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    [Giant Aneurysms in Coronary Arteries of a Young Woman].
    Kardiologiia 2018 Jan(1):84-89
    Institute of Cardiology of Russian Cardiology Scientific and Production Complex.
    Coronary arteries aneurysms with their thrombotic occlusion are known to be detected in young patients who have suffered Kawasaki disease in childhood. The other vascular beds are usually not involved. In the literature one can find not enough information regarding diagnostics of this pathology, as well as no specific treatment algorithm. Read More

    Consensus guidelines on the optimal management in interventional EUS procedures: results from the Asian EUS group RAND/UCLA expert panel.
    Gut 2018 Feb 20. Epub 2018 Feb 20.
    Department of Medicine, National University of Singapore, Singapore.
    Objectives: Interventional endoscopic ultrasonography (EUS) procedures are gaining popularity and the most commonly performed procedures include EUS-guided drainage of pancreatic pseudocyst, EUS-guided biliary drainage, EUS-guided pancreatic duct drainage and EUS-guided celiac plexus ablation. The aim of this paper is to formulate a set of practice guidelines addressing various aspects of the above procedures.

    Methods: Formulation of the guidelines was based on the best scientific evidence available. Read More

    [Kawasaki disease: an update].
    Rev Med Suisse 2018 Feb;14(594):384-389
    Unité d'immuno-allergologie et rhumatologie pédiatrique, Service de pédiatrie, Département femme-mère-enfant, CHUV, 1011 Lausanne.
    The cause of Kawasaki disease (KD) is still unknown, but an infectious origin and genetic susceptibility have been suggested. The American Heart Association has changed the guidelines for diagnosis and treatment of KD in 2017. One goal is to better identify patients most at risk, particularly young children with « incomplete » KD. Read More

    Infliximab Plus Intravenous Immunoglobulin (IVIG) Versus IVIG Alone as Initial Therapy in Children with Kawasaki Disease Presenting with Coronary Artery Lesions: Is Dual Therapy More Effective?
    Pediatr Infect Dis J 2018 Feb 15. Epub 2018 Feb 15.
    Background: We previously demonstrated that 80% of KD patients who develop coronary artery lesions (CALs) have them at diagnosis. We postulated that KD patients presenting with CALs represent a group that may benefit from more aggressive initial therapy. Infliximab has been shown to decrease inflammation in KD patients when added to standard therapy. Read More

    In vitro activity of various antibiotics against clinical strains of Legionella species isolated in Japan.
    J Infect Chemother 2018 Feb 17. Epub 2018 Feb 17.
    Research Division for Development of Anti-Infective Agents, Institute of Development, Aging and Cancer, Tohoku University, Japan.
    The activities of various antibiotics against 58 clinical isolates of Legionella species were evaluated using two methods, extracellular activity (minimum inhibitory concentration [MIC]) and intracellular activity. Susceptibility testing was performed using BSYEα agar. The minimum extracellular concentration inhibiting intracellular multiplication (MIEC) was determined using a human monocyte-derived cell line, THP-1. Read More

    Propylthiouracil-induced anti-neutrophil cytoplasmic antibody-associated vasculitis mimicking Kawasaki disease.
    Paediatr Int Child Health 2018 Feb 19:1-4. Epub 2018 Feb 19.
    a Department of Pediatrics , Asahi General Hospital , Chiba , Japan.
    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is rare in children and is characterised as necrotising vasculitis predominantly affecting small and medium-sized vessels. Propylthiouracil (PTU), an antithyroid drug, has been implicated in drug-induced AAV. In contrast, Kawasaki disease (KD) is a common systemic vasculitis, typically observed in children, which affects the medium-sized vessels, including the coronary arteries. Read More

    Oocyte-Activating Capacity of Fresh and Frozen-Thawed Spermatids in the Common Marmoset (Callithrix jacchus).
    Mol Reprod Dev 2018 Feb 19. Epub 2018 Feb 19.
    RIKEN BioResource Center, Tsukuba, Ibaraki 305-0074, Japan.
    The common marmoset (Callithrix jacchus) represents a promising nonhuman primate model for the study of human diseases because of its small size, ease of handling, and availability of gene-modified animals. Here, we aimed to devise reproductive technology for marmoset spermatid injection using immature males for a possible rapid generational turnover. Spermatids at each step could be identified easily by their morphology under differential interference microscopy: thus, early round spermatids had a round nucleus with a few nucleolus-like structures and abundant cytoplasm, as in other mammals. Read More

    Controversies in diagnosis and management of Kawasaki disease.
    World J Clin Pediatr 2018 Feb 8;7(1):27-35. Epub 2018 Feb 8.
    Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India.
    Kawasaki disease (KD) is a common medium vessel systemic vasculitis that usually occurs in small children. It has a predilection for the coronary arteries, but other medium sized arteries can also be involved. The etiology of this disorder remains a mystery. Read More

    Usefulness and safety of anakinra in refractory Kawasaki disease complicated by coronary artery aneurysm.
    Cardiol Young 2018 Feb 19:1-4. Epub 2018 Feb 19.
    3Pediatric Emergency unit and Infectious Diseases,CHU Lille and University of Lille,Lille,France.
    Kawasaki disease is an acute self-limited vasculitis of unknown aetiology. The prognosis depends mainly on coronary damage. There is no consensus regarding optimal adjunctive therapeutics for refractory forms to treatment by intravenous immunoglobulins and corticosteroids. Read More

    F-FPYBF-2, a new F-18 labelled amyloid imaging PET tracer: biodistribution and radiation dosimetry assessment of first-in-manF-FPYBF-2 PET imaging.
    Ann Nucl Med 2018 Feb 16. Epub 2018 Feb 16.
    Shiga Medical Center Research Institute, Moriyama, Japan.
    Objective: Recently, a benzofuran derivative for the imaging of β-amyloid plaques, 5-(5-(2-(2-(2-F-fluoroethoxy)ethoxy)ethoxy)benzofuran-2-yl)- N-methylpyridin-2-amine (F-FPYBF-2) has been validated as a tracer for amyloid imaging and it was found thatF-FPYBF-2 PET/CT is a useful and reliable diagnostic tool for the evaluation of AD (Higashi et al. Ann Nucl Med, https://doi.org/10. Read More

    Is Associated With Kawasaki Disease in Intravenous Immunoglobulin Responder Patients.
    Circ Genom Precis Med 2018 Feb;11(2):e002020
    From the Asan Institute for Life Sciences (Y.-C.K., J.-J.K., J.-K.L) and Department of Pediatrics, Asan Medical Center (J.J.Y.), University of Ulsan College of Medicine, Seoul, Korea; Department of Pediatrics, Chung-Ang University Hospital, Seoul, Korea (S.W.Y.); Department of Pediatrics, Kyung Hee University Hospital at Gangdong, Seoul, Korea (K.L.Y.); Department of Pediatrics, The Catholic University of Korea, Daejeon St. Mary's Hospital, Daejeon, Korea (K.-Y.L.); Department of Pediatrics, Chungnam National University Hospital, Daejeon, Korea (H.-R.K.); Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea (G.B.K.); Department of Pediatrics, University of Ulsan, Gangneung Asan Hospital, Gangneung, Korea (M.-K.H.); Department of Pediatrics, Inje University Paik Hospital, Busan, Korea (M.S.S.); Department of Pediatrics, Pusan National University Hospital, Busan, Korea (H.D.L.); Department of Pediatrics, Korea University Hospital, Seoul, Korea (K.S.H., G.Y.J.); and Department of Pediatrics, Ewha Womans University Hospital, Seoul, Korea (S.S., Y.M.H.)

    Innate immune responses following Kawasaki disease and toxic shock syndrome.
    PLoS One 2018 15;13(2):e0191830. Epub 2018 Feb 15.
    Murdoch Children's Research Institute, The Royal Children's Hospital, Melbourne, Vic, Australia.
    The pathogenesis of Kawasaki disease (KD) remains unknown and there is accumulating evidence for the importance of the innate immune system in initiating and mediating the host inflammatory response. We compared innate immune responses in KD and toxic shock syndrome (TSS) participants more than two years after their acute illness with control participants to investigate differences in their immune phenotype. Toxic shock syndrome shares many clinical features with KD; by including both disease groups we endeavoured to explore changes in innate immune responses following acute inflammatory illnesses more broadly. Read More

    The possible link between coeliac and Kawasaki diseases in Brazil: a cross-sectional study.
    BMJ Open 2018 02 14;8(2):e018803. Epub 2018 Feb 14.
    Interdisciplinary Laboratory of Biosciences and Celiac Disease Research Center, School of Medicine, University of Brasilia, Brasilia, Brazil.
    Background: Kawasaki disease (KD) is a self-limited acute systemic vasculitis of unknown aetiology that predominantly affects infants and young children eventually associated with immunological abnormalities. Coeliac disease (CD) is an inflammatory autoimmune disease characterised by a permanent gluten intolerance, which affects genetically susceptible individuals of any age group, and can cause intestinal and systemic symptoms. Association of CD with KD has been previously described in a single study that disclosed a surprisingly high prevalence of CD in children with a history of KD. Read More

    C-reactive protein and N-terminal pro-brain natriuretic peptide discrepancy: a differentiation of adenoviral pharyngoconjunctival fever from Kawasaki disease.
    Korean J Pediatr 2018 Jan 22;61(1):12-16. Epub 2018 Jan 22.
    Department of Pediatrics, Ewha Womans University School of Medicine, Seoul, Korea.
    Purpose: To differentiate adenoviral pharyngoconjunctival fever (PCF) from acute Kawasaki disease (KD) using laboratory tests before results of virus-real time polymerase chain reaction and ophthalmologic examination are obtained.

    Methods: Baseline patient characteristics and laboratory measurements were compared between 40 patients with adenovirus infection and 123 patients with KD.

    Results: The patients with adenovirus infection were generally older than those with KD (median: 3. Read More

    Superior Mesenteric Artery Syndrome Improved by Enteral Nutritional Therapy according to the Controlling Nutritional Status Score.
    Case Rep Gastroenterol 2017 Sep-Dec;11(3):729-735. Epub 2017 Nov 29.
    Department of Gastroenterological Surgery, Juntendo University Faculty of Medicine, Tokyo, Japan.
    Superior mesenteric artery syndrome (SMAS) is a relatively rare disease that involves bowel obstruction symptoms, such as vomiting and gastric distension, owing to the compression of the third portion of the duodenum from the front by the superior mesenteric artery (SMA) and from the rear by the abdominal aorta and the spine. SMAS is diagnosed on the basis of an upper gastrointestinal examination series indicating the obstruction of the third portion of the duodenum or a computed tomography scan indicating the narrowing of the branch angle between the aorta and the SMA (i.e. Read More

    [Burkholderia cepacia infection in children: a clinical analysis of 16 cases].
    Zhongguo Dang Dai Er Ke Za Zhi 2018 Feb;20(2):112-115
    Department of Pediatrics, Hunan Provincial People's Hospital, Changsha 410000, China.
    Objective: To investigate the distribution characteristics and clinical features of Burkholderia cepacia infection in children.

    Methods: A retrospective analysis was performed for the clinical data of 16 children with Burkholderia cepacia infection who were hospitalized between June 2012 and September 2017.

    Results: All 16 children with Burkholderia cepacia infection were sporadic cases. Read More

    Coronary manifestations of Kawasaki Disease in computed tomography coronary angiography.
    J Cardiovasc Comput Tomogr 2017 Dec 11. Epub 2017 Dec 11.
    Department of Diagnostic Imaging, National University Hospital (NUH), 5 Lower Kent Ridge Road, Singapore 119074. Electronic address:
    Coronary arteritis in Kawasaki disease can lead to serious complications such myocardial infarction and sudden death. The identification of coronary manifestations with a method that is minimally invasive and of low radiation exposure is therefore important in paediatric patients with Kawasaki disease. Coronary CT angiography can be an attractive alternative to invasive coronary angiography. Read More

    The association between oxidative stress and endothelial dysfunction in early childhood patients with Kawasaki disease.
    BMC Cardiovasc Disord 2018 02 9;18(1):30. Epub 2018 Feb 9.
    Department of Pediatrics, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192, Japan.
    Background: Oxidative stress has recently been shown to play an important role in the development of arteriosclerosis in patients with Kawasaki disease (KD); however, no study has investigated this association in early childhood patients with KD. In this study, we evaluated prospectively the association between the levels of oxidative stress and the endothelial function in early childhood patients with KD.

    Methods: We compared the derivatives of reactive oxygen metabolites (ROM), flow-mediated dilatation (FMD), and biological characteristics in a population of 50 children: 10 patients with KD and coronary artery lesions (CAL) (group 1), 15 KD patients without CAL (group 2), and 25 healthy age- and sex-matched children (group 3). Read More

    Significant impact of electrical storm on mortality in patients with structural heart disease and an implantable cardiac defibrillator.
    Int J Cardiol 2018 Mar;255:85-91
    Department of Research and Development, Tachikawa Medical Center, Niigata, Japan.
    Background: Electrical storm (E-Storm), defined as multiple episodes of ventricular arrhythmias within a short period of time, is an important clinical problem in patients with an implantable cardiac defibrillator (ICD) including cardiac resynchronization therapy devices capable of defibrillation. The detailed clinical aspects of E-Storm in large populations especially for non-ischemic dilated cardiomyopathy (DCM), however, remain unclear.

    Objective: This study was performed to elucidate the detailed clinical aspects of E-Storm, such as its predictors and prevalence among patients with structural heart disease including DCM. Read More

    Dengue-Triggered Kawasaki Disease: A Report of 2 Cases.
    J Clin Rheumatol 2018 Feb 7. Epub 2018 Feb 7.
    Pediatric Clinical Immunology and Rheumatology Postgraduate Institute of Medical Education and Research Chandigarh, India Department of Virology Postgraduate Institute of Medical Education and Research Chandigarh, India Allergy Immunology Unit Advanced Pediatrics Centre Postgraduate Institute of Medical Education and Research Chandigarh, India

    Incidence and predictors of lesion-specific ischemia by FFR: Learnings from the international ADVANCE registry.
    J Cardiovasc Comput Tomogr 2018 Feb 2. Epub 2018 Feb 2.
    Wakayama Medical University, Wakayama, Japan.
    Background: To date, the clinical utility of coronary computed tomography angiography (CTA)-derived fractional flow reserve (FFRCT) has been limited to trials and single center experiences. We herein report the incidence of abnormal FFRCT (≤0.80) and the relationship of lesion-specific ischemia to subject demographics, symptoms, and degree of stenosis in the multicenter, prospective ADVANCE registry. Read More

    Macrophages and cytotoxic T cells infiltrate the destructed mitral tissue in Kawasaki disease.
    BMJ Case Rep 2018 Feb 2;2018. Epub 2018 Feb 2.
    Kawasaki Disease Center, Fukuoka Children's Hospital, Fukuoka, Japan.
    Kawasaki disease (KD) is an acute febrile systemic vasculitic syndrome especially affecting medium-sized arteries, including the coronary artery. Inflammation may involve all organs, and valvulitis is one of the cardiovascular complications that occurs in the acute phase of KD. However, details regarding the mechanism are unclear. Read More

    Risk Factors for Relapse of Antineutrophil Cytoplasmic Antibody-associated Vasculitis in Japan: A Nationwide, Prospective Cohort Study.
    J Rheumatol 2018 Feb 1. Epub 2018 Feb 1.
    From the Department of Environmental and Preventive Medicine, and Department of Nephrology and Laboratory Medicine, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa; Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama; Department of Rheumatology and Clinical Immunology, Saitama Medical Center, Saitama; Division of Endocrinology and Metabolism, Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kagawa; Division of Rheumatology and Allergology, Departments of Internal Medicine and Dermatology, St. Marianna University School of Medicine, Kawasaki; Department of Hematology, Clinical Immunology and Infectious Diseases, Ehime University Graduate School of Medicine, Matsuyama; Department of Rheumatology, Faculty of Medicine, University of Tsukuba, Tsukuba; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Center for Nephrology and Urology, Division of Nephrology and Dialysis, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka; Third Department of Internal Medicine, Division of Immunology and Rheumatology, Hamamatsu University School of Medicine, Hamamatsu; Division of Rheumatology, Niigata Rheumatic Center, Shibata; Department of Pharmacovigilance, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University; Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine; Department of Respiratory Medicine, Toho University Omori Medical Center; Division of Nephrology, Tokyo Medical University Hachioji Medical Center; Department of Nephrology and Endocrinology, Graduate School of Medicine, The University of Tokyo; Nephrology and Rheumatology, First Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan. Supported by grants for Research on Rare and Intractable Diseases, the Ministry of Health, Labor and Welfare, Japan (nannti-ippann-044), and the Study Group for Strategic Exploration of Drug Seeds for ANCA-associated Vasculitis and Construction of Clinical Evidence from the Japan Agency for Medical Research and Development (16ek0109121h0002). A. Hara, MD, PhD, Department of Environmental and Preventive Medicine, Kanazawa University; T. Wada, MD, PhD, Department of Nephrology and Laboratory Medicine, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University; K. Sada, MD, PhD, Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences; K. Amano, MD, PhD, Department of Rheumatology and Clinical Immunology, Saitama Medical Center; H. Dobashi, MD, PhD, Division of Endocrinology and Metabolism, Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University; M. Harigai, MD, PhD, Department of Pharmacovigilance, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University; Y. Takasaki, MD, PhD, Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine; H. Yamada, MD, PhD, Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine; H. Hasegawa, MD, PhD, Department of Hematology, Clinical Immunology and Infectious Diseases, Ehime University Graduate School of Medicine; T. Hayashi, MD, PhD, Department of Rheumatology, Faculty of Medicine, University of Tsukuba; S. Fujimoto, MD, PhD, Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki; E. Muso, MD, PhD, Center for Nephrology and Urology, Division of Nephrology and Dialysis, Kitano Hospital, Tazuke Kofukai Medical Research Institute; T. Kawakami, MD, PhD, Department of Dermatology, St. Marianna University School of Medicine; S. Homma, MD, PhD, Department of Respiratory Medicine, Toho University Omori Medical Center; M. Yoshida, MD, PhD, Division of Nephrology, Tokyo Medical University, Hachioji Medical Center; J. Hirahashi, MD, PhD, Department of Nephrology and Endocrinology, Graduate School of Medicine, The University of Tokyo; N. Ogawa, MD, PhD, Third Department of Internal Medicine, Division of Immunology and Rheumatology, Hamamatsu University School of Medicine; S. Ito, MD, PhD, Division of Rheumatology, Niigata Rheumatic Center; H. Makino, MD, PhD, Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences; Y. Arimura, MD, PhD, Nephrology and Rheumatology, First Department of Internal Medicine, Kyorin University School of Medicine. Address correspondence to Dr. T. Wada, Department of Nephrology and Laboratory Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Faculty of Medicine, Kanazawa University, 13-1 Takara-machi, Kanazawa 920-8641, Japan. E-mail: Accepted for publication November 6, 2017.
    Objective: The aim was to elucidate the prognosis and risk factors associated with relapse during longterm remission maintenance therapy for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

    Methods: Patients with newly diagnosed AAV (n = 156) were registered in the Remission Induction Therapy in Japanese patients with ANCA-associated Vasculitides (RemIT-JAV) study, and among them, 83 patients who achieved remission were enrolled and followed up for 24 additional months in our nationwide, prospective cohort study (Co-RemIT-JAV; registration number UMIN 000006373). Patterns of maintenance therapy, effectiveness, and safety were evaluated from months 25 to 48 after the RemIT-JAV. Read More

    Rationale and design of the VISION study: a randomized, open-label study to evaluate the long-term safety of vonoprazan as maintenance treatment in patients with erosive esophagitis.
    Clin Exp Gastroenterol 2018 24;11:51-56. Epub 2018 Jan 24.
    Takeda Pharmaceutical Co., Ltd, Osaka, Japan.
    Erosive esophagitis (EE) occurs when the epithelial mucosa is damaged due to gastric acid reflux, and the incidence of this disease is increasing in Japan due to changes in diet and lifestyle. The condition can be treated using proton pump inhibitors (PPIs) that act by irreversibly blocking the H,K-ATPase responsible for acid secretion. Vonoprazan is a Kcompetitive channel inhibitor, which reversibly and potently inhibits gastric acid secretion. Read More

    Environmental epidemiology of Kawasaki disease: Linking disease etiology, pathogenesis and global distribution.
    PLoS One 2018 7;13(2):e0191087. Epub 2018 Feb 7.
    Labatt Family Heart Centre, Department of Pediatrics, The University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada.
    Background: The pathogenesis of Kawasaki disease (KD) is commonly ascribed to an exaggerated immunologic response to an unidentified environmental or infectious trigger in susceptible children. A comprehensive framework linking epidemiological data and global distribution of KD has not yet been proposed.

    Methods And Findings: Patients with KD (n = 81) were enrolled within 6 weeks of diagnosis along with control subjects (n = 87). Read More

    Mogamulizumab (Anti-CCR4) in HTLV-1-Associated Myelopathy.
    N Engl J Med 2018 02;378(6):529-538
    From the Department of Rare Diseases Research, Institute of Medical Science (T.S., A.L.G.C.-R., N.Y., N.A., Y.Y.), Medical Informatics (E.I.), and the Departments of Pharmacology (N.M.) and Neurology (Y.H.), St. Marianna University School of Medicine, and the Department of Advanced Medical Innovation, St. Marianna University Graduate School of Medicine (T.W., Y.Y.), Kawasaki, the Laboratory of Virus Control, Institute for Virus Research, Kyoto University, Kyoto (R.F., M.M.), the Department of Computational Biology and Medical Sciences, Graduate School of Frontier Sciences, University of Tokyo, Tokyo (K.U.), and the Department of Hematology, Rheumatology, and Infectious Diseases, Graduate School of Medical Sciences, Faculty of Life Sciences, Kumamoto University, Kumamoto (M.M.) - all in Japan.
    Background: Human T-lymphotropic virus type 1 (HTLV-1) causes the debilitating neuroinflammatory disease HTLV-1-associated myelopathy-tropical spastic paraparesis (HAM-TSP) as well as adult T-cell leukemia-lymphoma (ATLL). In patients with HAM-TSP, HTLV-1 infects mainly CCR4+ T cells and induces functional changes, ultimately causing chronic spinal cord inflammation. We evaluated mogamulizumab, a humanized anti-CCR4 monoclonal antibody that targets infected cells, in patients with HAM-TSP. Read More

    Epidemiology of primary systemic vasculitis in children: a population-based study from southern Sweden.
    Scand J Rheumatol 2018 Feb 7:1-8. Epub 2018 Feb 7.
    d Department of Rheumatology, Clinical Sciences Lund , Lund University , Lund , Sweden.
    Objectives: To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden.

    Methods: Potential cases of PSV [IgA vasculitis (IgAV, Henoch-Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu's arteritis (TAK)] were identified in a comprehensive regional healthcare register. The study area is Skåne, the southernmost county of Sweden (population 1. Read More

    Remission of hyperglycemia after withdrawal of oral antidiabetic drugs in Japanese patients with early stage of type 2 diabetes.
    J Diabetes Investig 2018 Feb 6. Epub 2018 Feb 6.
    Division of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School.
    Aims: To assess whether intervention with oral anti-diabetic drug in Japanese patients with the early stage of type 2 diabetes could provide a significant remission of the disease process.

    Methods: Patients with diabetes duration less than 5 years were randomized to the lifestyle modification (LFS), pioglitazone (PIO), or sulfonylurea (SU) treatment group. In phase 1 as on-treatment period and in phase 2 as off-treatment period, the duration kept HbA1c less than the target was compared among groups. Read More

    Failure to Predict High-Risk Kawasaki Disease Patients in a Population-Based Study Cohort in Germany.
    Pediatr Infect Dis J 2018 Feb 3. Epub 2018 Feb 3.
    Division of Epidemiology, Institute of Social Pediatrics and Adolescent Medicine, Ludwig-Maximilians-University Munich, Haydnstr. 5, 80336 Munich, Germany.
    Importance: Diverse scores on high-risk Kawasaki disease patients (KD) have proven a good prognostic validity in the Japanese population. However, data on non-Japanese has been inconclusive.

    Objective: Do the Kobayashi, Egami and Sano scores or application of up-to-date statistical methods (Random Forest) predict response to standard IVIG therapy and the risk of persistent CAA in patients with KD in a mainly Caucasian population in Germany?

    Design, Setting And Participants: Data on 442 children (German population-based survey, 2013 and 2014) were used to assess the prognostic validity of the Kobayashi, Egami and Sano scores for being refractory to IVIG treatment and for predicting the risk of persistent coronary artery aneurysm (CAA). Read More

    Dissociation of β-Sheet Stacking of Amyloid β Fibrils by Irradiation of Intense, Short-Pulsed Mid-infrared Laser.
    Cell Mol Neurobiol 2018 Feb 5. Epub 2018 Feb 5.
    Department of Laboratory Sciences, Gunma University Graduate School of Health Sciences, 3-39-22, Showa-machi, Maebashi, Gunma, 371-8511, Japan.
    Structure of amyloid β (Aβ) fibrils is rigidly stacked by β-sheet conformation, and the fibril state of Aβ is profoundly related to pathogenesis of Alzheimer's disease (AD). Although mid-infrared light has been used for various biological researches, it has not yet been known whether the infrared light changes the fibril structure of Aβ. In this study, we tested the effect of irradiation of intense mid-infrared light from a free-electron laser (FEL) targeting the amide bond on the reduction of β-sheet content in Aβ fibrils. Read More

    Genetic Analysis of Human Norovirus Strains in Japan in 2016-2017.
    Front Microbiol 2018 18;9. Epub 2018 Jan 18.
    Infectious Disease Surveillance Center, National Institute of Infectious Diseases, Musashimurayama, Japan.
    In the 2016/2017 winter season in Japan, HuNoV GII.P16-GII.2 strains (2016 strains) emerged and caused large outbreaks of acute gastroenteritis. Read More

    Atypical Kawasaki Disease Presenting with Hemiparesis and Aphasia: A Case Report.
    Iran J Med Sci 2018 Jan;43(1):86-89
    Department of Pediatric Neurology, Mazandaran University of Medical Sciences, Sari, Iran.
    Kawasaki disease (KD) is an inflammatory vasculitis. KD is classified into two groups based on clinical characteristics criteria, namely classic and incomplete. Cerebral vascular abnormality, especially arterial ischemic stroke (AIS) is very rare and unusual in KD. Read More

    Diagnostic predictors of Legionella pneumonia in Japan.
    J Infect Chemother 2018 Mar 2;24(3):159-163. Epub 2018 Feb 2.
    Research Division for Development of Anti-Infective Agents, Institute of Development, Aging and Cancer, Tohoku University, Japan.

    Epidemiology of Kawasaki Disease in Canada 2004 to 2014: Comparison of Surveillance Using Administrative Data vs Periodic Medical Record Review.
    Can J Cardiol 2017 Dec 15. Epub 2017 Dec 15.
    Labatt Family Heart Centre, Department of Pediatrics, The University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada. Electronic address:
    Background: We have previously documented an increase in the incidence of Kawasaki disease (KD) in Ontario followed by a stabilization from 1995 to 2006. We sought to validate the estimation of incidence of KD using administrative data and to describe the epidemiology of KD across Canada from 2004 to 2014.

    Methods: We queried the Canadian Hospital Discharge Database for hospital admissions associated with a discharge diagnosis of KD. Read More

    [Long-Term Survival in a Patient with Combined Hepatocellular-Cholangiocarcinoma Treated with Multidisciplinary Therapy - A Case Report].
    Gan To Kagaku Ryoho 2017 Nov;44(12):1065-1067
    Dept. of Surgery, Kobe Ekisaikai Hospital.
    The patient was a woman in her 50's. She went to the hospitalfor epigastric discomfort. Numerous hypovascular tumors spreading almost to the entire liver were detected via abdominal CT. Read More

    Aminopyrimidine Class Aggregation Inhibitor Effectively Blocks Aβ-Fibrinogen Interaction and Aβ-Induced Contact System Activation.
    Biochemistry 2018 Feb 9. Epub 2018 Feb 9.
    Patricia and John Rosenwald Laboratory of Neurobiology and Genetics, The Rockefeller University , New York, New York 10065, United States.
    Accumulating evidence suggests that fibrinogen, a key protein in the coagulation cascade, plays an important role in circulatory dysfunction in Alzheimer's disease (AD). Previous work has shown that the interaction between fibrinogen and β-amyloid (Aβ), a hallmark pathological protein in AD, induces plasmin-resistant abnormal blood clots, delays fibrinolysis, increases inflammation, and aggravates cognitive function in mouse models of AD. Since Aβ oligomers have a much stronger affinity for fibrinogen than Aβ monomers, we tested whether amyloid aggregation inhibitors could block the Aβ-fibrinogen interaction and found that some Aβ aggregation inhibitors showed moderate inhibitory efficacy against this interaction. Read More

    Coronary artery aneurysm combined with other multiple aneurysms at multiple locations: A case report and systematic review.
    Medicine (Baltimore) 2017 Dec;96(50):e9230
    Department of Cardiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
    Background: Coronary artery aneurysm (CAA) with concomitant aneurysms at multiple sites is quite unusual and rare. The characteristics and the etiology of this phenomenon are unknown.

    Methods: Herein, we present a case with right coronary aneurysm with concomitant abdominal aorta as well as right renal artery aneurysm. Read More

    F-FPYBF-2, a new F-18-labelled amyloid imaging PET tracer: first experience in 61 volunteers and 55 patients with dementia.
    Ann Nucl Med 2018 Jan 31. Epub 2018 Jan 31.
    Shiga Medical Center Research Institute, Moriyama, Japan.
    Objective: Recently, we developed a benzofuran derivative for the imaging of β-amyloid plaques, 5-(5-(2-(2-(2-F-fluoroethoxy)ethoxy)ethoxy)benzofuran-2-yl)-N-methylpyridin-2-amine (F-FPYBF-2) (Ono et al., J Med Chem 54:2971-9, 2011). The aim of this study was to assess the feasibility ofF-FPYBF-2 as an amyloid imaging PET tracer in a first clinical study with healthy volunteers and patients with various dementia and in comparative dual tracer study usingC-Pittsburgh Compound B (C-PiB). Read More

    Infliximab versus intravenous immunoglobulin for refractory Kawasaki disease: a phase 3, randomized, open-label, active-controlled, parallel-group, multicenter trial.
    Sci Rep 2018 Jan 31;8(1):1994. Epub 2018 Jan 31.
    Department of Pediatrics, NTT Sapporo Medical Center, Sapporo, Japan.
    We compared the efficacy and safety of infliximab with intravenous immunoglobulin (IVIG), a standard therapy, in a phase 3 trial (NCT01596335) for Japanese patients with Kawasaki disease (KD) showing persistent fever after initial IVIG. Patients with initial IVIG-refractory KD, aged 1-10 years, received a single dose of IV infliximab 5 mg/kg or IV polyethylene glycol-treated human immunoglobulin (VGIH) 2 g/kg on day 0. Primary outcome was defervescence rate within 48 h after the start of treatment. Read More

    Retrospective observational study indicates that the paediatric assessment triangle may suggest the severity of Kawasaki disease.
    Acta Paediatr 2018 Jan 31. Epub 2018 Jan 31.
    Clinical Research Support Center, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
    Aim: We examined whether the paediatric assessment triangle (PAT) could predict the severity of Kawasaki disease.

    Methods: We enroled patients diagnosed with Kawasaki disease between July 2012 and June 2016 at the emergency department of Tokyo Metropolitan Children's Medical Center in Tokyo, Japan. Triage nurses assigned participants to unstable or stable PAT groups. Read More

    Risk estimation model for nonalcoholic fatty liver disease in the Japanese using multiple genetic markers.
    PLoS One 2018 31;13(1):e0185490. Epub 2018 Jan 31.
    Department of Gastroenterology and Hepatology, Saiseikai Suita Hospital, Suita, Japan.
    The genetic factors affecting the natural history of nonalcoholic fatty liver disease (NAFLD), including the development of nonalcoholic steatohepatitis (NASH) and NASH-derived hepatocellular carcinoma (NASH-HCC), are still unknown. In the current study, we sought to identify genetic factors related to the development of NAFLD, NASH, and NASH-HCC, and to establish risk-estimation models for them. For these purposes, 936 histologically proven NAFLD patients were recruited, and genome-wide association (GWA) studies were conducted for 902, including 476 NASH and 58 NASH-HCC patients, against 7,672 general-population controls. Read More

    Pomalidomide with or without dexamethasone for relapsed/refractory multiple myeloma in Japan: a retrospective analysis by the Kansai Myeloma Forum.
    Int J Hematol 2018 Jan 29. Epub 2018 Jan 29.
    Department of Hematology and Oncology, Kyoto University, Kyoto, Japan.
    Determinants of the efficacy and safety of pomalidomide (POM) monotherapy or POM plus dexamethasone (DEX) (POM/DEX) for relapsed and refractory multiple myeloma (RRMM) were examined retrospectively in a real-world clinical practice setting in Japan. The subjects were 108 patients registered with the Kansai Myeloma Forum, who were treated with either POM or POM/DEX. Of these, 79 (73%), 73 (68%), and 58 (54%) were resistant to bortezomib (BTZ), lenalidomide (LEN), and both BTZ and LEN, respectively. Read More

    Epoetin beta pegol for treatment of anemia ameliorates deterioration of erythrocyte quality associated with chronic kidney disease.
    BMC Nephrol 2018 01 27;19(1):19. Epub 2018 Jan 27.
    Product Research Department, Chugai Pharmaceutical Co., Ltd, 200 Kajiwara, Kamakura, 247-8530, Japan.
    Background: Epoetin beta pegol (continuous erythropoietin receptor activator; C.E.R. Read More

    [Transient detection of lupus anticoagulant in acute phase of Kawasaki disease].
    Nihon Rinsho Meneki Gakkai Kaishi 2017 ;40(6):456-459
    National Center for Child Health and Development, Department of Postgraduate Education and Training.
      In Kawasaki disease (KD), endothelial damage and an elevation in coagulant factors provoke thrombosis. Lupus anticoagulant (LA) is strongly associated with the risk of thrombosis in patients with antiphospholipid syndrome; however, there has been no report of positive LA in KD patients. A previously healthy, 2-year-old boy was admitted due to fever, bilateral conjunctivitis, redness of the lips, and unilateral cervical lymphadenopathy. Read More

    Expression of activating natural killer-cell receptors is a hallmark of the innate-like T-cell neoplasm in peripheral T-cell lymphomas.
    Cancer Sci 2018 Jan 24. Epub 2018 Jan 24.
    Division of Hematology & Oncology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan.
    Peripheral T- or natural killer (NK)-cell lymphomas are rare and difficult-to-recognize diseases. It remains arduous to distinguish between NK cell- and cytotoxic T-lymphocyte-derived lymphomas through routine histological evaluation. To clarify the cells of origin, we focused on NK-cell receptors and examined the expression using immunohistochemistry in 22 cases with T- and NK-cell neoplasms comprising angioimmunoblastic T-cell lymphoma, anaplastic lymphoma kinase (ALK)-positive and -negative anaplastic large-cell lymphomas, extranodal NK/T-cell lymphoma, nasal type, monomorphic epitheliotropic intestinal T-cell lymphoma, aggressive NK-cell leukemia, and other peripheral T-cell lymphomas. Read More

    Multiple target autoantigens on endothelial cells identified in juvenile dermatomyositis using proteomics.
    Rheumatology (Oxford) 2018 Jan 18. Epub 2018 Jan 18.
    Department of Pediatrics and Genetics, Genomics, & Bioinformatics Program, Jacobs School of Medicine and Biomedical Sciences University at Buffalo, Buffalo, NY, USA.
    Objective: Although generally classified within the group of inflammatory myopathies, JDM displays many pathological features of vasculitis. Previous work has shown that AECA are abundant in other forms of vasculitis. We therefore investigated whether such antibodies might also be detected in JDM. Read More

    Serum levels of C1q/tumor necrosis factor related protein-1 in children with Kawasaki disease.
    Pediatr Res 2018 Jan 23. Epub 2018 Jan 23.
    Department of Cardiovascular Medicine, Children's Hospital of Chongqing Medical University, Chongqing, P.R. China.
    Objective: To investigate the serum C1q/tumor necrosis factor-related protein-1 (CTRP1) levels in children with acute Kawasaki disease (KD), as well as the relationship between CTRP1 levels and laboratory variables.

    Methods: Eighty-seven children with KD and 38 healthy controls (HC) were included in this study. General characteristics were obtained from all subjects. Read More

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