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https://www.readbyqxmd.com/read/27911804/familial-mediterranean-fever-mutations-lift-the-obligatory-requirement-for-microtubules-in-pyrin-inflammasome-activation
#1
Hanne Van Gorp, Pedro H V Saavedra, Nathalia M de Vasconcelos, Nina Van Opdenbosch, Lieselotte Vande Walle, Magdalena Matusiak, Giusi Prencipe, Antonella Insalaco, Filip Van Hauwermeiren, Dieter Demon, Delfien J Bogaert, Melissa Dullaers, Elfride De Baere, Tino Hochepied, Joke Dehoorne, Karim Y Vermaelen, Filomeen Haerynck, Fabrizio De Benedetti, Mohamed Lamkanfi
Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease worldwide. It is caused by mutations in the inflammasome adaptor Pyrin, but how FMF mutations alter signaling in FMF patients is unknown. Herein, we establish Clostridium difficile and its enterotoxin A (TcdA) as Pyrin-activating agents and show that wild-type and FMF Pyrin are differentially controlled by microtubules. Diverse microtubule assembly inhibitors prevented Pyrin-mediated caspase-1 activation and secretion of IL-1β and IL-18 from mouse macrophages and human peripheral blood mononuclear cells (PBMCs)...
November 22, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27910184/clinical-significance-of-pre-transplant-circulating-cd3-cd4-cd161-cell-frequency-on-the-occurrence-of-neutropenic-infections-after-allogeneic-stem-cell-transplantation
#2
Tae Woo Kim, Sung-Eun Lee, Ji-Young Lim, Da-Bin Ryu, Young-Woo Jeon, Jae-Ho Yoon, Byung-Sik Cho, Ki-Seong Eom, Yoo-Jin Kim, Hee-Je Kim, Seok Lee, Seok-Goo Cho, Dong-Wook Kim, Jong Wook Lee, Woo-Sung Min, Chang-Ki Min
BACKGROUND: Few studies have been performed to identify factors that are associated with an increased risk of infections during the neutropenic period in patients undergoing allogeneic stem cell transplantation (allo-SCT). The aim of this study was to identify the host immune cells responsible for infections before engraftment. METHODS: A total of 282 patients who underwent allo-SCT were enrolled. Peripheral blood samples were collected before conditioning therapy...
December 2, 2016: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/27907248/mycobacterium-ulcerans-toxin-mycolactone-may-enhance-host-seeking-and-oviposition-behavior-by-aedes-aegypti-l-diptera-culicidae
#3
M L Sanders, H R Jordan, C Serewis-Pond, L Zheng, M E Benbow, P L Small, J K Tomberlin
The ecological functions of many toxins continue to remain unknown for those produced by environmental pathogens. Mycobacterium ulcerans, the causative agent of the neglected tropical disease, Buruli ulcer, produces a cytotoxic macrolide, mycolactone, whose function(s) in the environment remains elusive. Through a series of dual-choice behavior assays, we show that mycolactone may be an interkingdom cue for the yellow fever mosquito, Aedes aegypti, seeking blood-meals as well as oviposition sites. Results provide novel insight into the evolution between bacteria and potential vectors...
December 1, 2016: Environmental Microbiology
https://www.readbyqxmd.com/read/27903264/adult-onset-still-s-disease-evaluation-of-prognostic-tools-and-validation-of-the-systemic-score-by-analysis-of-100-cases-from-three-centers
#4
Piero Ruscitti, Paola Cipriani, Francesco Masedu, Daniela Iacono, Francesco Ciccia, Vasiliki Liakouli, Giuliana Guggino, Francesco Carubbi, Onorina Berardicurti, Paola Di Benedetto, Marco Valenti, Giovanni Triolo, Gabriele Valentini, Roberto Giacomelli
BACKGROUND: Adult-onset Still's disease (AOSD) is rare inflammatory disease of unknown etiology that usually affects young adults. The more common clinical manifestations are spiking fevers, arthritis, evanescent rash, elevated liver enzymes, lymphadenopathy, hepatosplenomegaly, and serositis. The multi-visceral involvement of the disease and the different complications, such as macrophage activation syndrome, may strongly decrease the life expectancy of AOSD patients. METHODS: This study aimed to identify the positive and negative features correlated with the outcome of patients...
December 1, 2016: BMC Medicine
https://www.readbyqxmd.com/read/27899390/hyper-igd-and-periodic-fever-syndrome-hids-due-to-compound-heterozygosity-for-g336s-and-v377i-in-a-44-year-old-patient-with-a-27-year-history-of-fever
#5
Stefan Schlabe, Carolynne Schwarze-Zander, Peter Lohse, Jürgen Kurt Rockstroh
Hereditary autoinflammatory syndromes are a rare, but notable cause of fever of unknown origin. During the last few years, the knowledge of the genetic background has significantly increased. Here, we report a novel pathogenic mutation in the MVK gene as the cause of fever in a 44-year-old male patient with a history of fever over a period of 27 years.
November 29, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27894446/fever-of-unknown-origin-in-childhood
#6
REVIEW
Michael J Chusid
Childhood fever of unknown origin (FUO) is most often related to an underlying infection but can also be associated with a variety of neoplastic, rheumatologic, and inflammatory conditions. Repeated, focused reviews of patient history and physical examination are often helpful in suggesting a likely diagnosis. Diagnostic workup should be staged, usually leaving invasive testing for last. Advances in molecular genetic techniques have increased the importance of these assays in the diagnosis of FUO in children...
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27890079/detection-of-theileria-parva-in-tissues-of-cattle-undergoing-severe-east-coast-fever-disease-show-significant-parasite-dna-accumulation-in-the-spleen
#7
Cassandra L Olds, Tasha Paul, Glen A Scoles
Infiltration and proliferation of Theileria parva infected lymphocytes in bovine host lymphoid organs is one of the hallmarks of T. parva infection. The relative abundance of parasites within infected host tissues, both lymphoid and non-lymphoid is however unknown. Using quantitative PCR, we have shown that significantly higher concentrations of T. parva DNA are detected in the spleens of cattle undergoing severe disease compared to other organs.
December 15, 2016: Veterinary Parasitology
https://www.readbyqxmd.com/read/27889778/primary-intestinal-hodgkin-lymphoma-mimicking-intraabdominal-abscess-in-a-renal-transplant-recipient-a-case-report
#8
Biljana Mihaljevic, Darko Antic, Vojin Vukovic, Maja Perunicic Jovanovic, Visnja Lezaic, Nemanja Zaric, Mirko Kerkez, Vladimir Djordjevic
INTRODUCTION: Post-transplant lymphoproliferative disease (PTLD) comprises a variety of lymphoid and plasma cell disorders arising in patients with a solid organ transplant. Monomorphic lymphomas represent the most significant part of this wide spectrum, with the overall risk rising with the aggressiveness of lymphoid proliferation in comparison to the general population. The development of Hodgkin lymphoma is very rare in transplant recipients, comprising less than 6% of all monomorphic PTLD, while cases of primary intestinal Hodgkin lymphoma in these circumstances are anecdotal...
2016: Oncology Research and Treatment
https://www.readbyqxmd.com/read/27888598/adult-onset-still-s-disease-with-different-antibodies-a-case-report-and-review-of-literature
#9
Maryam Mobini, Roya Ghasemian, Fatemeh Zameni
 Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. There is not currently any specific serological markers for AOSD , and  diagnosis still relying on the exclusion of other likely diagnoses. Yamaguchi's criteria are used as a diagnostic criterion which contains negative serologic markers for other collagen vascular diseases including systemic lupus erythematosus and rheumatoid arthritis. Here we report a 28-year-old woman with arthralgia, fever, rash, leukocytosis, lymphadenopathy, sore throat, abnormal liver function and negative rheumatoid factor and ANA but  seropositive for anti-CCP, anti-dsDNA, and C-ANCA...
October 2016: Acta Medica Iranica
https://www.readbyqxmd.com/read/27884575/brugada-pattern-caused-by-a-flecainide-overdose
#10
Elena Tessitore, Majd Ramlawi, Olivier Tobler, Henri Sunthorn
BACKGROUND: Brugada pattern can be found on the electrocardiogram (ECG) of patients with altered mental status, usually with fever or drug intoxication. Diagnosis remains challenging, because the ECG changes are dynamic and variable. In addition, triggers are not always clearly identified. In patients with atrial fibrillation (AF), the use of class IC antidysrhythmic drugs can unmask a Brugada pattern on the ECG, especially if combined with other medications acting on sodium channels...
November 21, 2016: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27872770/herpes-simplex-virus-hepatitis-in-an-immunocompetent-host-resembling-hepatic-pyogenic-abscesses
#11
Carrie Down, Amit Mehta, Gayle Salama, Erika Hissong, Russell Rosenblatt, Michael Cantor, David Helfgott, Kristen Marks
Herpes simplex virus (HSV) hepatitis represents a rare complication of HSV infection, which can progress to acute liver failure and, in some cases, death. We describe an immunocompetent 67-year-old male who presented with one week of fever and abdominal pain. Computed tomography (CT) scan and magnetic resonance imaging (MRI) of the abdomen showed multiple bilobar hepatic lesions, some with rim enhancement, compatible with liver abscesses. Subsequent liver biopsy, however, revealed hepatocellular necrosis, HSV-type intranuclear inclusions, and immunostaining positive for herpes virus type 2 (HSV-2)...
2016: Case Reports in Hepatology
https://www.readbyqxmd.com/read/27867459/successful-treatment-of-giant-left-subclavian-artery-pseudoaneurysm-abutting-the-arch-of-the-aorta-and-descending-aorta
#12
Hamza Abdur Rahim Khan, Sehrish Batool, Fazal Wahab Khan, Saulat Hasnain Fatimi
A 28-year-old woman presented with 3-month history of fever of unknown origin and progressively increasing cough. She was diagnosed with pulmonary tuberculosis on bronchial lavage cultures. A chest X-ray performed on follow-up showed a new opacity in the left apical area of the chest. Computed tomography scan of chest showed a large 10 cm pseudoaneurysm of the left subclavian artery 1 cm from its take off from the arch of the aorta. The pseudoaneurysm was approached through a left posterolateral thoracotomy and opened following a proximal and distal control...
July 2016: Heart Views: the Official Journal of the Gulf Heart Association
https://www.readbyqxmd.com/read/27860302/clinical-and-genetic-association-radiological-findings-and-response-to-biological-therapy-in-seven-children-from-qatar-with-non-bacterial-osteomyelitis
#13
Taha Moussa, Venkatraman Bhat, Vishwanatha Kini, Basil M Fathalla
AIM: We aim to report the clinical manifestations, genetic testing results, magnetic resonance imaging (MRI) findings and biologics used in the management of non-bacterial osteomyelitis in our center. METHODS: We conducted a retrospective review of medical records. A previously proposed classification was implemented as follows: chronic recurrent multifocal osteomyelitis (CRMO), chronic non-bacterial osteomyelitis (CNBO) and acute non-bacterial osteomyelitis. RESULTS: Four females and three males with a median age at presentation of 6 years (6 months-14 years) presented with arthralgia (7/7), back pain (4/7), arthritis (4/7) and bone pain (2/7)...
November 9, 2016: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/27857926/intracerebral-hemorrhagic-infarction-after-cranioplasty-in-a-patient-with-sinking-skin-flap-syndrome
#14
Jai-Hyuck Han, Mou-Seop Lee
The sinking skin flap syndrome (SSFS) is a rare complication after a large craniectomy. Hemorrhage infarction after a cranioplasty is a very rare complication with only 4 cases to date. We report a case of the patient who underwent an autologous cranioplasty to treat SSFS that developed intracerebral hemorrhage infarction. A 20-year-old male was admitted to our emergency department with stuporous mentality. Emergent decompressive craniectomy (DC) have done. He had suffered from SSFS and fever of unknown origin (FUO) since DC...
October 2016: Korean Journal of Neurotrauma
https://www.readbyqxmd.com/read/27857837/autoimmune-polyendocrinopathy-and-hypophysitis-after-puumala-hantavirus-infection
#15
Marlene Tarvainen, Satu Mäkelä, Jukka Mustonen, Pia Jaatinen
: Puumala hantavirus (PUUV) infection causes nephropathia epidemica (NE), a relatively mild form of haemorrhagic fever with renal syndrome (HFRS). Hypophyseal haemorrhage and hypopituitarism have been described in case reports on patients with acute NE. Chronic hypopituitarism diagnosed months or years after the acute illness has also been reported, without any signs of a haemorrhagic aetiology. The mechanisms leading to the late-onset hormonal defects remain unknown. Here, we present a case of NE-associated autoimmune polyendocrinopathy and hypopituitarism presumably due to autoimmune hypophysitis...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27857032/eosinophilic-granulomatosis-with-polyangiitis-presenting-with-skin-rashes-eosinophilic-cholecystitis-and-retinal-vasculitis
#16
Mingbing Zeng, Xialin Liu, Yizhi Liu
BACKGROUND Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome (CSS), is a rare vasculitis of unknown etiology. Most of the patients have a long history of asthma and then develop autoimmune inflammation of small and medium-sized blood vessels, with consequent reduction of blood flow to various organs and tissues. EGPA can cause disorders in multiple systems; the most seriously affected organs are the retina, kidney, brain, cardiovascular system, and skin. CASE REPORT The patient was hospitalized for high fever and skin rashes and then developed right upper abdominal pain, decreased visual acuity, coma, and convulsions...
November 18, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27855227/evidence-that-processing-of-the-severe-fever-with-thrombocytopenia-syndrome-virus-gn-gc-polyprotein-is-critical-for-viral-infectivity-and-requires-an-internal-gc-signal-peptide
#17
Teresa Plegge, Heike Hofmann-Winkler, Martin Spiegel, Stefan Pöhlmann
The severe fever with thrombocytopenia syndrome virus (SFTSV) is an emerging, highly pathogenic bunyavirus against which neither antivirals nor vaccines are available. The SFTSV glycoproteins, Gn and Gc, facilitate viral entry into host cells. Gn and Gc are generated from a precursor protein, Gn/Gc, but it is currently unknown how the precursor is converted into the single proteins and whether this process is required for viral infectivity. Employing a rhabdoviral pseudotyping system, we demonstrate that a predicted signal sequence at the N-terminus of Gc is required for Gn/Gc processing and viral infectivity while potential proprotein convertase cleavage sites in Gc are dispensable...
2016: PloS One
https://www.readbyqxmd.com/read/27849599/yellow-fever-virus-capsid-protein-is-a-potent-suppressor-of-rna-silencing-that-binds-double-stranded-rna
#18
Glady Hazitha Samuel, Michael R Wiley, Atif Badawi, Zach N Adelman, Kevin M Myles
Mosquito-borne flaviviruses, including yellow fever virus (YFV), Zika virus (ZIKV), and West Nile virus (WNV), profoundly affect human health. The successful transmission of these viruses to a human host depends on the pathogen's ability to overcome a potentially sterilizing immune response in the vector mosquito. Similar to other invertebrate animals and plants, the mosquito's RNA silencing pathway comprises its primary antiviral defense. Although a diverse range of plant and insect viruses has been found to encode suppressors of RNA silencing, the mechanisms by which flaviviruses antagonize antiviral small RNA pathways in disease vectors are unknown...
November 29, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27843864/bilateral-painful-parotid-lumps-and-a-lump-in-the-groin-an-uncommon-presentation-of-common-kikuchi-s-disease
#19
Sumeet Prakash Mirgh, Jinendra Satiya, Jehangir Soli Sorabjee
Kikuchi-Fujimoto disease (KFD) is an under-recognized disease most commonly presenting with cervical lymphadenopathy, fever, and cytopenias in young females. Bilateral parotid enlargement is usually caused by infections (e.g., mumps) and autoimmune conditions (e.g., Sjogren syndrome). Parotid enlargement, inguinal lymphadenopathy, and pyrexia of unknown origin are uncommon presenting features of KFD and should be suspected in the appropriate setting.
April 2016: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/27832191/dengue-virus-infection-is-through-a-cooperative-interaction-between-a-mannose-receptor-and-clec5a-on-macrophage-as-a-multivalent-hetero-complex
#20
Yen-Lung Lo, Gunn-Guang Liou, Jia-Huei Lyu, Michael Hsiao, Tsui-Ling Hsu, Chi-Huey Wong
Dengue fever is a mosquito-borne viral pandemic disease that is widespread in the tropical and subtropical areas. Dengue virus uses human mannose-binding receptor (MR) and DC-SIGN on macrophages as primary receptors, and CLEC5A as signaling receptor to sense the dengue virus invasion and then to signal and stimulate macrophages to secrete cytokines. But the interplay between MR/DC-SIGN and CLEC5A is unknown. Here we demonstrate a plausible mechanism for the interaction, i.e. MR/DC-SIGN first attracts the virus with high avidity, and the virus concurrently interacts with CLEC5A in close proximity to form a multivalent hetero-complex and facilitate CLEC5A-mediated signal transduction...
2016: PloS One
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