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Hirayama K.,Tokyo Medical University | Kobayashi M.,Tokyo Medical University | Usui J.,University of Tsukuba | Arimura Y.,Kyorin University | And 6 more authors.
Nephrology Dialysis Transplantation | Year: 2015

Background Pulmonary involvement is one of the hallmark lesions of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) as well as rapidly progressive glomerulonephritis (RPGN). However, the pulmonary involvement of AAV patients seems to differ between Europe and Japan, as does the ANCA serotype. Methods This retrospective and prospective multicenter cohort study collected the clinical data of the features and outcomes of 1772 RPGN patients treated from 1989 to 2007 in Japan. Based on this nationwide RPGN survey, we analyzed the cases of 1147 AAV patients. Results We found that 52.3% of the AAV patients had pulmonary involvements: 15.4% of the AAV patients had alveolar hemorrhage (AH), 26.2% had interstitial lung disease (ILD), 2.8% had bronchial asthma, 2.4% had pulmonary granuloma and 12.8% had a chest X-ray abnormality without AH, ILD or pulmonary granuloma. Patient survival was significantly different among the following six groups: the 5-year survival rate was 41.5% in the patients with AH, 50.2% in those with ILD, 67.9% in those with bronchial asthma, 62.5% in those with pulmonary granuloma, 55.8% in those with chest X-ray abnormality and 73.3% in those without pulmonary involvement. AH was one of the predictors of 1-and 5-year mortality for patient survival in AAV, and ILD was added as one of the predictors of 5-year mortality. Conclusion In these AAV patients, not only AH but also ILD was frequently observed. AH was associated with the prognosis, but ILD was associated with the long-term prognosis of AAV. © 2015 The Author. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. Source


Kumakura A.,Tazuke Kofukai Medical Institute | Takahashi S.,Asahikawa University | Okajima K.,Asahikawa University | Hata D.,Tazuke Kofukai Medical Institute
Brain and Development | Year: 2014

Background: Forkhead box G1 gene (FOXG1) mutations and deletions are associated with a congenital variant of Rett syndrome (RTT). Nucleotide alterations of the coding region of FOXG1 have never caused dysmorphic features. Patient: An 8-year-old boy with the congenital variant of RTT who showed severe psychomotor deterioration, epilepsy, acquired microcephaly, and involuntary movements including jerky movements of the upper limbs and tongue protrusion. He showed dysmorphic features including round face, anteverted nostrils, and tented upper lips. Brain magnetic resonance imaging showed hypoplasia of the frontal lobes and the rostral part of the corpus callosum. The molecular cytogenetic analysis confirmed a de novo deletion of 14q12 including FOXG1 in this patient. Conclusion: We identified the smallest deletion of 14q12 involving FOXG1 among those previously reported. Dysmorphic facial features are a characteristic for the patients with chromosomal deletion including FOXG1. In our patient, C14orf23 is the only transcript other than FOXG1. Therefore, C14orf23 might be responsible for facial dysmorphism. © 2013 The Japanese Society of Child Neurology. Source


Kubo S.,Osaka City University | Nakanuma Y.,Kanazawa University | Takemura S.,Osaka City University | Sakata C.,Osaka City University | And 24 more authors.
Journal of Hepato-Biliary-Pancreatic Sciences | Year: 2014

Background An outbreak of cholangiocarcinoma occurred among workers in the offset color proof-printing department at a printing company in Japan. The aim of this study was to clarify the characteristics of the patients with cholangiocarcinoma. Methods This was a retrospective study conducted in 13 Japanese hospitals between 1996 to 2013. The clinicopathological findings of cholangiocarcinoma developed in 17 of 111 former or current workers in the department were investigated. Most workers were relatively young. Results The cholangiocarcinoma was diagnosed at 25-45 years old. They were exposed to chemicals, including dichloromethane and 1,2-dichloropropane. The serum γ-glutamyl transpeptidase activity was elevated in all patients. Dilated intrahepatic bile ducts without tumor-induced obstruction were observed in five patients. The cholangiocarcinomas arose from the large bile ducts. The precancerous or early cancerous lesions, such as biliary intraepithelial neoplasia and intraductal papillary neoplasm of the bile ducts, as well as non-specific bile duct injuries, such as fibrosis, were observed in various sites of the bile ducts in all eight patients for whom operative specimens were available. Conclusions The present results showed that cholangiocarcinomas occurred at a high incidence in relatively young workers of a printing company, who were exposed to chemicals including chlorinated organic solvents. © 2014 Japanese Society of Hepato-Biliary-Pancreatic Surgery. Source


Kinoshita M.,Osaka City University | Kubo S.,Osaka City University | Nakanuma Y.,Shizuoka Cancer Center | Sato Y.,Kanazawa University | And 18 more authors.
Journal of Hepato-Biliary-Pancreatic Sciences | Year: 2016

Background We aimed to identify the pathological characteristics of occupational cholangiocarcinoma. Methods We examined the location and distribution of the carcinomas: atypical epithelium including biliary intraepithelial neoplasia (BilIN) and intraductal papillary neoplasm of the bile duct (IPNB); and chronic bile duct injuries in operative or autopsy liver specimens from 16 patients. We examined the detailed pathological findings and diagnostic imaging of three patients. Immunohistochemical analysis using primary antibodies against γH2AX and S100P was performed. Results BilIN and chronic bile duct injury were observed in 16 patients, and IPNB or invasive IPNB was observed in 11 patients. BilIN, IPNB, and/or chronic bile duct injury were observed in almost all the large bile ducts. Regional dilatation of the bile ducts without tumor-induced obstruction revealed such pathological changes. Highly positive results for the γH2AX and S100P markers were noted in invasive carcinoma, BilIN, and IPNB, whereas positive results for γH2AX and negative results for S100P were noted in non-neoplastic biliary epithelium. Conclusions The carcinogenic process of occupational cholangiocarcinoma comprised chronic bile duct injury and DNA damage in almost all the large bile ducts, along with induction of precancerous lesions and development of invasive carcinoma. Such pathological findings reflected radiological changes on diagnostic imaging. Highlight In patients with occupational cholangiocarcinoma, Kinoshita and colleagues report a carcinogenic process consisting of chronic bile duct injury with DNA damage that occured within almost all of the large bile ducts, the induction of pre- or early cancerous lesions such as BilIN and IPNB, and the development of invasive carcinoma. © 2015 Japanese Society of Hepato-Biliary-Pancreatic Surgery. Source


Muso E.,Tazuke Kofukai Medical Institute | Mune M.,Ryoshukai Takaishi Fujii Hospital | Hirano T.,Showa University | Hattori M.,Tokyo Medical University | And 19 more authors.
Clinical and Experimental Nephrology | Year: 2015

Background: Hyperlipidemia is not merely a complication but a major exacerbating factor in longstanding nephrotic syndrome (NS). Low-density lipoprotein apheresis (LDL-A) has been reported to ameliorate dyslipidemia and induce rapid remission of NS. Several clinical studies have suggested the therapeutic efficacy of LDL-A, but the level of clinical evidence is insufficient. Therefore, a multicenter prospective study, POLARIS (Prospective Observational Survey on the Long-Term Effects of LDL Apheresis on Drug-Resistant Nephrotic Syndrome), was initiated in Japan. Method: Patients with drug-resistant NS were prospectively recruited into the study and treated with LDL-A in facilities that were registered in advance. In the POLARIS study design, the clinical data are to be followed up for 2 years. In the current study, we aimed at evaluating the short-term efficacy based on the treatment outcome of LDL-A immediately after completion of treatment. Results: Along with rapid improvement of hyperlipidemia, LDL-A significantly improved proteinuria and hypoproteinemia after treatment. More than half of the patients showed remission of NS based on the urinary protein level at the completion of LDL-A. The duration of NS before the start of treatment was significantly shorter in patients who responded to LDL-A. Conclusions: An analysis of patients registered in the POLARIS study indicated that LDL-A has short-term efficacy for drug-resistant NS. Rapid relief of dyslipidemia by LDL-A may provide early remission in about half of the NS patients who are resistant to conventional medication. Completion of the POLARIS study may reveal additional long-term effects of LDL-A in these patients. © 2014, Japanese Society of Nephrology. Source

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