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https://www.readbyqxmd.com/read/29036198/the-homeodomain-interacting-protein-kinase-hpk-1-preserves-protein-homeostasis-and-longevity-through-master-regulatory-control-of-the-hsf-1-chaperone-network-and-torc1-restricted-autophagy-in-caenorhabditis-elegans
#1
Ritika Das, Justine A Melo, Manjunatha Thondamal, Elizabeth A Morton, Adam B Cornwell, Beresford Crick, Joung Heon Kim, Elliot W Swartz, Todd Lamitina, Peter M Douglas, Andrew V Samuelson
An extensive proteostatic network comprised of molecular chaperones and protein clearance mechanisms functions collectively to preserve the integrity and resiliency of the proteome. The efficacy of this network deteriorates during aging, coinciding with many clinical manifestations, including protein aggregation diseases of the nervous system. A decline in proteostasis can be delayed through the activation of cytoprotective transcriptional responses, which are sensitive to environmental stress and internal metabolic and physiological cues...
October 16, 2017: PLoS Genetics
https://www.readbyqxmd.com/read/29026069/id3-regulates-the-mdc1-mediated-dna-damage-response-in-order-to-maintain-genome-stability
#2
Jung-Hee Lee, Seon-Joo Park, Gurusamy Hariharasudhan, Min-Ji Kim, Sung Mi Jung, Seo-Yeon Jeong, In-Youb Chang, Cheolhee Kim, Eunae Kim, Jihyeon Yu, Sangsu Bae, Ho Jin You
MDC1 plays a critical role in the DNA damage response (DDR) by interacting directly with several factors including γ-H2AX. However, the mechanism by which MDC1 is recruited to damaged sites remains elusive. Here, we show that MDC1 interacts with a helix-loop-helix (HLH)-containing protein called inhibitor of DNA-binding 3 (ID3). In response to double-strand breaks (DSBs) in the genome, ATM phosphorylates ID3 at serine 65 within the HLH motif, and this modification allows a direct interaction with MDC1. Moreover, depletion of ID3 results in impaired formation of ionizing radiation (IR)-induced MDC1 foci, suppression of γ-H2AX-bound MDC1, impaired DSB repair, cellular hypersensitivity to IR, and genomic instability...
October 12, 2017: Nature Communications
https://www.readbyqxmd.com/read/29025045/clinical-features-and-outcomes-in-secondary-adult-hemophagocytic-lymphohistiocytosis
#3
Meng Zhou, Li Li, Qiaolei Zhang, Shanshan Ma, Jianai Sun, Lixia Zhu, Danlei Lu, Jingjing Zhu, De Zhou, Yanlong Zheng, Xiudi Yang, Mixue Xie, Mingyu Zhu, Xiujin Ye, Wanzhuo Xie
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by an infrequent but immune-mediated life-threatening disease, with confusing clinical manifestations, rapidly deteriorating health, high morbidity and mortality and challenging diagnosis. METHODS: To improve the recognition and understanding of this disease, we analyzed clinical and laboratory findings and prognostic factors from 205 adult patients diagnosed with HLH in a large cohort managed at a single medical center from January 2011 to December 2015...
September 15, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/29024023/chronic-active-epstein-barr-virus-infection-with-cutaneous-lymphoproliferation-haemophagocytosis-in-the-skin-and-haemophagocytic-syndrome
#4
S Tokoro, T Namiki, K Miura, K Watanabe, A Arai, K Imadome, H Yokozeki
Patients with chronic active Epstein-Barr virus (EBV) infections (CAEBV) present with cutaneous manifestations including hydroa vacciniforme-like eruptions.(1) Haemophagocytic lymphohistiocytosis (HLH), a fatal complication, can occur in severe cases of CAEBV.(2,3) We retrieved 25 cases of CAEBV treated in our hospital and histopathologically characterized the skin lesions of 3 of those cases, including one case with haemophagocytosis. This is a novel report of CAEBV with haemophagocytosis in the skin. This article is protected by copyright...
October 12, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28982299/multivariate-analysis-of-prognosis-for-patients-with-natural-killer-t-cell-lymphoma-associated-hemophagocytic-lymphohistiocytosis
#5
Zhili Jin, Yini Wang, Jingshi Wang, Lin Wu, Ruijun Pei, Wenyuan Lai, Zhao Wang
OBJECTIVE: A major cause of hemophagocytic lymphohistiocytosis (HLH) is malignant neoplasms of the blood system, among which NK/T cell lymphoma is one of the most common risk factors. Patients with NK/T cell lymphoma hemophagocytic lymphohistiocytosis (NK/T-LAHS) have a worse prognosis and higher mortality. We aimed to explore the factors that affect the prognosis of NK/T-LAHS. METHODS: Clinical data of 42 patients with NK/T-LAHS diagnosed by Beijing Friendship Hospital from June 2008 to June 2016 were analyzed retrospectively...
October 6, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28978596/safe-usage-of-anakinra-and-dexamethasone-to-treat-refractory-hemophagocytic-lymphohistiocytosis-secondary-to-acute-disseminated-histoplasmosis-in-a-patient-with-hiv-aids
#6
Anthony J Ocon, Birju D Bhatt, Cynthia Miller, Ruben A Peredo
Hemophagocytic lymphohistiocytosis (HLH) is a serious life-threatening disease if not recognised early. In patients with HIV/AIDS, this association has been reported following acute opportunistic infections, including histoplasmosis. However, optimal treatment is not known. We describe a male aged 46 years with AIDS who developed HLH following acute disseminated histoplasmosis. Presenting symptoms included fever, hepatosplenomegaly and pancytopenia. Bone marrow biopsy confirmed HLH. Initially, he was refractory to the treatment with amphotericin B, antiretroviral therapy and intravenous immunoglobulin (IVIG)...
October 4, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28975999/combining-multiple-biomarkers-differentiates-between-active-sjia-sjia-mas-and-ebv-hlh
#7
Lehn K Weaver
Cytokine storm syndromes are a clinically heterogeneous group of conditions resulting from a maladaptive host response to an inflammatory trigger. These syndromes lead to rapid progression of immune-mediated damage to healthy tissues resulting in life-threatening multisystem organ failure. Prompt recognition of disease and medical intervention to limit damage to healthy tissues is essential to prevent cytokine storm morbidity and mortality. However, the diagnosis of cytokine storm syndromes is challenging given the clinical heterogeneity in disease presentations...
October 4, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28970704/hemophagocytic-lymphohistiocytosis-masquerading-as-acute-liver-failure-a-single-center-experience
#8
Nitin Jagtap, Mithun Sharma, Gupta Rajesh, Padaki Nagaraja Rao, Sekaran Anuradha, Manu Tandan, Mohan Ramchandani, Duvvuru Nageshwar Reddy
BACKGROUND/AIM: Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening disorder of extreme inflammation and unregulated immune response which require prompt recognition and early introduction of definitive therapy. HLH can present with wide range of hepatic dysfunction ranging from mild elevation of transaminases to liver failure. This study is carried out to describe the clinical and laboratory presentation of HLH. METHODS: Patients who were diagnosed with HLH between January 2013 and December 2015 were retrospectively included in this study...
September 2017: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/28964826/randomized-four-arm-dose-response-clinical-trial-to-optimize-resistance-exercise-training-for-older-adults-with-age-related-muscle-atrophy
#9
Michael J Stec, Anna Thalacker-Mercer, David L Mayhew, Neil A Kelly, S Craig Tuggle, Edward K Merritt, Cynthia J Brown, Samuel T Windham, Louis J Dell'Italia, C Scott Bickel, Brandon M Roberts, Kristina M Vaughn, Irina Isakova-Donahue, Gina M Many, Marcas M Bamman
PURPOSE: The myriad consequences of age-related muscle atrophy include reduced muscular strength, power, and mobility; increased risk of falls, disability, and metabolic disease; and compromised immune function. At its root, aging muscle atrophy results from a loss of myofibers and atrophy of the remaining type II myofibers. The purpose of this trial (NCT02442479) was to titrate the dose of resistance training (RT) in older adults in an effort to maximize muscle regrowth and gains in muscle function...
September 28, 2017: Experimental Gerontology
https://www.readbyqxmd.com/read/28957822/comparing-hemophagocytic-lymphohistiocytosis-in-pediatric-and-adult-patients
#10
Christian A Wysocki
PURPOSE OF REVIEW: Hemophagocytic lymphohistiocytosis (HLH) has long been thought of primarily as a pediatric disease. However, this syndrome may occur secondary to underlying malignancies, infections, and autoimmune diseases, in adult patients. Here, we seek to highlight similarities and differences between pediatric and adult HLH, knowledge gaps, and areas of active research. RECENT FINDINGS: Malignancy is a more frequent driver of HLH in adults, present in nearly half...
September 27, 2017: Current Opinion in Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28956548/epstein-barr-virus-associated-haemophagocytic-lymphohistiocytosis-presenting-with-acute-sensorineural-hearing-loss-a-case-report-and-review-of-the-literature
#11
Ferhat Arslan, Ergenekon Karagöz, Hüseyin Saffet Beköz, Bahadir Ceylan, Ali Mert
Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening catastrophic and rarely seen complication of EBV infection especially in adults. While typical presentation of EBV infection is easily diagnosed as mononucleosis syndrome in teenagers and adults, some atypical clinical presentations may be challenged. We did not encounter any patient presenting with sudden sensorineural hearing loss associated with EBV infection in our English medical literature research (1966-2016)...
September 1, 2017: Le Infezioni in Medicina
https://www.readbyqxmd.com/read/28944149/a-case-of-leprosy-erythema-nodosum-leprosum-and-hemophagocytic-syndrome-a-continuum-of-manifestations-of-same-agent-host-interactions
#12
Prasan Kumar Panda, Ramjas Prajapati, Arvind Kumar, Manisha Jana, Pradeep Immanuel, Pranay Tanwar, Naveet Wig
A young adult man with 4-years history of lepromatous leprosy (received irregularly multidrug therapy) presented with two and half years history of symptoms suggestive of chronic erythema nodosum leprosum (ENL), initially responded to steroids and thalidomide, but later on failed. During the last 2-months, he developed fever, vomiting, and subsequently altered sensorium. On evaluation, he had hepatosplenomegaly, hyponatremia, hyperferritinemia, hemophagocytosis in bone marrow aspiration, lobular panniculitis in skin biopsy, and multiple parenchymal nodules in chest imaging...
August 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/28938698/a-systematic-review-of-malignancy-associated-hemophagocytic-lymphohistiocytosis-that-needs-more-attentions
#13
REVIEW
Hongluan Wang, Lixia Xiong, Weiping Tang, Ying Zhou, Fei Li
As an infrequent but potentially life-threatening hyperinflammatory syndrome, hemophagocytic lymphohistiocytosis (HLH) is clinically characterized with prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Malignancy-associated HLH (M-HLH), one type of acquired HLH, usually presents variable overlaps of symptoms with other types of HLH, thus resulting in higher incidence of misdiagnosis and mortality. In recent years, with the increasing awareness to this disease, the diagnosis and management of HLH have gained more and more attention, and improvements have been made accordingly...
August 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/28938257/childhood-visceral-leishmaniasis-distinctive-features-and-diagnosis-of-a-re-emerging-disease-an-11-year-experience-from-a-tertiary-referral-center-in-athens-greece
#14
Panagiotis Krepis, Adamantia Krepi, Ioanna Argyri, Anastasios Aggelis, Alexandra Soldatou, Maria Tsolia
BACKGROUND: Visceral leishmaniasis (VL) remains a public health issue in Greece. The aim of this study was to describe the clinical and epidemiological characteristics of pediatric VL in our region as well as to evaluate the laboratory findings and the diagnostic techniques that are applied. METHODS: We retrospectively reviewed the medical records of all children diagnosed with VL in an 11-year period at a tertiary public hospital in the region of Athens. Demographic features, clinical information and laboratory findings were accessed...
September 20, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28936583/effective-immunological-guidance-of-genetic-analyses-including-exome-sequencing-in-patients-evaluated-for-hemophagocytic-lymphohistiocytosis
#15
Sandra Ammann, Kai Lehmberg, Udo Zur Stadt, Christian Klemann, Sebastian F N Bode, Carsten Speckmann, Gritta Janka, Katharina Wustrau, Mirzokhid Rakhmanov, Ilka Fuchs, Hans C Hennies, Stephan Ehl
We report our experience in using flow cytometry-based immunological screening prospectively as a decision tool for the use of genetic studies in the diagnostic approach to patients with hemophagocytic lymphohistiocytosis (HLH). We restricted genetic analysis largely to patients with abnormal immunological screening, but included whole exome sequencing (WES) for those with normal findings upon Sanger sequencing. Among 290 children with suspected HLH analyzed between 2010 and 2014 (including 17 affected, but asymptomatic siblings), 87/162 patients with "full" HLH and 79/111 patients with "incomplete/atypical" HLH had normal immunological screening results...
September 21, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28936212/how-viruses-contribute-to-the-pathogenesis-of-hemophagocytic-lymphohistiocytosis
#16
Ellen Brisse, Carine H Wouters, Graciela Andrei, Patrick Matthys
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening, hyperinflammatory syndrome, characterized by the uncontrolled activation of macrophages and T cells, eliciting key symptoms such as persistent fever, hepatosplenomegaly, pancytopenia, hemophagocytosis, hyperferritinemia, and coagulopathy. Viral infections are frequently implicated in the onset of active HLH episodes, both in primary, genetic HLH as in the secondary, acquired form. Infections with herpesviruses such as Epstein-Barr virus and cytomegalovirus are the most common...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28935695/confirmed-efficacy-of-etoposide-and-dexamethasone-in-hlh-treatment-long-term-results-of-the-cooperative-hlh-2004-study
#17
Elisabet Bergsten, AnnaCarin Horne, Maurizio Aricó, Itziar Astigarraga, R Maarten Egeler, Alexandra H Filipovich, Eiichi Ishii, Gritta Janka, Stephan Ladisch, Kai Lehmberg, Kenneth L McClain, Milen Minkov, Scott Montgomery, Vasanta Nanduri, Diego Rosso, Jan-Inge Henter
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome comprising familial/genetic HLH (FHL) and secondary HLH. In the HLH-94 study, with an estimated 5-year probability of survival (pSu) of 54% (95% CI, 48-60%), systemic therapy included etoposide, dexamethasone and, from week nine, cyclosporine A (CSA). HSCT was indicated in patients with familial/genetic, relapsing, or severe and persistent disease. In HLH-2004, CSA was instead administered upfront, aiming to reduce pre-HSCT mortality and morbidity...
September 21, 2017: Blood
https://www.readbyqxmd.com/read/28932715/hemophagocytic-lymphohistiocytosis-hlh-elusive-diagnosis-of-disseminated-mycobacterium-avium-complex-infection
#18
Eloy E Ordaya, Sulieman Abu Jarir, Robert Yoo, Pranatharthi H Chandrasekar
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome of hyperinflammation leading to an uncontrolled and ineffective immune response, associated with high mortality. CASE REPORT: A 26-year-old woman with acute lymphoblastic leukemia, 8 months in remission, was found to have HLH. Without any improvement, stem cell transplantation was considered. Then, Mycobacterium avium complex (MAC) infection was identified as cause of her HLH. With appropriate therapy for infection, HLH improved and transplantation was averted...
September 2017: Germs
https://www.readbyqxmd.com/read/28931369/acute-hiv-infection-presenting-as-hemophagocytic-lymphohistiocytosis-case-report-and-review-of-the-literature
#19
Farheen Manji, Evan Wilson, Etienne Mahe, John Gill, John Conly
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is an uncommon systemic inflammatory condition that can result from infections, autoimmune diseases and malignancies. It is a rarely reported life threatening complication of an acute HIV infection, with only ten documented case reports per our literature search. We present a case of HLH secondary to acute HIV infection with a negative HIV antibody-based assay and high plasma viral load. CASE PRESENTATION: A 45 year old male with a past medical history of well controlled hypertension presented with fever, dizziness and non-bloody diarrhea...
September 20, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28925994/chimeric-antigen-receptor-t-cell-therapy-assessment-and-management-of-toxicities
#20
REVIEW
Sattva S Neelapu, Sudhakar Tummala, Partow Kebriaei, William Wierda, Cristina Gutierrez, Frederick L Locke, Krishna V Komanduri, Yi Lin, Nitin Jain, Naval Daver, Jason Westin, Alison M Gulbis, Monica E Loghin, John F de Groot, Sherry Adkins, Suzanne E Davis, Katayoun Rezvani, Patrick Hwu, Elizabeth J Shpall
Immunotherapy using T cells genetically engineered to express a chimeric antigen receptor (CAR) is rapidly emerging as a promising new treatment for haematological and non-haematological malignancies. CAR-T-cell therapy can induce rapid and durable clinical responses, but is associated with unique acute toxicities, which can be severe or even fatal. Cytokine-release syndrome (CRS), the most commonly observed toxicity, can range in severity from low-grade constitutional symptoms to a high-grade syndrome associated with life-threatening multiorgan dysfunction; rarely, severe CRS can evolve into fulminant haemophagocytic lymphohistiocytosis (HLH)...
September 19, 2017: Nature Reviews. Clinical Oncology
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