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https://www.readbyqxmd.com/read/28807357/development-and-initial-validation-of-the-macrophage-activation-syndrome-primary-hemophagocytic-lymphohistiocytosis-score-a-diagnostic-tool-that-differentiates-primary-hemophagocytic-lymphohistiocytosis-from-macrophage-activation-syndrome
#1
Francesca Minoia, Francesca Bovis, Sergio Davì, Antonella Insalaco, Kai Lehmberg, Susan Shenoi, Sheila Weitzman, Graciela Espada, Yi-Jin Gao, Jordi Anton, Toshiyuki Kitoh, Ozgur Kasapcopur, Helga Sanner, Rosa Merino, Itziar Astigarraga, Maria Alessio, Michael Jeng, Vyacheslav Chasnyk, Kim E Nichols, Zeng Huasong, Caifeng Li, Concetta Micalizzi, Nicolino Ruperto, Alberto Martini, Randy Q Cron, Angelo Ravelli, AnnaCarin Horne
OBJECTIVE: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis. STUDY DESIGN: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample...
August 11, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28806468/an-overview-of-hemophagocytic-lymphohistiocytosis
#2
Ysabella M Esteban, Jill L O de Jong, Melissa S Tesher
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by a dysregulated hyperinflammatory response associated with aberrant activation of lymphocytes and macrophages that results in hypercytokinemia. It is classically divided into two types: (1) primary or familial HLH and (2) secondary HLH. Familial HLH is generally an autosomal recessive condition, whereas secondary HLH is usually associated with infectious diseases, autoinflammatory and autoimmune diseases (where it is more commonly known as macrophage activation syndrome), malignancy, immunosuppression, hematopoietic stem cell transplantation, organ transplantation, HIV infection, and metabolic diseases...
August 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28804953/basic-helix-loop-helix-transcription-factors-in-evolution-roles-in-development-of-mesoderm-and-neural-tissues
#3
REVIEW
Fuki Gyoja
Basic helix-loop-helix (bHLH) transcription factors have attracted the attention of developmental and evolutionary biologists for decades because of their conserved functions in mesodermal and neural tissue formation in both vertebrates and fruit flies. Their evolutionary history is of special interest because it will likely provide insights into developmental processes and refinement of metazoan-specific traits. This review briefly considers advances in developmental biological studies on bHLHs/HLHs. I also discuss recent genome-wide surveys and molecular phylogenetic analyses of these factors in a wide range of metazoans...
August 14, 2017: Genesis: the Journal of Genetics and Development
https://www.readbyqxmd.com/read/28793334/spheroid-growth-in-ovarian-cancer-alters-transcriptome-responses-for-stress-pathways-and-epigenetic-responses
#4
Trillitye Paullin, Chase Powell, Christopher Menzie, Robert Hill, Feng Cheng, Christopher J Martyniuk, Sandy D Westerheide
Ovarian cancer is the most lethal gynecological cancer, with over 200,000 women diagnosed each year and over half of those cases leading to death. These poor statistics are related to a lack of early symptoms and inadequate screening techniques. This results in the cancer going undetected until later stages when the tumor has metastasized through a process that requires the epithelial to mesenchymal transition (EMT). In lieu of traditional monolayer cell culture, EMT and cancer progression in general is best characterized through the use of 3D spheroid models...
2017: PloS One
https://www.readbyqxmd.com/read/28791218/cytomegalovirus-induced-hemophagocytic-lymphocytic-histiocytosis-in-two-pediatric-patients-with-acute-lymphoblastic-leukemia
#5
Benjamin Waddell, Chris Belcher, Emily Willey
Hemophagocytic lymphohistiocytosis (HLH) is a rare inflammatory condition with tissue destruction due to abnormal immune activation. We present a series of 2 cases of cytomegalovirus-induced HLH in children during maintenance chemotherapy for acute lymphoblastic leukemia. These cases emphasize the importance of considering secondary HLH in this high-risk subset of pediatric patients.
2017: IDCases
https://www.readbyqxmd.com/read/28762620/a-case-of-chediak-higashi-syndrome-presented-with-accelerated-phase-could-be-treated-effectively-by-unrelated-cord-blood-transplantation
#6
Yan Zhang, Zhiyong Gao, Xinjian Yu
CHS is a rare immunodeficiency syndrome with defects in the functions of cytotoxic cells and neutrophils. Approximately 85% of patients with CHS undergo an AP within the first decade, which is similar to FHLH. Chemotherapy could induce transient remission, but only allogeneic HCT could correct the underlying genetic defect and prevent relapse. We reported a case of CHS diagnosed at 19 months, who had an elder brother who had previously succumbed to the same disease. The little girl presented with severe AP manifestations including recurrent high fever, enlarged superficial lymph nodes, and extraordinary hepatosplenomegaly occupying the whole abdominal and pelvic cavity...
August 1, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28762079/elevated-serum-ferritin-is-not-specific-for-hemophagocytic-lymphohistiocytosis
#7
Zaher K Otrock, Karl G Hock, Sarah B Riley, Theo de Witte, Charles S Eby, Mitchell G Scott
Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, syndrome of excessive and ineffective activation of the immune system. The majority of the reported data on HLH is from pediatric patients and lacks specificity. This makes HLH diagnosis challenging especially in adults where HLH is triggered by many conditions and can resemble many disease entities. Elevated ferritin is one of the diagnostic criteria for HLH. We determined the conditions associated with elevated ferritin at our medical center to assess how specific ferritin is for predicting HLH...
July 31, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28760295/diagnostic-challenges-of-hemophagocytic-lymphohistiocytosis
#8
REVIEW
Zaher K Otrock, Naval Daver, Hagop M Kantarjian, Charles S Eby
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by excessive activation of the immune system, resulting in overproduction of inflammatory cytokines. Patients usually present with high fever, cytopenias, hyperferritinemia, and hepatosplenomegaly, and their disease process ranges from mild to fatal multiorgan failure. HLH is a heterogeneous group of disorders that can be triggered by infections, neoplasms, or autoimmune diseases. The HLH diagnosis can be difficult to confidently confirm in critically ill patients while waiting for pathology or reference laboratory results to return, delaying the diagnosis with significantly worsened outcomes...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28741867/targeting-of-a-helix-loop-helix-transcriptional-regulator-by-a-short-helical-peptide
#9
Cornelia Roschger, Saskia Neukirchen, Brigitta Elsässer, Mario Schubert, Nicole Maeding, Thomas Verwanger, Barbara Krammer, Chiara Cabrele
The Id proteins (Id1-4) are cell cycle regulators that play a key role during development, in cancer and vascular disorders. They contain a conserved helix-loop-helix (HLH) domain that folds into a parallel four-helix bundle upon self- or heteroassociation with basic-HLH transcription factors. By using such protein-protein interactions, the Id proteins inhibit cell differentiation and promote cell cycle progression. Accordingly, their supporting role in cancer has been convincingly demonstrated, which makes these proteins interesting therapeutic targets...
July 25, 2017: ChemMedChem
https://www.readbyqxmd.com/read/28738028/ebv-hlh-children-with-reductions-in-cd4-t-cells-and-excessive-activation-of-cd8-t-cells
#10
Cheng Yang, Xiujuan Zhu, Ting Zhang, Qing Ye
BACKGROUND: In this report, we analyzed the number and activation profiles of T cells in EBV-HLH patients and assessed their value for the diagnosis of EBV-HLH. METHODS: We compared the quantity of CD4+ T cells and CD8+ T cells in peripheral blood among HLH patients, a sepsis group and healthy controls. We then analyzed variations in the activation marker in CD4+ T cells and CD8+ T cells. RESULTS: The percentage of CD4+ T cells decreased in the EBV-HLH patients compared to healthy controls, which induced significant differences in the ratio of CD4+ T cells/CD8+ T cells...
July 24, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28734827/multi-omics-analyses-of-starvation-responses-reveal-a-central-role-for-lipoprotein-metabolism-in-acute-starvation-survival-in-c-%C3%A2-elegans
#11
Eva Bang Harvald, Richard R Sprenger, Kathrine Brændgaard Dall, Christer S Ejsing, Ronni Nielsen, Susanne Mandrup, Alejandro Brenes Murillo, Mark Larance, Anton Gartner, Angus I Lamond, Nils J Færgeman
Starvation causes comprehensive metabolic changes, which are still not fully understood. Here, we used quantitative proteomics and RNA sequencing to examine the temporal starvation responses in wild-type Caenorhabditis elegans and animals lacking the transcription factor HLH-30. Our findings show that starvation alters the abundance of hundreds of proteins and mRNAs in a temporal manner, many of which are involved in central metabolic pathways, including lipoprotein metabolism. We demonstrate that premature death of hlh-30 animals under starvation can be prevented by knockdown of either vit-1 or vit-5, encoding two different lipoproteins...
July 26, 2017: Cell Systems
https://www.readbyqxmd.com/read/28732360/a-systematic-review-of-malignancy-associated-hemophagocytic-lymphohistiocytosis-that-needs-more-attentions
#12
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...
July 14, 2017: Oncotarget
https://www.readbyqxmd.com/read/28723775/the-neutrophil-to-lymphocyte-ratio-could-be-a-good-diagnostic-marker-and-predictor-of-relapse-in-patients-with-adult-onset-still-s-disease-a-strobe-compliant-retrospective-observational-analysis
#13
Ji-Yeoun Seo, Chang-Hee Suh, Ju-Yang Jung, Ar-Reum Kim, Ji Won Yang, Hyoun-Ah Kim
The neutrophil-to-lymphocyte ratio (NLR) is the proportion of absolute neutrophil count to lymphocytes on routine complete blood count (CBC) tests, and has been studied as a simple marker of the systemic inflammatory response. This study was performed to investigate whether the NLR could be used as a tool to diagnose and predict prognosis in cases of adult-onset Still's disease (AOSD).We retrospectively reviewed 164 patients with suspected AOSD. Among 164 patients with suspected AOSD, 37 patients received another diagnosis (such as viral infection) and were compared with the 127 patients who received a diagnosis of AOSD...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28722256/diagnostic-and-prognostic-value-of-low-percentage-of-glycosylated-ferritin-in-acquired-hemophagocytic-lymphohistiocytosis-a-single-center-study
#14
M Nabergoj, M Marinova, G Binotto, L Brugnaro, M Zaninotto, M Plebani, G Semenzato, F Vianello
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome characterized by an excessive immune activation. Glycosylated ferritin (GF) level has been proposed as highly specific of HLH. METHODS: We have studied 12 subjects with HLH according to the HLH-04 trial criteria and 11 patients with a clinical and laboratoristic suspicion of HLH. The percentage of GF was measured by an in-house assay. RESULTS: The only biomarkers that were significantly different in the two groups were fraction of GF (P<...
July 18, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28711715/novel-peptide-motifs-from-lysozyme-suppress-pro-inflammatory-cytokines-in-macrophages-by-antagonizing-toll-like-receptor-and-lps-scavenging-action
#15
Hisham R Ibrahim, Kosuke Hamasaki, Takeshi Miyata
Lysozyme is commonly found in spots where bacterial infections are most likely to enter the body. Earlier we found that lysozyme possesses five antimicrobial peptide motifs in its N-terminal region which can be generated by newborn pepsin. In this study, we explore the role of these peptides in the anti-inflammatory activity of lysozyme. The five peptides, helix1 (H1), helix2 (H2), H1 and H2 connected with a loop (HLH), H2 extended with either 2 β-strands (H2-S12) or 3 β-strands (H2-S13), were synthesized and examined for anti-inflammatory action...
July 12, 2017: European Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28704468/thmyc4e-candidate-blue-aleurone-1-gene-controlling-the-associated-trait-in-triticum-aestivum
#16
Na Li, Shiming Li, Kunpu Zhang, Wenjie Chen, Bo Zhang, Daowen Wang, Dengcai Liu, Baolong Liu, Huaigang Zhang
Blue aleurone is a useful and interesting trait in common wheat that was derived from related species. Here, transcriptomes of blue and white aleurone were compared for isolating Blue aleurone 1 (Ba1) transferred from Thinopyrum ponticum. In the genes involved in anthocyanin biosynthesis, only a basic helix-loop-helix (bHLH) transcription factor, ThMYC4E, had a higher transcript level in blue aleurone phenotype, and was homologous to the genes on chromosome 4 of Triticum aestivum. ThMYC4E carried the characteristic domains (bHLH-MYC_N, HLH and ACT-like) of a bHLH transcription factor, and clustered with genes regulating anthocyanin biosynthesis upon phylogenetic analysis...
2017: PloS One
https://www.readbyqxmd.com/read/28700505/sepsis-due-to-streptococcus-pneumoniae-associated-with-secondary-hemophagocytic-lymphohistiocytosis-in-a-splenectomized-patient-for-spherocytosis-a-case-report
#17
Victoria Birlutiu, Rares Mircea Birlutiu
RATIONALE: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome that is characterized by an inappropriate hyperinflammatory immune response - primary, as a consequence of a genetic defect of NK cells and cytotoxic T lymphocytes or - secondary, in the progression of infections, rheumatic or autoimmune diseases, malignancies or metabolic diseases. PATIENT CONCERNS: We present the case of a secondary HLH due to Streptococcus pneumoniae infection in a splenectomised patient for spherocytosis, a 37-year-old patient who was splenectomised in childhood for spherocytosis, without immuneprophylaxis induced by antipneumococcal vaccine...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28700492/an-unusual-association-between-hemophagocytic-lymphohistiocytosis-mixed-connective-tissue-disease-and-autoimmune-hemolytic-anemia-a-case-report
#18
Amar H Kelkar, Anushi A Shah, Sherri L Yong, Zohair Ahmed
RATIONALE: In the adult patient, hemophagocytic lymphohistiocytosis (HLH) is uncommon and frequently difficult to diagnose due to its nonspecific presentation and numerous complications. PATIENT CONCERNS: Herein, we present the case of a 25-year-old female who initially presented for evaluation of persistent fevers and fatigue. She was found to have splenomegaly, generalized lymphadenopathy, pancytopenia, and acute hepatic failure. DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Her course was further complicated by the development of nephrotic syndrome and autoimmune hemolytic anemia (AIHA)...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28697170/critically-ill-children-with-hemophagocytic-lymphohistiocytosis-a-case-series-of-14-patients
#19
Esther Huimin Leow, Shui Yen Soh, Ah Moy Tan, Yee Hui Mok, Mei Yoke Chan, Jan Hau Lee
Children with hemophagocytic lymphohistiocytosis (HLH) are at an increased risk of critical illness. In this study, we described the clinical characteristics of critically ill children with HLH and identify factors associated with poor clinical outcomes. Children who were diagnosed with HLH with emergent admission to Children's Intensive Care Unit (CICU) between January 1, 2000 and October 31, 2015 were included. The primary outcome was CICU mortality. Over the 15-year period, there were 14 critically ill patients with HLH with 23 CICU admissions...
August 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28693354/perinatal-outcomes-of-congenital-heart-disease-after-emergent-neonatal-cardiac-procedures
#20
Aline Wolter, Helene Holtmann, Andreea Kawecki, Jan Degenhardt, Christian Enzensberger, Oliver Graupner, Hakan Akintürk, Can Yerebakan, Markus Khalil, Dietmar Schranz, Roland Axt-Fliedner
PURPOSE: We compared outcome of neonates with prenatal and postnatal diagnosis of congenital heart disease presenting in our paediatric heart centre between 2005-05/2015 who underwent an emergent intervention within 48 hours postpartum. MATERIALS AND METHODS: In 52/111 (46.8%) with emergent intervention, congenital heart disease was diagnosed prenatally, in 59/111 (53.2%) with no specialized fetal echocardiography, diagnosis was made postnatally. In 98/111 (88...
July 10, 2017: Journal of Maternal-fetal & Neonatal Medicine
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