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Aleksandra Gil-Krzewska, Yousuke Murakami, Giovanna Peruzzi, Kevin J O'Brien, Melissa A Merideth, Andrew R Cullinane, William A Gahl, John E Coligan, Bernadette R Gochuico, Konrad Krzewski
Hermansky-Pudlak syndrome (HPS) encompasses disorders with abnormal function of lysosomes and lysosome-related organelles, and some patients who develop immunodeficiency. The basic mechanisms contributing to immune dysfunction in HPS are ill-defined. We analysed natural killer (NK) cells from patients diagnosed with HPS-1, HPS-2, HPS-4, and an unreported HPS subtype. NK cells from an HPS-2 and an unreported HPS subtype share a similar cellular phenotype with defective granule release and cytotoxicity, but differ in cytokine exocytosis...
October 21, 2016: British Journal of Haematology
Benoit Peyronnet, Grégoire Robert, Vincent Comat, Morgan Rouprêt, Fernando Gomez-Sancha, Jean-Nicolas Cornu, Vincent Misrai
PURPOSE: To compare the learning curves, perioperative and early functional outcomes after HoLEP and GreenLEP. METHODS: Data from the first 100 consecutive cases treated by GreenLEP and HoLEP by two surgeons were prospectively collected from dedicated databases and analysed retrospectively. En-bloc GreenLEP and two-lobar HoLEP enucleations were conducted using the GreenLight HPS™ 2090 laser and Lumenis™ holmium laser. Patients' characteristics, perioperative outcomes and functional outcomes after 1, 3 and 6 months were compared between groups...
October 20, 2016: World Journal of Urology
Gavin Cloherty, Stephane Chevaliez, Christoph Sarrazin, Christine Herman, Vera Holzmayer, George Dawson, Benjamin Maasoumy, Johannes Vermehren, Heiner Wedemeyer, Jordan J Feld, Jean-Michel Pawlotsky
Approval of Ledipasvir/Sofosbuvir for the treatment of chronic hepatitis C (HCV) includes the truncation of therapy from 12 to 8 weeks in treatment naïve, non-cirrhotic patients with baseline HCV RNA levels <6 million IU/mL (6.8 log10 IU/mL). The aim of this study was to evaluate this clinical cutoff with a different widely used commercially available HCV RNA test. Results from samples tested prospectively with Roche High Pure TaqMan HCV 2.0 test (HPS) were compared to those tested retrospectively with the Abbott RealTime HCV RNA test (ART)...
October 20, 2016: Scientific Reports
Akimune Kaga, Hiroshi Watanabe, Hiroki Miyabayashi, Takaya Metoki, Setsuko Kitaoka, Satoru Kumaki
Neonatal toxic shock syndrome-like exanthematous disease (NTED) is a newly recognized neonatal infectious disease, caused by the superantigen toxic shock syndrome toxin-1 (TSST-1). TSST-1 is mainly produced by methicillin-resistant Staphylococcus aureus, and the immune responses to TSST-1 are known to cause toxic shock syndrome, a life-threatening infectious disease. The clinical symptoms of NTED are skin rash, fever, and thrombocytopenia, but severe thrombocytopenia is rare in term infants with NTED. Although the cause of NTED is the same as that of toxic shock syndrome, the clinical symptoms of NTED are milder than toxic shock syndrome...
2016: Tohoku Journal of Experimental Medicine
Toshiyuki Takahashi, Yasuyuki Sato, Takeshi Yamazaki, Asako Hayashi, Takayuki Okamoto
BACKGROUND: Most patients with congenital nephrotic syndrome (CNS) exhibit a failure to thrive. A previous study reported that five of 41 (12 %) infants with CNS had hypertrophic pyloric stenosis (HPS) requiring surgery. The reason for this is undetermined, and there are few reports regarding the relationship between these conditions or their clinical course. CASE DIAGNOSIS/TREATMENT: We present the case of a 4-month-old girl with CNS. She did not show typical manifestations of HPS, but thickened mucosal and submucosal layers and hypertrophy of the pyloric muscle were detected by repeated ultrasound examinations prior to the diagnosis of HPS...
October 18, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Hai-Long Cao, Xue Chen, Shao-Chun Du, Wen-Jing Song, Wei-Qiang Wang, Meng-Que Xu, Si-Nan Wang, Mei-Yu Piao, Xiao-Cang Cao, Bang-Mao Wang
BACKGROUND: Colorectal serrated polyp is considered as histologically heterogeneous lesions with malignant potential in western countries. However, few Asian studies have investigated the comprehensive clinical features of serrated polyps in symptomatic populations. The aim of the study was to evaluate the features of colorectal serrated polyps in a Chinese symptomatic population. METHODS: Data from all consecutive symptomatic patients were documented from a large colonoscopy database and were analyzed...
2016: Chinese Medical Journal
N Bel Feki, S Zayet, I Ben Ghorbel, M-H Houman
The lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) - the association of acquired factor II deficiency and lupus anticoagulant - is a rare disease that may cause a predisposition not only to thrombosis but also to severe bleeding. We are reporting on a 36-year-old female patient presenting with co-existing cerebral venous thrombosis and subdural hemorrhage. The coagulation screening showed a prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and a normal fibrinogen level and platelet count...
October 12, 2016: Journal des Maladies Vasculaires
Keisuke Goto, Daichi Maeda, Yukitsugu Kudo-Asabe, Takashi Hibiya, Akimasa Hayashi, Masashi Fukayama, Kenichi Ohashi, Akiteru Goto
AIMS: Hidradenoma papilliferum (HP) is a benign vulvar neoplasm that arises from anogenital mammary-like glands, and its morphology is similar to mammary intraductal papilloma. The aim of this study was to investigate oncogenic mutations involved in the tumourigenesis of HP. We focused specifically on PIK3CA and AKT1 mutations, which are both reported to be detected in 33% of mammary intraductal papillomas. METHODS: In total, seven HP cases were analysed. Clinicopathological analyses and immunohistochemistry for oestrogen receptor, p63, smooth muscle actin (SMA), p53 and β-catenin were performed...
October 14, 2016: Journal of Clinical Pathology
Fernanda Ferreira Rios, Luiz Antônio Rodrigues de Freitas, Liana Codes, Genario Oliveira Santos Junior, Maria Isabel Schinoni, Raymundo Paraná
:  Introduction and aim. Non-cirrhotic idiopathic portal hypertension (NCIPH), also known as hepatoportal sclerosis (HPS) is a disease of uncertain etiology. However, various pathophysiological mechanisms has been postulated, including chronic or recurrent infections and exposure to drugs or toxins. In this context, it appears to be of multifactorial etiology or resulting from a portal vascular endothelium aggression. It is important to consider whether the use of dietary supplements and herbs can trigger or contribute to the occurance of HPS...
November 2016: Annals of Hepatology
Ahmad Abu Turab Naqvi, Farah Anjum, Faez Iqbal Khan, Asimul Islam, Faizan Ahmad, Md Imtaiyaz Hassan
Helicobacter pylori is a Gram-negative bacteria that is responsible for gastritis in human. Its spiral flagellated body helps in locomotion and colonization in the host environment. It is capable of living in the highly acidic environment of the stomach with the help of acid adaptive genes. The genome of H. pylori 26695 strain contains 1,555 coding genes that encode 1,445 proteins. Out of these, 340 proteins are characterized as hypothetical proteins (HP). This study involves extensive analysis of the HPs using an established pipeline which comprises various bioinformatics tools and databases to find out probable functions of the HPs and identification of virulence factors...
September 2016: Genomics & Informatics
Megan K Mills, Colin D Strickland, Mary K Jesse, Peter A Lowry, Omer Mei-Dan, Jonathan A Flug
Osteoarthritis of the hip remains a prevalent disease condition that influences ever-changing treatment options. Procedures performed to correct anatomic variations, and, in turn, prevent or slow the progression of osteoarthritis, are aptly referred to as types of hip preservation surgery (HPS). Conditions that predispose individuals to femoroacetabular impingement (FAI), including pincer- and cam-type morphology, and hip dysplasia are specifically targeted in HPS. Common surgical interventions include acetabuloplasty, osteochondroplasty, periacetabular osteotomy (PAO), and derotational femoral osteotomy (DFO)...
October 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Amine Namouchi, Marta Gómez-Muñoz, Stephan A Frye, Line Victoria Moen, Torbjørn Rognes, Tone Tønjum, Seetha V Balasingham
BACKGROUND: As an intracellular human pathogen, Mycobacterium tuberculosis (Mtb) is facing multiple stressful stimuli inside the macrophage and the granuloma. Understanding Mtb responses to stress is essential to identify new virulence factors and pathways that play a role in the survival of the tubercle bacillus. The main goal of this study was to map the regulatory networks of differentially expressed (DE) transcripts in Mtb upon various forms of genotoxic stress. We exposed Mtb cells to oxidative (H2O2 or paraquat), nitrosative (DETA/NO), or alkylation (MNNG) stress or mitomycin C, inducing double-strand breaks in the DNA...
October 10, 2016: BMC Genomics
M Carrabba, A Vichi, G Vultaggio, S Pallari, R Paravina, M Ferrari
OBJECTIVES: To evaluate surface roughness and gloss of feldspathic ceramic blocks for chairside CAD/CAM systems before and after finishing and polishing. METHODS: VITA Mark II ceramic blocks for the CEREC CAD/CAM system were cut perpendicularly in order to obtain a total of 70 specimens (14 × 18 × 3 mm). The flat surface was roughened using a grinder/polisher with dry 120-grit silicone-carbide paper. Surface roughness and gloss were measured using a digital profilometer (Ra) and a glossmeter (GU), respectively...
October 10, 2016: Operative Dentistry
Serena Halls, Rebecca-Jane Law, Jeremy G Jones, David A Markland, Peter J Maddison, Jeanette M Thom
OBJECTIVES: Although exercise is an important factor in the management of rheumatoid arthritis (RA), research indicates that patients perceive that health professionals (HPs) are uncertain about the place of exercise in treatment and its relationship with joint damage. The present study investigated the perceptions of HPs regarding the effects of exercise on joint health in RA patients. METHODS: A questionnaire investigating perceptions of exercise and joint health was distributed via professional networks and websites...
October 6, 2016: Musculoskeletal Care
Patricia Struthers, Lisa Wegner, Petra de Koker, Wondwossen Lerebo, Renette J Blignaut
Health promoting schools, as conceptualised by the World Health Organisation, have been developed in many countries to facilitate the health-education link. In 1994, the concept of health promoting schools was introduced in South Africa. In the process of becoming a health promoting school, it is important for schools to monitor and evaluate changes and developments taking place. The Health Promoting Schools (HPS) Monitoring Questionnaire was developed to obtain opinions of students about their school as a health promoting school...
October 2, 2016: Health Promotion International
Helena Kilander, Birgitta Salomonsson, Johan Thor, Jan Brynhildsen, Siw Alehagen
OBJECTIVES: A substantial proportion of women who undergo an abortion continue afterwards without switching to more effective contraceptive use. Many subsequently have repeat unintended pregnancies. This study, therefore, aimed to identify and describe health professionalś experiences of providing contraceptive counselling to women seeking an abortion. METHODS: We interviewed 21 health professionals (HPs), involved in contraceptive counselling of women seeking abortion at three differently sized hospitals in Sweden...
September 30, 2016: European Journal of Contraception & Reproductive Health Care
Emer O'Loughlin, Susan Hourihan, Jeremy Chataway, E Diane Playford, Afsane Riazi
PURPOSE: The majority of people with multiple sclerosis (pwMS) initially present with discreet periods of relapses followed by partial remission of symptoms (RRMS). Over time, most pwMS transition to secondary progressive MS (SPMS), characterized by a gradual accumulation of disability. This study aimed to explore the experiences, coping and needs associated with transitioning from RRMS to SPMS. METHOD: Data were collected via semi-structured interviews with nine pwMS and seven specialist MS health professionals (HPs)...
August 16, 2016: Disability and Rehabilitation
Hong-Su Park, Il-Soo Lim, Sang-Kyu Kim, Toh-Kyung Kim, Choi-Kyu Park, Sang-Geon Yeo
Fowl adenovirus serotype 4 (FAdV-4) is the causative agent of hydropericardium syndrome (HPS), a highly pathogenic disease in poultry. In the present study, hexon, penton base, and fiber-2 genes encoding major capsid proteins were analyzed in four FAdV-4 isolates from HPS-affected chickens in Korea. Nucleotide sequences of the entire hexon (2811 bases), penton base (1578 bases), and fiber-2 (1425 bases) genes from the Korean isolates were 97.5-99.3, 99.1-99.7, and 95.5-99.0 % identical, respectively, to those of foreign FAdV-4 isolates...
September 28, 2016: Virus Genes
J Matthew Barnett, Xiao-Ying Yu, Kurtis P Recknagle, John A Glissmeyer
A planned laboratory space and exhaust system modification to the Pacific Northwest National Laboratory Material Science and Technology Building indicated that a new evaluation of the mixing at the air sampling system location would be required for compliance to ANSI/HPS N13.1-2011. The modified exhaust system would add a third fan, thereby increasing the overall exhaust rate out the stack, thus voiding the previous mixing study. Prior to modifying the radioactive air emissions exhaust system, a three-dimensional computational fluid dynamics computer model was used to evaluate the mixing at the sampling system location...
November 2016: Health Physics
Rajvinder Singh, Leonardo Zorrón Cheng Tao Pu, Doreen Koay, Alastair Burt
It is currently known that colorectal cancers (CRC) arise from 3 different pathways: the adenoma to carcinoma chromosomal instability pathway (50%-70%); the mutator "Lynch syndrome" route (3%-5%); and the serrated pathway (30%-35%). The World Health Organization has classified serrated polyps into three types of lesions: hyperplastic polyps (HP), sessile serrated adenomas/polyps (SSA/P) and traditional serrated adenomas (TSA), the latter two strongly associated with development of CRCs. HPs do not cause cancer and TSAs are rare...
September 14, 2016: World Journal of Gastroenterology: WJG
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