, cerebral cortex and subcortex. Oligoclonal bands were positive in the cerebral spinal fluid (CSF). She experienced a good recovery after steroid treatment. Four months later, she developed right vision loss. Repeated MRI showed multiple cranial lesions different from those involved in the first attack in both size and distribution. An abnormal high signal was also observed in the front and intraorbital regions of the right optic nerve. The patient's vision progressively improved, and she obtained a full recovery following the administration of steroids. A diagnosis of multiphasic disseminated encephalomyelitis manifesting with optic neuritis was made.

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A 78-year-old man was admitted for the treatment of internal carotid artery stenosis. The left internal carotid artery was occluded and stenosis of the right internal carotid artery was progressive. The patient had a history of renal cholesterol embolism associated with percutaneous peripheral intervention. Stenting of the right internal carotid artery was successfully performed via the right brachial artery, and cholesterol embolism was not noted after the procedure. This case suggests that carotid artery stenting (CAS) performed via a brachial approach is less likely to elicit cholesterol embolism than CAS performed via a femoral approach.

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We herein report the case of a 21-year-old woman with refractory adult-onset Still's disease who developed central venous catheter-related methicillin-resistant Staphylococcus aureus sepsis during aggressive immunosuppressive therapy. She subsequently experienced septic pulmonary embolism (SPE) and sacroiliitis during treatment with intravenous vancomycin and was successfully treated with long-term oral linezolid therapy. This case suggests that the occurrence of methicillin-resistant Staphylococcus aureus infection in immunosuppressive patients can trigger severe clinical manifestations such as SPE and septic sacroiliitis and that linezolid is suitable for treating such conditions.

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A 73-year-old woman with breast cancer and metastasis under chemotherapy suffered from fever, pleural effusion and pericardial effusion. Despite the administration of treatment with cefozopran and prednisolone, the patient's fever relapsed. An electrocardiogram identified a new complete atrioventricular block and an echocardiogram revealed vegetation with an unusual pseudotumoral mass in the right atrium. Blood cultures grew Listeria monocytogenes. The patient was eventually diagnosed with right-sided infective endocarditis, which improved following the six-week administration of ampicillin and gentamicin. Homemade yoghurt was suspected to be the cause of infection in this case. Listeria endocarditis is rare; however, physicians should pay more attention to preventing this fatal disease in immunocompromised patients.

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