Objective Differences in microvascular integrity can diversely influence the functional assessment of epicardial coronary artery disease in each patient. We investigated the relevance of the index of microcirculatory resistance (IMR) and fractional flow reserve (FFR) of intermediate coronary lesions.
Methods The IMR and FFR were measured in 67 intermediate coronary lesions of the left anterior descending artery of 67 patients, by using a pressure sensor/thermistor-tipped guidewire.
Results To assess the differences in FFR in relationship to the IMR value, patients were divided into tertile IMR groups as follows: Low-IMR (n=22, IMR 14±3), Mid-IMR (n=23, IMR 21±2), and High-IMR (n=22, IMR 36±10). An analysis of variance showed that the High-IMR group had significantly higher FFR values (0.87±0.07) than the Low-IMR group (0.81±0.08) (p=0.03). Functionally significant lesions with FFR ≤0.8 accounted for 9% of lesions in the High-IMR group, 36% in the Low-IMR group and 22% in the Mid-IMR group (p=0.02). In the multivariate logistic analysis, the IMR value was an independent determinant of FFR ≤0.8 (p=0.03).
Conclusion In patients with a high IMR, intermediate lesions as identified with visual estimation were more frequently functionally insignificant. The IMR can provide additional information in understanding the mismatch between the anatomical and functional severity of intermediate coronary stenosis.

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Objective Air-leak syndrome (ALS) is a life-threatening pulmonary complication following allogeneic bone marrow transplantation (allo-BMT) which is thought to be associated with graft-versus-host disease (GVHD). Recently, it has been reported that pleuroparenchymal fibroelastosis (PPFE) also occurs after allo-BMT and often causes ALS. We sought to extract common features of ALS caused by PPFE after allo-BMT.
Methods The clinical data of patients who developed ALS caused by PPFE after undergoing allo-BMT (ALS-PPFE) between April 1996 and December 2007 at our institution were collected and reviewed retrospectively. The clinical findings, radiological and pathological features and treatment outcomes of ALS-PPFE were assessed.
Results Five patients who developed ALS had histologically proven PPFE (four men, one woman: median age, 37 years). The age of onset of ALS-PPFE was 13 to 109 months (median, 68.8 months) after BMT. Alkylating agents were used as conditioning chemotherapy for BMT in all patients. Only one patient developed chronic GVHD (limited type). The common radiological findings were subpleural thickening and traction bronchiectasis predominantly in the bilateral upper lung fields. The histological pulmonary specimens showed no findings of bronchiolitis obliterans or GVHD. Immunosuppressive therapy was not effective in any of the cases, and all patients died of respiratory failure with or without lung transplantation.
Conclusion ALS-PPFE is an extremely late-onset noninfectious pulmonary complication of allo-BMT. This complication is progressive, resistant to immunosuppressive treatment and has a poor prognosis. No association was found between PPFE and GVHD.

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Objective Many studies on the cost of Parkinson disease (PD) have been published; however, there are limited studies pertaining to this issue in Asia. This study looks to assess the direct medical costs of patients with PD at a university hospital in Japan by calculating the average monthly direct medical costs of PD patients from July to December 2008.
Methods We enrolled 724 consecutive patients (411 women and 313 men) with PD who were registered in Japan's "Specified Disease Treatment Research Program" and obtained data on the total direct medical costs of all patients.
Results Values are reported as the mean (standard deviation). The major finding of the direct medical cost analysis was that the outpatient clinic cost per subject (n=715) was USD 485.74 (376.31) per month. A multivariate analysis revealed that a younger age, the presence of wearing-off, hallucination, and longer disease duration increased the direct medical cost significantly. Disease severity had no influence on the direct medical costs. A longer disease duration was significantly correlated with higher hospitalization costs.
Conclusion The direct medical cost of PD in Japan was found to be similar to that in Western countries. Costs due to productivity loss exceeded the direct costs, and they may be reduced through the better integration of PD patients in the work environment.

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Objective Rheumatoid arthritis (RA) is a chronic inflammatory disease accompanied by periarticular and systemic osteoporosis. Fibroblast growth factor 23 (FGF23), which is mainly produced by osteocytes, circulates to the kidneys and regulates bone metabolism. We herein assessed serum FGF23 and its relationship to inflammation and osteoporosis in patients with RA.
Methods Sixty-one patients with RA were included. Serum concentrations of FGF23 were determined using a sandwich enzyme-linked immunosorbent assay.
Results The mean (± standard deviation) serum FGF23 concentration was 34.9±9.2 (range, 21.0-61.0) pg/mL. The serum FGF23 level was significantly and positively correlated with the erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) levels, disease activity score-28 based on the ESR (DAS-28 ESR) and DAS-28 CRP (r=0.261, p=0.044, r=0.280, p=0.029, r=0.409, p=0.001 and r=0.421, p=0.001, respectively). The serum matrix metalloproteinase-3 level was also significantly and positively correlated with the serum FGF23 level (r=0.331, p=0.015). Concentrations of type I collagen cross-linked N-telopeptide in the serum was significantly correlated with the serum FGF23 level (r=0.272, p=0.034). Neither the bone mineral density in the femoral neck nor lumbar was significantly correlated with the serum FGF23 level. Serum phosphate, calcium, 25-hydroxy vitamin D, and intact parathyroid hormone were not related to the serum FGF23 level.
Conclusion In patients with RA, serum FGF23 is correlated with inflammation, the disease activity of RA, and bone absorption markers. Serum FGF23 may be associated with abnormal bone absorption related to RA inflammation. Further studies are necessary to clarify the mechanism underlying this association.

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Combination chemotherapy of mFOLFOX6 (5-fluorouracil, leucovorin, and oxaliplatin) plus panitumumab, a fully human monoclonal antibody against epidermal growth factor receptor (EGFR), is one of the standard treatments for metastatic colorectal cancer (mCRC) without KRAS mutation. A few reports suggested no need of dose adjustment of cetuximab, a similar chimeric anti-EGFR antibody, in patients with renal impairment. However, panitumumab combined with cytotoxic drugs for hemodialysis patients has not been reported. We herein report a case of a hemodialysis mCRC patient successfully treated with mFOLFOX6 and panitumumab combination therapy.

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Mesenteric panniculitis (MP) is a benign fibroinflammatory process characterized by the presence of fat necrosis, chronic inflammation and fibrosis in the mesentery. Although various causal factors, such as malignancy, chronic inflammatory conditions and autoimmune processes, have been identified, the precise etiology remains unknown. We herein report a rare case of MP accompanying Sjögren's syndrome in which a mass lesion and intestinal stenosis were observed simultaneously. This condition led to ileus, which was effectively treated using prednisolone.

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A 63-year-old man with abdominal discomfort was referred to our hospital. Colonoscopy revealed a hemispherical-shaped protruding cecal mass of approximately 10 mm in size with a normal mucosal surface. Biopsy specimens showed nodules consisting of the proliferation of atypical lymphoid cells. Mucosa-associated lymphoid tissue (MALT) lymphoma was diagnosed based on the histological and immunohistochemical findings. Since upper gastrointestinal endoscopy demonstrated Helicobacter pylori-associated atrophic gastritis, eradication therapy was administered. The cecal mass disappeared completely within three months after triple therapy. Therefore, H. pylori eradication therapy may be a useful treatment option for cecal MALT lymphoma.

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We herein report the rare case of a 76-year-old woman who underwent cholecystectomy with bile duct resection for advanced gallbladder cancer associated with pancreaticobiliary maljunction (PBM) and subsequently developed multiple cancers of the pancreaticobiliary system (the distal bile duct, intrahepatic duct and pancreatic duct) after the operation. We performed conventional endoscopic retrograde cholangiopancreatography (ERCP) using a side-viewing scope to evaluate the masses in the distal bile duct and the pancreatic duct. We also performed ERCP using double-balloon enteroscopy (DBE) to observe the mass in the intrahepatic duct. It was possible to directly observe the lesion using DBE and to perform a biopsy under visual control. All lesions were correctly diagnosed by the combination of ERCP using different endoscopes. The present case suggests that it is necessary to pay close attention (with regard to carcinogenesis) to the whole pancreaticobiliary system in patients with PBM. In addition, the combination of ERCP using DBE and a side-viewing scope may be useful for making a precise diagnosis in patients with altered biliary anatomy who have multiple cancers of the pancreaticobiliary system.

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A 78-year-old man who had been diagnosed with idiopathic thrombocytopenic purpura (ITP) was admitted to our hospital with chest pain, cold sweating and nausea. An electrocardiogram and echocardiogram revealed an ST elevated acute lateral myocardial infarction. He underwent an immediate cardiac catheterization. An occluded left circumflex artery was detected by coronary angiography. Reperfusion was performed successfully by non-slip element balloon angioplasty alone, without stenting, to avoid prolonged dual anti-platelet therapy. In this report we discussed the management strategies of acute myocardial infarction in a patient with concomitant ITP.

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Acute myocardial infarction, hyperhomocysteinemia and pulmonary tuberculosis (PTB) are rare in individuals under the age of 30 years. We herein report the case of a 27-year-old man who presented with intermittent chest pain, elevated homosysteine level, and PTB. The patient was treated successfully with a combination of medications and percutaneous coronary intervention. This uncommon case highlights that homocysteine, folate and B vitamins levels should be regularly evaluated, and that chest X-rays or thoracic computed tomography should be ordered routinely to exclude PTB in patients under the age of 30 years who present acute myocardial infarction and lack the traditional risk factors.

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Renal complications are rare in patients with Prader-Willi syndrome (PWS). We herein experienced a 31-year-old woman with PWS, in whom a renal biopsy showed IgA nephropathy and severe intimal thickening of the interlobular arteries. The patient was admitted to our hospital due to proteinuria and microscopic hematuria after an upper respiratory infection. The occurrence of cardiovascular events has been reported as a cause of death in obese PWS patients. Because chronic kidney disease is generally a risk factor for cardiovascular disease, early detection checkups are essential in obese PWS patients to monitor the possible development of cardiovascular disease.

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A 79-year-old man on continuous ambulatory peritoneal dialysis (CAPD) developed abdominal pain and cloudy peritoneal fluid two days after colonoscopy that revealed multiple diverticula. The white blood cell count was 9,000 cells/μL, C-reactive protein level was 6.86 mg/dL, and the white blood cell count of the peritoneal fluid was 7,800 cells/μL, suggesting acute peritonitis. Empiric therapy consisting of cefazolin and ceftazidime slowly improved the patient's symptoms. The initial microbiological examination of the peritoneal fluid demonstrated Morganella morganii. He was changed from CAPD to hemodialysis. It is important to consider M. morganii peritonitis in patients with colonic diverticula.

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Yellow nail syndrome (YNS) is a rare disorder characterized by the triad of yellow, thickened nails, lymphedema, and respiratory manifestations such as pleural effusions, bronchiectasis, and recurrent lower respiratory tract infections. We report a case of YNS showing pulmonary interlobular septal thickening on thoracic computed tomography, implying the presence of lymphatic edema. The patient showed both the remission and relapse of yellow nail with different lung treatments over a long clinical course.

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This paper describes endobronchial embolization using silicone spigots (EESS), which is a potential treatment option for hemoptysis. A 63-year-old man with massive hemoptysis was treated with EESS to the left B³, and bronchial artery embolization (BAE) was subsequently performed. However, the patient's hemosputum persisted and we performed another bronchoscopy. Bleeding was found from the left B¹⁺². This was also treated with EESS. Subsequently, the patient achieved complete hemostasis with no complications for four months. EESS can prevent suffocation and can be a definitive treatment for achieving hemostasis in patients with recurrent hemoptysis after BAE.

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A 53-year-old woman with chronic myeloid leukemia received allogeneic hematopoietic stem cell transplantation. After neutrophil engraftment, her platelet count exceeded 100,000/μL at day 64. While she was receiving corticosteroid treatment for chronic graft versus host disease (GVHD), her platelets suddenly dropped to 6,000/μL at day 210 and she was diagnosed with immune thrombocytopenia (ITP). Corticosteroids, intravenous high-dose gamma globulin (IVIg) and a splenectomy failed to increase her platelet count. She developed bacterial pneumonia at day 599 and antibiotic therapy was initiated. Soon after, her platelet count continuously increased. Her GVHD and ITP are now in remission without any ongoing treatment.

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Thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome is a unique clinicopathologic variant of multicentric Castleman's disease that has recently been identified in Japan. Previous reports have shown that affected patients typically respond to immunosuppressive therapy, such as prednisolone and tocilizumab. However, the optimal treatment for refractory TAFRO syndrome, which can be fatal, remains unclear. We herein report a case of tocilizumab-resistant TAFRO syndrome successfully treated with cyclosporin A, indicating that cyclosporine A may be an alternative therapy for refractory TAFRO syndrome.

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Lymphocyte-rich classical Hodgkin lymphoma (LRCHL) is a rare subtype of Hodgkin lymphoma with a favorable prognosis, and an aggressive clinical course of LRCHL is uncommon. A 55-year-old man suffering from swelling in the left neck was diagnosed with LRCHL with extranodal lesions in the lung and bone marrow. Initially, he received standard ABVD chemotherapy; however, disease progression, accompanied by hemophagocytic syndrome (HPS), occurred during the second course of ABVD. He received two subsequent courses of intensive chemotherapy containing high-dose steroids, cyclophosphamide, and etoposide. Nevertheless, this therapy was only temporarily effective, and he died of due to an aggressive disease progression accompanied by uncontrollable HPS and severe coagulopathy.

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A 53-year-old man was diagnosed with adult T-cell leukemia-lymphoma (ATL) acute type transformed from chronic type. A bone marrow analysis showed diffuse infiltration of abnormal lymphocytes and diffuse fibrotic change. He received unrelated cord blood transplantation (CBT) following reduced-intensity conditioning with complete remission of ATL after two courses of chemotherapy and achieved neutrophil and platelet engraftment. At 99 days after CBT, a bone marrow biopsy showed apparent resolution of myelofibrosis. These results suggest the therapeutic potential of CBT for patients with chemosensitive ATL with myelofibrosis.

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