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Hoffman ' s syndrome

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https://www.readbyqxmd.com/read/28535116/is-mannitol-the-treatment-of-choice-for-patients-with-ciguatera-fish-poisoning
#1
Michael E Mullins, Robert S Hoffman
CONTEXT: Ciguatera fish poisoning arises primarily from consumption of carnivorous reef fish caught in tropical and sub-tropical waters. Ciguatoxins, a class of tasteless, heat-stable, polycyclic toxins produced by dinoflagellates, accumulate through the food chain and concentrate in various carnivorous fish, such as groupers, barracudas, wrasses, amberjack, kingfishes, and eels. Characteristics of ciguatera fish poisoning include early nausea, vomiting, and diarrhea in the first one to two days post ingestion, followed by the appearance of sensory disturbances...
May 23, 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28421594/anti-aggregation-therapy-in-patients-with-acute-coronary-syndrome-recommendations-for-medical-emergency-teams-experts-standpoint
#2
Jacek Kubica, Piotr Adamski, Przemysław Paciorek, Jerzy R Ładny, Zbigniew Kalarus, Waldemar Banasiak, Wacław Kochman, Jarosław Gorący, Beata Wożakowska-Kapłon, Eliano Pio Navarese, Andrzej Kleinrok, Robert Gil, Maciej Lesiak, Jarosław Drożdż, Aldona Kubica, Krzysztof J Filipiak, Jarosław Kaźmierczak, Aleksander Goch, Stefan Grajek, Andrzej Basiński, Łukasz Szarpak, Grzegorz Grześk, Piotr Hoffman, Wojciech Wojakowski, Zbigniew Gąsior, Sławomir Dobrzycki, Jolanta M Siller-Matula, Adam Witkowski, Wiktor Kuliczkowski, Marcin Gruchała, Dariusz Timler, Grzegorz Opolski, Dariusz Dudek, Jacek Legutko, Marzenna Zielińska, Jarosław Wójcik
No abstract text is available yet for this article.
2017: Kardiologia Polska
https://www.readbyqxmd.com/read/28237630/-18-f-fluoro-2-deoxyglucose-pet-informs-neutrophil-accumulation-and-activation-in-lipopolysaccharide-induced-acute-lung-injury
#3
Rosana S Rodrigues, Fernando A Bozza, Christopher J Hanrahan, Li-Ming Wang, Qi Wu, John M Hoffman, Guy A Zimmerman, Kathryn A Morton
INTRODUCTION: Molecular imaging of the earliest events related to the development of acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) could facilitate therapeutic development and patient management. We previously reported that (18)F-fluoro-2-deoxyglucose ((18)F-FDG) PET identifies ALI/ARDS prior to radiographic abnormalities. The purpose of this study was to establish the time courses of (18)F-FDG uptake, edema and neutrophil recruitment in an endotoxin-induced acute lung injury model and to examine molecular events required for (14)C-2DG uptake in activated neutrophils...
May 2017: Nuclear Medicine and Biology
https://www.readbyqxmd.com/read/28094040/a-phase-2-study-of-alisertib-mln8237-in-recurrent-or-persistent-uterine-leiomyosarcoma-an-nrg-oncology-gynecologic-oncology-group-study-0231d
#4
David M Hyman, Michael W Sill, Heather A Lankes, Richard Piekarz, Mark S Shahin, Mildred R Ridgway, Floor Backes, Meaghen E Tenney, Cara A Mathews, James S Hoffman, Carol Aghajanian, Martee L Hensley
OBJECTIVE: This two-stage Phase II study assessed the activity of single agent alisertib in patients with recurrent/persistent uterine leiomyosarcoma (uLMS). METHODS: Eligibility criteria included histologically-confirmed, recurrent or persistent uLMS, age≥18, 1-2 prior cytotoxic regimens, and RECIST version 1.1 measurable disease. The primary objective of the study was to evaluate the efficacy of alisertib through the frequency of patients with objective tumor responses and the frequency who survived event-free for at least 6months (EFS6)...
January 2017: Gynecologic Oncology
https://www.readbyqxmd.com/read/28067909/smchd1-mutations-associated-with-a-rare-muscular-dystrophy-can-also-cause-isolated-arhinia-and-bosma-arhinia-microphthalmia-syndrome
#5
Natalie D Shaw, Harrison Brand, Zachary A Kupchinsky, Hemant Bengani, Lacey Plummer, Takako I Jones, Serkan Erdin, Kathleen A Williamson, Joe Rainger, Alexei Stortchevoi, Kaitlin Samocha, Benjamin B Currall, Donncha S Dunican, Ryan L Collins, Jason R Willer, Angela Lek, Monkol Lek, Malik Nassan, Shahrin Pereira, Tammy Kammin, Diane Lucente, Alexandra Silva, Catarina M Seabra, Colby Chiang, Yu An, Morad Ansari, Jacqueline K Rainger, Shelagh Joss, Jill Clayton Smith, Margaret F Lippincott, Sylvia S Singh, Nirav Patel, Jenny W Jing, Jennifer R Law, Nalton Ferraro, Alain Verloes, Anita Rauch, Katharina Steindl, Markus Zweier, Ianina Scheer, Daisuke Sato, Nobuhiko Okamoto, Christina Jacobsen, Jeanie Tryggestad, Steven Chernausek, Lisa A Schimmenti, Benjamin Brasseur, Claudia Cesaretti, Jose E García-Ortiz, Tatiana Pineda Buitrago, Orlando Perez Silva, Jodi D Hoffman, Wolfgang Mühlbauer, Klaus W Ruprecht, Bart L Loeys, Masato Shino, Angela M Kaindl, Chie-Hee Cho, Cynthia C Morton, Richard R Meehan, Veronica van Heyningen, Eric C Liao, Ravikumar Balasubramanian, Janet E Hall, Stephanie B Seminara, Daniel Macarthur, Steven A Moore, Koh-Ichiro Yoshiura, James F Gusella, Joseph A Marsh, John M Graham, Angela E Lin, Nicholas Katsanis, Peter L Jones, William F Crowley, Erica E Davis, David R FitzPatrick, Michael E Talkowski
Arhinia, or absence of the nose, is a rare malformation of unknown etiology that is often accompanied by ocular and reproductive defects. Sequencing of 40 people with arhinia revealed that 84% of probands harbor a missense mutation localized to a constrained region of SMCHD1 encompassing the ATPase domain. SMCHD1 mutations cause facioscapulohumeral muscular dystrophy type 2 (FSHD2) via a trans-acting loss-of-function epigenetic mechanism. We discovered shared mutations and comparable DNA hypomethylation patterning between these distinct disorders...
February 2017: Nature Genetics
https://www.readbyqxmd.com/read/28012642/postoperative-cerebral-and-somatic-near-infrared-spectroscopy-saturations-and-outcome-in-hypoplastic-left-heart-syndrome
#6
George M Hoffman, Nancy S Ghanayem, John P Scott, James S Tweddell, Michael E Mitchell, Kathleen A Mussatto
BACKGROUND: Circulatory vulnerability reflected by low systemic venous oxygen saturation after surgical palliation of hypoplastic left heart syndrome predicts adverse neurologic outcome and reduced survival, and targeting venous saturation may improve outcome. We herein test the hypothesis that near-infrared spectroscopy (NIRS)-derived cerebral and somatic/renal regional saturations can predict survival. METHODS: Patient data, from a prospective Institutional Review Board-approved registry of hemodynamic measures after initial palliation of hypoplastic left heart syndrome, were analyzed with logistic and multivariable mixed regression methods to determine relationships between standard hemodynamic measures, direct and NIRS measures of saturation, and outcome...
December 21, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27811147/development-of-the-autoinflammatory-disease-damage-index-addi
#7
Nienke M Ter Haar, Kim V Annink, Sulaiman M Al-Mayouf, Gayane Amaryan, Jordi Anton, Karyl S Barron, Susanne M Benseler, Paul A Brogan, Luca Cantarini, Marco Cattalini, Alexis-Virgil Cochino, Fabrizio De Benedetti, Fatma Dedeoglu, Adriana A De Jesus, Ornella Della Casa Alberighi, Erkan Demirkaya, Pavla Dolezalova, Karen L Durrant, Giovanna Fabio, Romina Gallizzi, Raphaela Goldbach-Mansky, Eric Hachulla, Veronique Hentgen, Troels Herlin, Michaël Hofer, Hal M Hoffman, Antonella Insalaco, Annette F Jansson, Tilmann Kallinich, Isabelle Koné-Paut, Anna Kozlova, Jasmin B Kuemmerle-Deschner, Helen J Lachmann, Ronald M Laxer, Alberto Martini, Susan Nielsen, Irina Nikishina, Amanda K Ombrello, Seza Ozen, Efimia Papadopoulou-Alataki, Pierre Quartier, Donato Rigante, Ricardo Russo, Anna Simon, Maria Trachana, Yosef Uziel, Angelo Ravelli, Marco Gattorno, Joost Frenkel
OBJECTIVES: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency...
November 3, 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27728221/a-case-of-hoffmans-syndrome
#8
R M Verghese, S K Singh, A K Abott, Y Singh
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27521439/phenotypic-spectrum-of-gabra1-from-generalized-epilepsies-to-severe-epileptic-encephalopathies
#9
Katrine Johannesen, Carla Marini, Siona Pfeffer, Rikke S Møller, Thomas Dorn, Cristina Elena Niturad, Elena Gardella, Yvonne Weber, Marianne Søndergård, Helle Hjalgrim, Mariana Nikanorova, Felicitas Becker, Line H G Larsen, Hans A Dahl, Oliver Maier, Davide Mei, Saskia Biskup, Karl M Klein, Philipp S Reif, Felix Rosenow, Abdallah F Elias, Cindy Hudson, Katherine L Helbig, Susanne Schubert-Bast, Maria R Scordo, Dana Craiu, Tania Djémié, Dorota Hoffman-Zacharska, Hande Caglayan, Ingo Helbig, Jose Serratosa, Pasquale Striano, Peter De Jonghe, Sarah Weckhuysen, Arvid Suls, Kai Muru, Inga Talvik, Tiina Talvik, Hiltrud Muhle, Ingo Borggraefe, Imma Rost, Renzo Guerrini, Holger Lerche, Johannes R Lemke, Guido Rubboli, Snezana Maljevic
OBJECTIVE: To delineate phenotypic heterogeneity, we describe the clinical features of a cohort of patients with GABRA1 gene mutations. METHODS: Patients with GABRA1 mutations were ascertained through an international collaboration. Clinical, EEG, and genetic data were collected. Functional analysis of 4 selected mutations was performed using the Xenopus laevis oocyte expression system. RESULTS: The study included 16 novel probands and 3 additional family members with a disease-causing mutation in the GABRA1 gene...
September 13, 2016: Neurology
https://www.readbyqxmd.com/read/27424812/antiretroviral-therapy-for-the-prevention-of-hiv-1-transmission
#10
RANDOMIZED CONTROLLED TRIAL
Myron S Cohen, Ying Q Chen, Marybeth McCauley, Theresa Gamble, Mina C Hosseinipour, Nagalingeswaran Kumarasamy, James G Hakim, Johnstone Kumwenda, Beatriz Grinsztejn, Jose H S Pilotto, Sheela V Godbole, Suwat Chariyalertsak, Breno R Santos, Kenneth H Mayer, Irving F Hoffman, Susan H Eshleman, Estelle Piwowar-Manning, Leslie Cottle, Xinyi C Zhang, Joseph Makhema, Lisa A Mills, Ravindre Panchia, Sharlaa Faesen, Joseph Eron, Joel Gallant, Diane Havlir, Susan Swindells, Vanessa Elharrar, David Burns, Taha E Taha, Karin Nielsen-Saines, David D Celentano, Max Essex, Sarah E Hudelson, Andrew D Redd, Thomas R Fleming
BACKGROUND: An interim analysis of data from the HIV Prevention Trials Network (HPTN) 052 trial showed that antiretroviral therapy (ART) prevented more than 96% of genetically linked infections caused by human immunodeficiency virus type 1 (HIV-1) in serodiscordant couples. ART was then offered to all patients with HIV-1 infection (index participants). The study included more than 5 years of follow-up to assess the durability of such therapy for the prevention of HIV-1 transmission. METHODS: We randomly assigned 1763 index participants to receive either early or delayed ART...
September 1, 2016: New England Journal of Medicine
https://www.readbyqxmd.com/read/27208704/a-computational-approach-to-early-sepsis-detection
#11
Jacob S Calvert, Daniel A Price, Uli K Chettipally, Christopher W Barton, Mitchell D Feldman, Jana L Hoffman, Melissa Jay, Ritankar Das
OBJECTIVE: To develop high-performance early sepsis prediction technology for the general patient population. METHODS: Retrospective analysis of adult patients admitted to the intensive care unit (from the MIMIC II dataset) who were not septic at the time of admission. RESULTS: A sepsis early warning algorithm, InSight, was developed and applied to the prediction of sepsis up to three hours prior to a patient's first five hour Systemic Inflammatory Response Syndrome (SIRS) episode...
July 1, 2016: Computers in Biology and Medicine
https://www.readbyqxmd.com/read/26542436/effect-of-intercurrent-operation-and-cerebral-oxygenation-on-developmental-trajectory-in-congenital-heart-disease
#12
George M Hoffman, Cheryl L Brosig, Laurel M Bear, James S Tweddell, Kathleen A Mussatto
BACKGROUND: Children with congenital heart disease are at increased risk of abnormal neurodevelopment (ND). Demographic and perioperative physiologic factors have both been associated with developmental outcome. The acute physiologic effect of a surgical procedure, anesthesia, and hospitalization may offset any potential advantage gained from anatomic correction and circulatory palliation. The specific risk/benefit balance on ND outcome of the insult of the operation, offset by the benefit of improved anatomy and physiology, has not been addressed...
February 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/26107372/the-actn3-r577x-polymorphism-is-associated-with-cardiometabolic-fitness-in-healthy-young-adults
#13
Chelsea L Deschamps, Kimberly E Connors, Matthias S Klein, Virginia L Johnsen, Jane Shearer, Hans J Vogel, Joseph M Devaney, Heather Gordish-Dressman, Gina M Many, Whitney Barfield, Eric P Hoffman, William E Kraus, Dustin S Hittel
Homozygosity for a premature stop codon (X) in the ACTN3 "sprinter" gene is common in humans despite the fact that it reduces muscle size, strength and power. Because of the close relationship between skeletal muscle function and cardiometabolic health we examined the influence of ACTN3 R577X polymorphism over cardiovascular and metabolic characteristics of young adults (n = 98 males, n = 102 females; 23 ± 4.2 years) from our Assessing Inherent Markers for Metabolic syndrome in the Young (AIMMY) study. Both males and females with the RR vs XX genotype achieved higher mean VO2 peak scores (47...
2015: PloS One
https://www.readbyqxmd.com/read/26056255/tr1-cells-but-not-foxp3-regulatory-t-cells-suppress-nlrp3-inflammasome-activation-via-an-il-10-dependent-mechanism
#14
Yu Yao, Jens Vent-Schmidt, Matthew D McGeough, May Wong, Hal M Hoffman, Theodore S Steiner, Megan K Levings
The two best-characterized types of CD4(+) regulatory T cells (Tregs) are Foxp3(+) Tregs and Foxp3(-) type 1 regulatory (Tr1) cells. The ability of Foxp3(+) Tregs and Tr1 cells to suppress adaptive immune responses is well known, but how these cells regulate innate immunity is less defined. We discovered that CD44(hi)Foxp3(-) T cells from unmanipulated mice are enriched in Tr1 cell precursors, enabling differentiation of cells that express IL-10, as well as Tr1-associated cell surface markers, CD49b and LAG-3, and transcription factors, cMaf, Blimp-1, and AhR...
July 15, 2015: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/25986310/extracorporeal-treatment-for-salicylate-poisoning-systematic-review-and-recommendations-from-the-extrip-workgroup
#15
REVIEW
David N Juurlink, Sophie Gosselin, Jan T Kielstein, Marc Ghannoum, Valéry Lavergne, Thomas D Nolin, Robert S Hoffman
STUDY OBJECTIVE: Salicylate poisoning is a challenging clinical entity associated with substantial morbidity and mortality. The indications for extracorporeal treatments such as hemodialysis are poorly defined. We present a systematic review of the literature along with evidence- and consensus-based recommendations on the use of extracorporeal treatment in salicylate poisoning. METHODS: The Extracorporeal Treatments in Poisoning (EXTRIP) Workgroup is a multidisciplinary group with international representation whose aim is to provide evidence-based recommendations on the use of extracorporeal treatments in poisoning...
August 2015: Annals of Emergency Medicine
https://www.readbyqxmd.com/read/25695144/ice-water-submersion-for-rapid-cooling-in-severe-drug-induced-hyperthermia
#16
Larissa K Laskowski, Adaira Landry, Susi U Vassallo, Robert S Hoffman
CONTEXT: The optimal method of cooling hyperthermic patients is controversial. Although controlled data support ice water submersion, many authorities recommend a mist and fan technique. We report two patients with drug-induced hyperthermia, to demonstrate the rapid cooling rates of ice water submersion. CASE DETAILS: Case 1. A 27-year-old man presented with a sympathomimetic toxic syndrome and a core temperature of 41.4°C after ingesting 4-fluoroamphetamine. He was submerged in ice water and his core temperature fell to 38°C within 18 minutes (a mean cooling rate of 0...
March 2015: Clinical Toxicology
https://www.readbyqxmd.com/read/25423351/the-inflammasomes-and-autoinflammatory-syndromes
#17
REVIEW
Lori Broderick, Dominic De Nardo, Bernardo S Franklin, Hal M Hoffman, Eicke Latz
Inflammation, a vital response of the immune system to infection and damage to tissues, can be initiated by various germline-encoded innate immune-signaling receptors. Among these, the inflammasomes are critical for activation of the potent proinflammatory interleukin-1 cytokine family. Additionally, inflammasomes can trigger and maintain inflammatory responses aimed toward excess nutrients and the numerous danger signals that appear in a variety of chronic inflammatory diseases. We discuss our understanding of how inflammasomes assemble to trigger caspase-1 activation and subsequent cytokine release, describe how genetic mutations in inflammasome-related genes lead to autoinflammatory syndromes, and review the contribution of inflammasome activation to various pathologies arising from metabolic dysfunction...
2015: Annual Review of Pathology
https://www.readbyqxmd.com/read/24852785/dissecting-cellulitis-perifolliculitis-capitis-abscedens-et-suffodiens-a-comprehensive-review-focusing-on-new-treatments-and-findings-of-the-last-decade-with-commentary-comparing-the-therapies-and-causes-of-dissecting-cellulitis-to-hidradenitis-suppurativa
#18
REVIEW
Noah Scheinfeld
Dissecting cellulitis (DC) also referred to as to as perifolliculitis capitis abscedens et suffodiens (Hoffman) manifests with perifollicular pustules, nodules, abscesses and sinuses that evolve into scarring alopecia. In the U.S., it predominantly occurs in African American men between 20-40 years of age. DC also occurs in other races and women more rarely. DC has been reported worldwide. Older therapies reported effective include: low dose oral zinc, isotretinoin, minocycline, sulfa drugs, tetracycline, prednisone, intralesional triamcinolone, incision and drainage, dapsone, antiandrogens (in women), topical clindamycin, topical isotretinoin, X-ray epilation and ablation, ablative C02 lasers, hair removal lasers (800nm and 694nm), and surgical excision...
May 16, 2014: Dermatology Online Journal
https://www.readbyqxmd.com/read/24738941/lymphoma-associated-hemophagocytic-syndrome-it-s-going-viral
#19
COMMENT
James Hoffman, Izidore S Lossos
No abstract text is available yet for this article.
September 2014: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/24602844/effects-of-early-versus-delayed-initiation-of-antiretroviral-treatment-on-clinical-outcomes-of-hiv-1-infection-results-from-the-phase-3-hptn-052-randomised-controlled-trial
#20
RANDOMIZED CONTROLLED TRIAL
Beatriz Grinsztejn, Mina C Hosseinipour, Heather J Ribaudo, Susan Swindells, Joseph Eron, Ying Q Chen, Lei Wang, San-San Ou, Maija Anderson, Marybeth McCauley, Theresa Gamble, Nagalingeshwaran Kumarasamy, James G Hakim, Johnstone Kumwenda, Jose H S Pilotto, Sheela V Godbole, Suwat Chariyalertsak, Marineide Gonçalves de Melo, Kenneth H Mayer, Susan H Eshleman, Estelle Piwowar-Manning, Joseph Makhema, Lisa A Mills, Ravindre Panchia, Ian Sanne, Joel Gallant, Irving Hoffman, Taha E Taha, Karin Nielsen-Saines, David Celentano, Max Essex, Diane Havlir, Myron S Cohen
BACKGROUND: Use of antiretroviral treatment for HIV-1 infection has decreased AIDS-related morbidity and mortality and prevents sexual transmission of HIV-1. However, the best time to initiate antiretroviral treatment to reduce progression of HIV-1 infection or non-AIDS clinical events is unknown. We reported previously that early antiretroviral treatment reduced HIV-1 transmission by 96%. We aimed to compare the effects of early and delayed initiation of antiretroviral treatment on clinical outcomes...
April 2014: Lancet Infectious Diseases
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