Objective To investigate the clinical factors, including intravascular ultrasound (IVUS) findings, associated with percutaneous coronary intervention (PCI)-related periprocedural myonecrosis (PM) in patients with stable coronary artery disease (CAD).
Methods The study included 46 patients (mean age 65±2 y, 37 men) with stable CAD who underwent elective PCI using IVUS and a Doppler flow guidewire. In addition to routine IVUS measurements, the presence of deep echo attenuation (DEA) without calcification was assessed. The sum of all high-intensity transient signals (HITS) after a balloon deflation procedure was calculated using a Doppler flow guidewire. PM was defined as an elevation in troponin T to >0.03 ng/mL 24-hour after PCI. Patients were divided into 2 groups on the basis of the presence or absence of PM.
Results PM was identified in 17 patients (37%). Estimated glomerular filtration ratio was lower in the PM group (p=0.021). Target vessel distribution was different between the groups (p=0.004). Positive remodeling and DEA, as observed on IVUS, were more common in the PM group (p=0.03 and p<0.0001, respectively). The total number of HITS was higher in the PM group (p=0.003). Logistic regression analysis demonstrated that the presence of DEA (p=0.003) was the sole factor associated with the occurrence of PM.
Conclusion These results demonstrated that DEA is an important factor associated with PM in patients who underwent an elective PCI. Therefore, IVUS to determine the presence of DEA should be performed before PCI.

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Objective The associations of insulin resistance and glycemic control with the risk of kidney stones were explored.
Methods Generally healthy Japanese (n=2,171) who visited Saiseikai Central Hospital (Tokyo, Japan) for a health check were included in a cross-sectional study. We calculated odds ratios (OR) of having kidney stones in terms of four measures: fasting serum insulin, homeostasis model assessment of insulin resistance (HOMA-IR), fasting plasma glucose (FPG) and glycosylated hemoglobin (HbA1c), adjusting for possible risk factors for kidney stones.
Results Fasting serum insulin and HOMA-IR were non-significantly associated with the risk of kidney stones, whereas FPG and HbA1c were significantly associated. Compared with those with an FPG of <100 mg/dL, the ORs in those with an FPG of 100 to <126 mg/dL and ≥126 mg/dL were 1.38 (95% confidence interval [CI] =0.95-2.00) and 1.83 (95% CI =1.09-3.06) (p for trend =0.016). In relation to those with an HbA1c of <5.5%, the ORs in those with an HbA1c of 5.5 to <6.0%, 6.0% to <6.5% and ≥6.5% were 1.16 (95% CI =0.76-1.79), 1.25 (95% CI =0.70-2.23) and 1.98 (95% CI =1.11-3.52), respectively (p for trend =0.027). The significant associations between glycemic control measures and the risk of kidney stones were preserved even after the adjustment for factors related to insulin resistance.
Conclusion Glycemic control could be an independent risk factor for kidney stones.

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Objective The incidence of malignant pleural mesothelioma (MPM) in Japan is predicted to increase over the next few decades. Prognostic factors remain unclear although several studies have reported this disease. In this study, we examined the prognostic factors of MPM from single institution practice data and tested the scoring systems of past reports.
Methods We retrospectively obtained clinical data from the medical records of patients who were diagnosed with MPM from 1991 to 2010. The European Organization for Research and Treatment of Cancer prognostic score (EPS) was calculated.
Results We surveyed the records of 68 patients. Univariate analysis showed that significant prognostic factors were histological type, stage, performance status (PS), chemotherapy, and lactate dehydrogenase (LD). Multivariate analysis identified stage, PS and LD as independent prognostic factors. Low-risk group (EPS ≤1.27) survival was significantly better than that of the high-risk group (EPS >1.27) (17.0 months vs. 8.0 months; p=0.002).
Conclusion Stage, PS and LD were demonstrated to be independent prognostic factors. An EPS >1.27 was still considered a poor prognosis indicator in the practice data of MPM.

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Objective Respiratory muscle weakness causes alveolar hypoventilation and reduced lung compliance in neuromuscular disorders. Lung inflation is important to prevent secondary pulmonary complications however respiratory and laryngeal dysfunction often hamper lung inflation. There is a need for a convenient and low-cost device that enables effective lung inflation. We tested a lung inflation training method using a positive end-expiratory pressure (PEEP) valve.
Methods Vital capacity (VC), maximum insufflation capacity (MIC) and peak cough flow (PCF) as well as PEEP lung inflation capacity (PIC) were assessed in 93 neuromuscular patients. Consecutive PIC training was done for 4 months in six tracheostomized Duchenne muscular dystrophy (DMD) patients and PIC was assessed before and after training.
Results PIC training was practicable in all participants and no serious adverse events were detected. PIC was significantly higher than VC or MIC in all disorders, although MIC was higher than VC in DMD only. Patients with dysphagia showed lower MIC and PCF compared with non dysphagic patients. PIC was as low as 345±77 mL in tracheostomized DMD patients; however consecutive training increased it up to 619±205 mL.
Conclusion The PEEP valve enabled effective lung inflation regardless of laryngeal function. Consecutive training can improve lung condition even in advanced cases. Early introduction of PIC training could be effective at preventing respiratory complications in patients with neuromuscular disorders.

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Background and Aim Eosinophilic phenotype, sputum eosinophil count ≥3%, of asthma is a valuable clinical parameter. However, measurement of sputum inflammatory cells is time-consuming and requires specialized personnel. A simpler surrogate, such as peripheral eosinophil count, would be beneficial. The aim of this study was to assess the value of peripheral eosinophil count for predicting the eosinophilic phenotype of adult asthmatics.
Materials and Methods This retrospective study was conducted in 192 adult asthmatics. Sputum and venous blood were collected at the same visit and analyzed for cell differential. Receiver-operating characteristic (ROC) curve analysis was used to assess the relationship between eosinophilic phenotype and peripheral eosinophil count.
Results Peripheral eosinophil count, derived from the ROC curve, of more than 210 cells/mm³ yielded 67.7% sensitivity and 66.2% specificity [area under the curve (AUC)=0.698, p=0.0001] for identifying the eosinophilic phenotype in the total patients. Multiple regression analysis revealed that steroid treatment and atopy significantly influenced the peripheral eosinophil count. Within the steroid-naïve subgroup, a peripheral eosinophil count of >190 cells/mm³ yielded 76.3% sensitivity and 67.4% specificity (AUC=0.730, p<0.0001) for identifying eosinophilic phenotype, while in the steroid treated subgroup, the AUC was 0.554 (p=0.4985). Within the steroid-naïve non-atopic subgroup, a peripheral eosinophil count of >190 cells/mm³ yielded 73.1% sensitivity and 79.3% specificity (AUC=0.809, p=0.0001) for identifying the eosinophilic phenotype, while in the steroid-naïve atopic subgroup, the AUC was 0.588 (p=0.2723).
Conclusion Peripheral eosinophil count is able to identify the presence of eosinophilic phenotype in steroid-naïve non-atopic adult asthmatics with moderate accuracy.

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A liver neoplasm was found in a 63-year-old man with alcoholic liver disease. Sonazoid-enhanced ultrasonography (US) showed that the neoplasm was isoechoic at the early vascular phase and hypoechoic at the post-vascular phase. Gadolinium ethoxybenzyl-diethylenetriamine-enhanced magnetic resonance imaging (MRI) showed that the neoplasm was hypointense at the hepatobiliary phase. We suspected that it was a malignant tumor. By needle biopsy, however, the neoplasm was diagnosed as an inflammatory pseudotumor (IPT). We encountered a rare case of hepatic IPT, the differential diagnosis of which was difficult to distinguish from malignant tumor. Here, we report new US and MRI findings of hepatic IPT.

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Poorly differentiated neuroendocrine carcinoma is a very rare malignancy, but it is characterized by agressive histological features and a poor clinical prognosis. We report a 42-year-old man who had poorly differentiated neuroendocrine carcinoma of the pancreas with multiple liver metastases. We administrated combined chemotherapy with S-1 and gemcitabine. This treatment was efficacious and well tolerated, and then this patient obtained objective partial response for 7 months and survived for 13 months after the diagnosis. This case suggests that S-1 and gemcitabine combination produce beneficial responses for patients with this disease.

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Recent histological and clinical studies have suggested the existence of 2 distinct types of autoimmune pancreatitis (AIP): type 1 AIP related to IgG4, exhibiting lymphoplasmacytic sclerosing pancreatitis (LPSP), and type 2 AIP related to granulocyte epithelial lesions (GELs), exhibiting idiopathic duct-centric chronic pancreatitis (IDCP). We herein present a case of type 1 AIP with histologically proven LPSP with GELs. This patient had neither serum IgG4 elevation nor MPD narrowing. In this case, the clinically and histologically atypical findings for type 1 AIP are intriguing.

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A 52-year-old woman with Takayasu arteritis developed acute coronary syndrome and received percutaneous coronary intervention (PCI). The patient experienced restenosis three times even with drug-eluting stent (DES) implantation. We started steroid administration after the fourth PCI to reduce inflammation due to autoimmunity. With DES and a steroid combination, the patient remained free of chest pain, and a follow-up angiography demonstrated good patency of the stent site. Since in-stent restenosis may result from a complicated combination of neointimal proliferation and autoimmune mechanisms, physicians should consider a combination of DES and a steroid for the treatment of coronary artery disease in Takayasu arteritis.

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Treatment for a 51-year-old man with pulmonary tuberculosis was initiated with isoniazid, rifampicin, ethambutol, and pyrazinamide, and discontinued after 3 weeks because of skin rash and itchiness. Isoniazid monotherapy was restarted after 2 weeks. Two days later, the platelet count decreased from 150 to 4×10³/mm³. Despite platelet transfusion and oral corticosteroid treatment, the platelet count did not recover. However, after a 2-day administration of intravenous immunoglobulin, the platelet count recovered to 209×10³/mm³. This report describes a case of isoniazid-induced thrombocytopenia that was reversed with intravenous immunoglobulin, thus highlighting the efficacy of this treatment for this rare condition.

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A 62-year-old man with hypertrophic obstructive cardiomyopathy (HOCM) had complete atrioventricular (AV) block and subsequent cardiac standstill. A previous electrocardiogram revealed a bifascicular block pattern. Because he also suffered from infective endocarditis of the native aortic valve, surgical therapy (dual-chamber permanent pacing, myectomy of the left ventricular outflow tract, and valve replacement) was performed. Complete AV block unrelated to a procedure is a rare complication in patients with HOCM, but it may be life-threatening. Therefore, a pre-existing cardiac conduction disturbance should be specifically recognized as the aura of a higher degree of AV block.

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A 55-year-old man showed a serum creatinine level of 1.51 mg/dL, CRP of 0.79 mg/dL, and proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) of 43.9 EU (normal range: below 10). The serum levels and ratios of IgG1, IgG2, IgG3, and IgG4 to total IgG were 1,570 mg/dL (49%), 1,190 mg/dL (37%), 82 mg/dL (3%), and 351 mg/dL (11%), respectively. Positron emission tomography and CT with ¹⁸F-fluorodeoxyglucose (PET-CT) demonstrated retroperitoneal fibrosis. After a diagnosis of IgG4-related retroperitoneal fibrosis with PR3-ANCA was made, oral prednisolone improved serum creatinine and the titer of PR3-ANCA to normal levels, with no abnormal findings on PET-CT.

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We present two cases with steroid-resistant nephrotic syndrome (SRNS) and two cases with steroid-dependent nephrotic syndrome (SDNS) due to focal segmental glomerulonephritis (FSGS) who were treated with a single dose of rituximab (375 mg/m²). Although the two cases with SRNS showed no response, the two cases with SDNS achieved complete remission. The patients in whom the peripheral B-cell counts subsequently increased after the administration of rituximab demonstrated a relapse. Rituximab may be an effective treatment agent for SDNS with FSGS and the peripheral B-cell count may be a useful marker in such patients for preventing disease relapse.

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We report a case of long-standing sinobronchial syndrome complicated by microscopic polyangiitis (MPA) during the clinical course. The patient showed a mild elevation of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) 17 months prior to the diagnosis of MPA. Subsequently, her MPO-ANCA level gradually became more elevated, and finally her MPO-ANCA level peaked when purpura appeared. Histologic examination of the skin biopsy was consistent with leukocytoclastic vasculitis. Based on the pathological and clinical findings, a diagnosis of MPA was made. Corticosteroid therapy finally led to a remission of MPA with normalized MPO-ANCA titers.

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Myeloid sarcoma (MS) is a tumor consisting of myeloid blasts that occurs at an anatomical site other than the bone marrow. We report the case of a 38-year-old man with duodenal MS who underwent an allogeneic bone marrow transplant in a non-complete remission (CR) state. After the transplant, residual disease was suspected on a fluorodeoxyglucose positron emission tomography (FDG-PET)/CT scan, and additional radiotherapy resulted in CR, which has been maintained for 21 months. FDG-PET/CT scanning is useful for evaluating residual myeloid sarcoma during the peritransplant period.

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Acute liver failure is an unusual complication in multiple myeloma. Here, we report a case of multiple myeloma with light chain deposition disease (LCDD) that presented with progressive jaundice due to intrahepatic cholestasis. Diagnosis was made after liver biopsy that showed deposition of kappa light chains occupying perisinusoidal spaces. The patient developed encephalopathy and liver failure and died despite prompt initiation of dexamethasone therapy. The current prognosis of multiple myeloma patients with liver failure due to LCDD is dismal. New therapeutic strategies might improve this condition.

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We report the case of a 35-year-old woman with chronic active Epstein-Barr virus (EBV) infection (CAEBV). She underwent allogeneic bone marrow transplantation (BMT) from an unrelated male donor and achieved a complete response. However, her CAEBV relapsed one year after BMT. EBV-infected cells proliferated clonally and revealed a 46XY karyotype. In addition, the infecting EBV strain differed from that detected before BMT. These findings indicated that her disease had developed from donor cells. This is the first report of donor cell-derived CAEBV that recurred after transplantation, suggesting that host factors may be responsible for the development of this disease.

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Japanese spotted fever (JSF), first reported in 1984, is a rickettsial disease characterized by high fever, rash, and eschar formation. A 61-year-old man was admitted to a local hospital in Nagasaki City, Japan, after several days of high fever and generalized skin erythema. His condition deteriorated and laboratory findings indicated disseminated intravascular coagulation (DIC). The patient was transferred to our hospital with mental disturbance and status epilepticus. Treatment included minocycline, and new quinolone. Definitive diagnosis was made with a serological test showing increased antibody levels against Rickettsia japonica. Rickettsial infections are rare, but should be seriously considered for the differential diagnosis of aseptic meningitis and encephalitis, as they show no response to conventional antibiotic treatment.

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There have been reports of various etiologies associated with reversible splenial lesions manifested as obviously restricted diffusion. We describe for a 27-year-old woman suffering from late postpartum preeclampsia associated with reversible splenial lesions. This association has not been reported to date. Awareness that profound late postpartum preeclampsia can rarely result in abnormalities of the splenium of the corpus callosum (SCC) may help clinicians avoid unnecessary diagnostic procedures and therapeutic interventions. Moreover, it must be stated that prediction of the clinical course based on DWI and ADC values should be used with caution.

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Hereditary motor sensory neuropathy type VI (HMSN VI) is hereditary neuropathy accompanied by optic neuropathy. The feasibility of Coenzyme Q10 (CoQ10) as a treatment for subacute visual impairment of HMSN VI was examined.
A 37-year-old patient with HMSN VI with a novel mitofusin 2 mutation was treated with high dose of CoQ10 (200 mg/day) for eight months.
Visual impairment was partially resolved after CoQ10 therapy.
High dose CoQ10 therapy may improve the prognosis of subacute visual impairment in HMSN VI. To confirm the effectiveness of CoQ10 on HMSN VI, further studies are needed.

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We herein describe a rare case of meningeal carcinomatosis in a 77-year-old woman who had bilateral deafness as the initial symptom of a previously undetected colon cancer malignancy. Meningeal carcinomatosis should be considered in the differential diagnosis in cases of abrupt-onset sensorineural deafness. Both MRI scans and cerebrospinal fluid evaluation are necessary diagnostic tools, and should be used in conjunction as each of these procedures could have false-negative results. This should apply even when there is no known primary malignancy.

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We describe a patient with dermatomyositis who presented with rapidly developing severe muscle weakness complicated by massive pleural effusion with interstitial lung disease. Myopathological analysis was suggestive of dermatomyositis. This patient showed both anti-Jo-1 and anti-SRP antibodies in serum. To our knowledge, the coexistence of these two myositis-specific autoantibodies (MSA) is considered extremely rare and is clearly an exception to the rule of having only one MSA. Our findings provide compelling evidence that the coexistence of these two MSAs may lead to more severe clinical symptoms, interacting in a complex fashion, thus expanding the clinical spectrum of idiopathic inflammatory myopathy.

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We describe a patient with posterior spinal artery (PSA) syndrome due to vertebral artery (VA) dissection. A 63-year-old woman developed neck pain, bilateral shoulder and arm numbness, and paraparesis after prolonged neck extension during a dental procedure. Neurological examination revealed sensory deficits in the legs, paraparesis, cerebellar ataxia, urinary retention and constipation. Magnetic resonance imaging disclosed T2-hyperintense lesions in the posterolateral C4-C7 cord with partial enhancement. T1-hyperintensity and stenosis were found in the right VA at C3-C5. These clinicoradiological findings suggested bilateral PSA syndrome and unilateral VA dissection. This is the fourth report of VA dissection-induced PSA syndrome.

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Anti-aquaporin-4 (AQP4) antibody is highly specific for neuromyelitis optica (NMO) and NMO spectrum disorder. Brainstem lesions sometimes show involvement in NMO and NMO spectrum disorder, and onset is usually diagnosed in young or middle-aged adults. Here, we report the case of an 87-year-old woman with recurrent brainstem lesions and subsequent severe longitudinally extensive cervical cord lesions who was found to be positive for anti-AQP4 antibody. In patients with recurrent brainstem lesions, even in the elderly and those with symptoms mimicking infarction, NMO spectrum disorder should be considered as a differential diagnosis.

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Although antiphospholipid antibody syndrome (APS) is an autoimmune condition that is primarily characterized by arterial or venous thrombosis or pregnancy morbidity and the presence of antiphospholipid antibodies (aPL), recent reviews have introduced non-thromboembolic manifestations. We describe the case of a 58-year-old woman with vegetation on the aortic valve, whose initial presentation of APS abruptly developed into diffuse pulmonary hemorrhage. Despite consecutive plasma exchange procedures and the administration of corticosteroids and high-dose intravenous immunoglobulin, multiple brain infarctions developed, and the patient died of pneumonia. Although anecdotal, this case might serve as a useful example of the non-standard complications of fulminant APS.

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