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https://www.readbyqxmd.com/read/28550411/isolation-characterization-and-expression-of-proto-oncogene-cmyc-in-large-yellow-croaker-larimichthys-crocea
#1
Yonghua Jiang, Kunhuang Han, Shihai Chen, Yilei Wang, Ziping Zhang
cMyc is a vital transcription factor that involves in the regulation of cell proliferation, growth, differentiation, and apoptosis. In the present study, cMyc in Larimichthys crocea (Lc-cMyc) was cloned and analyzed for investigating its function. The full-length cDNA of Lc-cMyc was 2089 bp encoding a 440-amino-acid protein (Lc-cMyc). Lc-cMyc had the characteristic helix-loop-helix-leucine-zipper (HLH-LZ) DNA-binding domain and highly conservative in evolution. The expression of Lc-cMyc was detected by quantitative real-time PCR (qRT-PCR) and in situ hybridization, respectively...
May 27, 2017: Fish Physiology and Biochemistry
https://www.readbyqxmd.com/read/28548191/-hemophagocytic-lymphohistiocytosis-experience-in-27-patients
#2
Fernando Warley, Belén M Bonella, M Silvina Odstrcil-Bobillo, Victoria Otero, Gabriel Waisman, Gisela Bendelman, Diego Giunta, Verónica Peuchot, Catalina M Ungaro
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation Aim: To describe the clinical characteristics, causes and survival associated with HLH. MATERIAL AND METHODS: Review of medical records of patients with HLH attended between 2004 and 2016. They were classified according to their probable cause in: associated with immunosuppression, cancer, post-infectious or idiopathic. Kaplan-Meier survival analysis was performed...
March 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/28527376/the-effectiveness-of-cutoff-walls-to-control-saltwater-intrusion-in-multi-layered-coastal-aquifers-experimental-and-numerical-study
#3
Antoifi Abdoulhalik, Ashraf A Ahmed
The objective of this study was to examine the performance of cutoff walls in controlling saltwater intrusion in stratified heterogeneous coastal aquifers. Numerical and laboratory experiments were completed in laboratory-scale aquifer where the effectiveness of cutoff walls was assessed in three different configurations, including a homogeneous scenario, a stratified aquifer with high K-low K-high K pattern (case HLH) and another stratified aquifer with low K-high K-low K pattern (case LHL). The results show that the cutoff wall was effective in reducing the saltwater wedge in all the investigated cases of layered-aquifers with toe length reduction of up to 43%...
May 17, 2017: Journal of Environmental Management
https://www.readbyqxmd.com/read/28523896/hemophagocytic-lymphohistiocytosis-in-a-newborn-presenting-as-blueberry-muffin-baby
#4
Krista N Larson, Sergio R Gaitan, Benjamin J Stahr, Dean S Morrell
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome characterized by uncontrolled activation and proliferation of proinflammatory cytokines. Initial presentation commonly includes fever, hepatosplenomegaly, and pancytopenia; 6 to 65% of cases also have a concurrent cutaneous eruption. We present the case of a 6-day-old premature infant boy with congenital severe thrombocytopenia, anemia, and hepatosplenomegaly who presented with several cutaneous violaceous papules and nodules and was found to have HLH...
May 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28521652/case-report-patient-with-unexplained-high-fever-and-pancytopenia
#5
Kelly De Schuyter, Tom Lodewyck
OBJECTIVE AND IMPORTANCE: We present a case of hemophagocytic lymphohistiocytosis (HLH), giving insight in how to establish diagnosis and start appropiate treatment. CLINICAL PRESENTATION: A 45-year-old male presented at the emergency ward with high fever and pancytopenia. Repeat bone marrow aspirates showed hemophagocytosis. Extensive work-up with exclusion of other infectious and malignant diseases, eventually lead us to the diagnosis of hemophagocytic lymphohistiocytosis...
May 19, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28516082/multifocal-recurrent-osteomyelitis-and-hemophagocytic-lymphohistiocytosis-in-a-boy-with-partial-dominant-ifn-%C3%AE-r1-deficiency-case-report-and-review-of-the-literature
#6
Aidé Tamara Staines-Boone, Caroline Deswarte, Edna Venegas Montoya, Luz María Sánchez-Sánchez, Jorge Alberto García Campos, Teodoro Muñiz-Ronquillo, Jacinta Bustamante, Francisco J Espinosa-Rosales, Saul Oswaldo Lugo Reyes
Mutations in the genes coding for cytokines, receptors, second messengers, and transcription factors of interferon gamma (IFN-γ) immunity cause Mendelian susceptibility to mycobacterial disease (MSMD). We report the case of a 7-year-old male patient with partial dominant (PD) IFN-γ receptor 1 deficiency who had suffered from multifocal osteomyelitis attributable to bacille Calmette-Guérin vaccination since the age of 18 months. He developed hemophagocytic lymphohistiocytosis (HLH), a hyper-inflammatory complication, and died with multiorgan dysfunction, despite having been diagnosed and treated relatively early...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28500507/impact-of-severe-hematological-abnormalities-in-the-outcome-of-hospitalized-patients-with-influenza-virus-infection
#7
A Lalueza, H Trujillo, J Laureiro, B Ayuso, P Hernández-Jiménez, C Castillo, M Torres, D Folgueira, O Madrid, C Díaz-Pedroche, E Arrieta, C Arévalo, C Lumbreras
Although hematological abnormalities have been described among patients with influenza virus infection, little is known about their impact on the outcome of the patients. The aim of this study was to assess the frequency and clinical impact of severe hematological abnormalities in patients with confirmed influenza virus infection. This was an observational retrospective study including all adult patients with diagnosis of influenza virus infection hospitalized from January to May 2016 in our institution. Influenza virus infection was diagnosed by means of rRT-PCR assay performed on respiratory samples...
May 13, 2017: European Journal of Clinical Microbiology & Infectious Diseases
https://www.readbyqxmd.com/read/28497365/clinical-utility-of-soluble-interleukin-2-receptor-in-hemophagocytic-syndromes-a-systematic-scoping-review
#8
REVIEW
Molly Lin, Sujin Park, Anna Hayden, Dean Giustini, Martina Trinkaus, Morris Pudek, Andre Mattman, Marion Schneider, Luke Y C Chen
The serum-soluble interleukin-2 receptor (sIL-2r) level is considered an important diagnostic test and disease marker in hemophagocytic syndromes/hemophagocytic lymphohistiocytosis (HPS/HLH). However, this cytokine receptor is rarely measured in clinical practice and has been excluded from recent diagnostic/classification criteria such as the HScore and macrophage activation syndrome (MAS) 16. We performed a systematic scoping review of 64 articles (1975-2016) examining the clinical utility of sIL-2r in HPS/HLH...
May 12, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28494931/hemophagocytic-lymphohistiocytosis-in-acute-african-swine-fever-clinic
#9
Z R Karalyan, Z R Ter-Pogossyan, N Yu Karalyan, Z B Semerjyan, M R Tatoyan, S A Karapetyan, E M Karalova
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) usually has been defined as the combination of a proliferation of cytologically benign, actively phagocytic macrophages in bone marrow, spleen, lymph nodes, etc. in association with fever, cytopenia, splenomegaly, and hypertriglyceridemia. HLH is often triggered by viral infection. The aim of this study was to ascertain the features of HLH involvement in African swine fever virus (ASFV) (genotype II) pathogenesis. METHODS: The serum levels of macrophage colony-stimulating factor (MCSF) and granulocyte-macrophage colony-stimulating factor (GMCSF), as well as the histological constitution (for hemophagocytic macrophages detection) of various organs of pigs infected with ASFV genotype II were investigated...
May 2017: Veterinary Immunology and Immunopathology
https://www.readbyqxmd.com/read/28471834/hemophagocytic-lymphohistiocytosis-in-patients-with-metastatic-malignant-melanoma
#10
Sarah Stabler, Coralie Becquart, Florent Dumezy, Louis Terriou, Laurent Mortier
Hemophagocytic lymphohistiocytosis (HLH) is an autoinflammatory disease that classically occurs because of infections, autoinflammatory, or autoimmune diseases, hematologic cancers, and rarely because of solid tumor. We report a rare case of HLH attributed to metastatic malignant melanoma treated without corticosteroid and with a nonfatal outcome thanks to specific therapies: etoposide for HLH and a selective inhibitor of mutated forms of BRAF kinase associated with a MEK inhibitor for melanoma.
May 3, 2017: Melanoma Research
https://www.readbyqxmd.com/read/28470180/associations-of-the-intellectual-disability-gene-myt1l-with-helix-loop-helix-gene-expression-hippocampus-volume-and-hippocampus-activation-during-memory-retrieval
#11
Agnes Kepa, Lourdes Martinez Medina, Susanne Erk, Deepak P Srivastava, Alinda Fernandes, Roberto Toro, Sabine Lévi, Barbara Ruggeri, Cathy Fernandes, Franziska Degenhardt, Stephanie H Witt, Andreas Meyer-Lindenberg, Jean-Christophe Poncer, Jean-Luc Martinot, Marie-Laure Paillère Martinot, Christian P Müller, Andreas Heinz, Henrik Walter, Gunter Schumann, Sylvane Desrivières
The fundamental role of the brain-specific myelin transcription factor 1-like (MYT1L) gene in cases of intellectual disability and in the aetiology of neurodevelopmental disorders is increasingly recognised. Yet, its function remains under-investigated. Here, we identify a network of helix-loop-helix (HLH) transcriptional regulators controlled by MYT1L, as indicated by our analyses in human neural stem cells and in the human brain. Using cell-based knockdown approaches and microarray analyses we found that (i) MYT1L is required for neuronal differentiation and identified ID1, a HLH inhibitor of premature neurogenesis, as a target...
May 4, 2017: Neuropsychopharmacology: Official Publication of the American College of Neuropsychopharmacology
https://www.readbyqxmd.com/read/28468610/a-case-report-of-novel-mutation-in-prf1-gene-which-causes-familial-autosomal-recessive-hemophagocytic-lymphohistiocytosis
#12
Mohammad Reza Bordbar, Farzaneh Modarresi, Mohammad Ali Farazi Fard, Hassan Dastsooz, Nader Shakib Azad, Mohammad Ali Faghihi
BACKGROUND: Hemophagocytic Lymphohistiocytosis (HLH) is a life-threatening immunodeficiency and multi-organ disease that affects people of all ages and ethnic groups. Common symptoms and signs of this disease are high fever, hepatosplenomegaly, and cytopenias. Familial form of HLH disease, which is an autosomal recessive hematological disorder is due to disease-causing mutations in several genes essential for NK and T-cell granule-mediated cytotoxic function. For an effective cytotoxic response from cytotoxic T lymphocyte or NK cell encountering an infected cell or tumor cell, different processes are required, including trafficking, docking, priming, membrane fusion, and entry of cytotoxic granules into the target cell leading to apoptosis...
May 3, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28459018/visceral-leishmaniasis-associated-hemophagocytosis-a-tale-of-two-unexpected-diagnoses-from-a-nonendemic-region
#13
Rashmi Kaul Raina, Sujeet Raina, Manupriya Sharma
A case of visceral leishmaniasis (VL)-associated hemophagocytic lymphohistiocytosis (HLH) in an immunocompetent native from a nonendemic area was reported. The patient belonged to Ravi river valley area (altitude 996 meters above the mean sea level) of Chamba, Himachal Pradesh, India. VL and HLH were not a differential diagnosis. Identification of the Leishman-Donovan bodies and hemophagocytosis in bone marrow aspirate and biopsy provided the diagnosis. The patient recovered to the treatment with amphotericin B...
January 2017: Tropical Parasitology
https://www.readbyqxmd.com/read/28458669/differences-in-granule-morphology-yet-equally-impaired-exocytosis-among-cytotoxic-t-cells-and-nk-cells-from-chediak-higashi-syndrome-patients
#14
Samuel C C Chiang, Stephanie M Wood, Bianca Tesi, Himmet Haluk Akar, Waleed Al-Herz, Sandra Ammann, Fatma Burcu Belen, Umran Caliskan, Zühre Kaya, Kai Lehmberg, Turkan Patiroglu, Huseyin Tokgoz, Ayşegül Ünüvar, Wendy J Introne, Jan-Inge Henter, Magnus Nordenskjöld, Hans-Gustaf Ljunggren, Marie Meeths, Stephan Ehl, Konrad Krzewski, Yenan T Bryceson
Chediak-Higashi syndrome (CHS) is caused by autosomal recessive mutations in LYST, resulting in enlarged lysosomal compartments in multiple cell types. CHS patients display oculocutaneous albinism and may develop life-threatening hemophagocytic lymphohistiocytosis (HLH). While NK cell-mediated cytotoxicity has been reported to be uniformly defective, variable defects in T cell-mediated cytotoxicity has been observed. The latter has been linked to the degree of HLH susceptibility. Since the discrepancies in NK cell- and T cell-mediated cellular cytotoxicity might result from differences in regulation of cytotoxic granule release, we here evaluated perforin-containing secretory lysosome size and number in freshly isolated lymphocytes from CHS patients and furthermore compared their exocytic capacities...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28452857/a-case-of-hemophagocytic-lymphohistiocytosis-and-kawasaki-disease-concurrent-or-overlapping-diagnoses
#15
Allison S Bechtel, Michael Joyce
Hemophagocytic lymphohistiocytosis (HLH) is not always suspected at the time of presentation. It is often mistaken for other diagnoses; complicated by the fact that it is often associated with an inciting event that has significant overlap. Kawasaki disease, along with other disorders, such as Ebstein Barr Virus infection, are conditions that may lead HLH. Our patient had a presentation that was consistent with Kawasaki disease on initial presentation, however subsequently met the diagnostic criteria of HLH...
April 27, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28452182/local-injection-of-lenti-olig2-at-lesion-site-promotes-functional-recovery-of-spinal-cord-injury-in-rats
#16
Bo-Tao Tan, Long Jiang, Li Liu, Ying Yin, Ze-Ru-Xin Luo, Zai-Yun Long, Sen Li, Le-Hua Yu, Ya-Min Wu, Yuan Liu
AIMS: Olig2 is one of the most critical factors during CNS development, which belongs to b-HLH transcription factor family. Previous reports have shown that Olig2 regulates the remyelination processes in CNS demyelination diseases models. However, the role of Olig2 in contusion spinal cord injury (SCI) and the possible therapeutic effects remain obscure. This study aims to investigate the effects of overexpression Olig2 by lentivirus on adult spinal cord injury rats. METHODS: Lenti-Olig2 expression and control Lenti-eGFP vectors were prepared, and virus in a total of 5 μL (10(8) TU/mL) was locally injected into the injured spinal cord 1...
June 2017: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/28446488/eculizumab-in-a-child-with-atypical-haemolytic-uraemic-syndrome-and-haemophagocytic-lymphohistiocytosis-triggered-by-cytomegalovirus-infection
#17
Gloria M Fraga-Rodriguez, Sonia Brió-Sanagustin, Eulalia Turón-Viñas, Bradley P Dixon, Eduardo Carreras-González
We present the case of a 21-month-old girl with two rare and life-threatening conditions, atypical haemolytic uraemic syndrome (aHUS) and haemophagocytic lymphohistiocytosis (HLH), triggered by a cytomegalovirus (CMV) infection. Soon after admission, the girl became anuric and required continuous venovenous haemodiafiltration.Initial treatments included methylprednisolone, fibrinogen and plasma infusion (for HLH), plasmapheresis (for thrombotic microangiopathy), immunoglobulins (for inflammation), ganciclovir (for CMV infection) and the antibiotic cefotaxime...
April 26, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28446487/haemophagocytic-lymphohistiocytosis-as-a-consequence-of-untreated-b-cell-chronic-lymphocytic-leukaemia
#18
Chris Bailey, Claire Dearden, Kirit Ardeshna
Haemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome characterised by defective cytotoxic function and hypercytokinaemia leading to macrophage expansion and haemophagocytosis. Patients often present with unexplained fevers, hepatosplenomegaly and pancytopenia, with elevation in serum ferritin and triglyceride. Acquired forms are triggered by infection, malignancy or rheumatological disorders. HLH in the setting of chronic lymphocytic leukaemia is rarely reported, however, and is usually associated with infection or as a consequence of chemotherapy...
April 26, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28433984/haemophagocytic-lymphohistiocytosis-presenting-as-hellp-syndrome-a-diagnostic-and-therapeutic-challenge
#19
Robert Noel Kerley, Raymond Michael Kelly, Mary Rose Cahill, Louise Clare Kenny
Haemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, haematological disorder, which can be clinically challenging to diagnose and manage. We report a case of HLH in a previously healthy 33-year-old primigravida. The patient presented at 22 weeks gestation with dyspnoea, abdominal pain, anaemia, thrombocytopenia and elevated liver enzymes suggestive of HELLP syndrome.HELLP, a syndrome characterised by haemolysis, elevated liver enzymes and low platelets is considered a severe form of pre-eclampsia...
April 22, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28433836/recent-advances-in-diagnostic-and-therapeutic-guidelines-for-primary-and-secondary-hemophagocytic-lymphohistiocytosis
#20
REVIEW
Smita Ramachandran, Fauzia Zaidi, Archana Aggarwal, Rani Gera
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by excessive activation of macrophages and T cells resulting from defective cytotoxicity. It is potentially life threatening due to the large amounts of cytokines released by the activated macrophages and lymphocytes secondary to a hyperinflammatory response. It has a high fatality in children with an incidence of approximately 1.2cases/million per year. METHOD: The literature was extensively searched in PubMed, MEDLINE and Google scholar...
November 3, 2016: Blood Cells, Molecules & Diseases
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