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Background: The characteristic structure of motor neurons (MNs), particularly of the long axons, becomes damaged in the early stages of amyotrophic lateral sclerosis (ALS). However, the molecular pathophysiology of axonal degeneration remains to be fully elucidated., Method: Two sets of isogenic human-induced pluripotent stem cell (hiPSCs)-derived MNs possessing the single amino acid difference (p.H517D) in the fused in sarcoma (FUS) were constructed. By combining MN reporter lentivirus, MN specific phenotype was analyzed. Moreover, RNA profiling of isolated axons were conducted by applying the microfluidic devices that enable axon bundles to be produced for omics analysis. The relationship between the target gene, which was identified as a pathological candidate in ALS with RNA-sequencing, and the MN phenotype was confirmed by intervention with si-RNA or overexpression to hiPSCs-derived MNs and even in vivo. The commonality was further confirmed with other ALS-causative mutant hiPSCs-derived MNs and human pathology., Findings: We identified aberrant increasing of axon branchings in FUS-mutant hiPSCs-derived MN axons compared with isogenic controls as a novel phenotype. We identified increased level of Fos-B mRNA, the binding target of FUS, in FUS-mutant MNs. While Fos-B reduction using si-RNA or an inhibitor ameliorated the observed aberrant axon branching, Fos-B overexpression resulted in aberrant axon branching even in vivo. The commonality of those phenotypes was further confirmed with other ALS causative mutation than FUS., Interpretation: Analyzing the axonal fraction of hiPSC-derived MNs using microfluidic devices revealed that Fos-B is a key regulator of FUS-mutant axon branching. FUND: Japan Agency for Medical Research and development; Japanese Ministry of Education, Culture, Sports, Science and Technology Clinical Research, Innovation and Education Center, Tohoku University Hospital; Japan Intractable Diseases (Nanbyo) Research Foundation; the Kanae Foundation for the Promotion of Medical Science; and "Inochi-no-Iro" ALS research grant., (Copyright © 2019 The Authors. Published by Elsevier B.V. All rights reserved.)

Introduction: We describe the successful treatment of a patient with extended-aortic prosthetic graft infection (PGI) by surgery limited to the infected lesion based on 18 F-fluorodeoxyglucose positron emission tomography/computed tomography ( 18 F-FDG-PET/CT) findings., Presentation of Case: A 54-year-old man, who had undergone three graft replacements (GRs) for extended-thoracic aortic aneurysms, was diagnosed with PGI complicated by an aorto-esophageal fistula. On the basis of 18 F-FDG PET/CT findings, we performed a redo total arch replacement, preserving the other prosthesis where abnormal FDG uptake was not detected. All the resected tissues were positive for gram positive coccus. There were no signs of infection recurrence at 2 years postoperatively., Discussion: Since activated inflammatory cells such as macrophages uptake FDG, FDG-PET/CT clarifies the localization of the infected prosthesis precisely., Conclusion: Surgery localized to the infected sites detected by FDG-PET/CT can be an effective option for PGI in cases with previous multiple GRs., (Copyright © 2019 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Introduction: Optimal timing of surgical treatment for infective endocarditis (IE) complicated by intracranial hemorrhage remains controversial., Presentation of Case: A 43-year-old man with IE received appropriate antibiotic therapy but had recurrence of cerebral infarction and intracranial hemorrhage (ICH). Emergency valve surgery was performed 2days after ICH onset because of heart failure and recurrence of cerebral complications. Postoperatively, he showed no neurologic symptoms; neuroimaging showed no enlargement of ICH., Discussion: Postoperative risk of neurologic deterioration may be relatively lower than previously thought in patients with IE who undergo surgery within 1 month after ICH onset., Conclusions: Emergency surgery in patients with ICH is justified in cases of multiple indications for such small ICH. Further evaluation regarding the risk of subsequent hemorrhage in patients with ICH who require emergency valve surgery is warranted., (Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.)