keyword
MENU ▼
Read by QxMD icon Read
search

HLH

keyword
https://www.readbyqxmd.com/read/27893685/acute-liver-failure-caused-by-hemophagocytic-lymphohistiocytosis-in-adults-a-case-report-and-review-of-the-literature
#1
Shide Lin, Ying Li, Jun Long, Qichuan Liu, Fangwan Yang, Yihuai He
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition that can be caused by a primary or acquired disorder of uncontrolled immune response. Liver injury is a common complication of HLH; however, HLH presenting as acute liver failure (ALF) has rarely been reported in adults. CASE SUMMARY: A 34-year-old man was admitted to our hospital with nausea and fatigue persisting for 2 weeks and jaundice for 1 week. He had hyperthermia at the onset of disease...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27884963/ectopic-fat-deposition-contributes-to-age-associated-pathology-in-caenorhabditis-elegans
#2
Konstantinos Palikaras, Meropi Mari, Barbara Petanidou, Angela Pasparaki, George Filippidis, Nektarios Tavernarakis
Age-dependent collapse of lipid homeostasis results in spillover of lipids and excessive fat deposition in non-adipose tissues. Ectopic fat contributes to lipotoxicity and has been implicated in the development of a metabolic syndrome that increases risk of age-associated diseases. However, the molecular mechanisms coupling ectopic fat accumulation with ageing remain obscure. Here, we use nonlinear imaging modalities to visualize and quantify age-dependent ectopic lipid accumulation in Caenorhabditis elegans...
November 24, 2016: Journal of Lipid Research
https://www.readbyqxmd.com/read/27883371/severe-fever-with-thrombocytopenia-syndrome-presenting-with-hemophagocytic-lymphohistiocytosis
#3
Jongmin Lee, Gyeongmin Jeong, Ji Hun Lim, Hawk Kim, Sun Whan Park, Won Ja Lee, Jae Bum Jun
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne disease caused by the newly discovered SFTS Bunyavirus, and there have been no case reports of SFTS patients presenting with hemophagocytic lymphohistiocytosis (HLH) in the English literature. We report a case of SFTS presenting with HLH in a 73-year-old immunocompetent male farmer. Although the patient had poor prognostic factors for SFTS, such as old age and central nervous system symptoms, he recovered fully with supportive care...
November 11, 2016: Infection & Chemotherapy
https://www.readbyqxmd.com/read/27879539/clinically-mild-encephalitis-encephalopathy-with-a-reversible-splenial-lesion-accompanied-by-epstein-barr-virus-hemophagocytic-lymphohistiocytosis-a-case-report-and-review-of-the-literature
#4
Hiroshi Yamaguchi, Toshiaki Ishida, Takehito Yokoi, Tsukasa Tanaka, Azusa Maruyama, Hiroaki Nagase, Daiichiro Hasegawa, Ken-Ichi Imadome, Hiroki Takeda, Yoshiyuki Kosaka, Yoshiyuki Uetani
Central nervous system involvement in hemophagocytic lymphohistiocytosis (HLH) is associated with a poor outcome. For such patients, it is unknown whether more aggressive therapies, such as intrathecal methotrexate or hydrocortisone, are inevitably required. We present a very rare case of 3-year-old Japanese girl who developed mild encephalitis/encephalopathy with a reversible splenial lesion, accompanied by Epstein-Barr virus-associated HLH, and review previous similar reports. Our case and previous reports suggest that mild encephalitis/encephalopathy with a reversible splenial lesion accompanied by Epstein-Barr virus-associated HLH has a relatively good prognosis, even in the absence of intrathecal treatments...
November 22, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27879391/rice-leaf-angle-and-grain-size-are-affected-by-the-osbul1-transcriptional-activator-complex
#5
Seonghoe Jang, Gynheung An, Hsing-Yi Li
Rice atypical HLH protein Oryza sativa BRASSINOSTEROID UPREGULATED 1-LIKE 1 (OsBUL1) is preferentially expressed in the laminar joint where it controls cell elongation and positively affects leaf angles. OsBUL1 knockout mutant (osbul1) and transgenic rice for double stranded RNA interference (dsRNAi) of OsBUL1 produced erected leaves with smaller grains whereas OsBUL1 overexpressors and an activation tagging line of OsBUL1 exhibited increased laminar inclination and grain size. Moreover, OsBUL1 expression was induced by brassinolide (BL) and osbul1 did not respond to BL treatment...
November 22, 2016: Plant Physiology
https://www.readbyqxmd.com/read/27875098/hlh-30-tfeb-mediated-autophagy-functions-in-a-cell-autonomous-manner-for-epithelium-intrinsic-cellular-defense-against-bacterial-pore-forming-toxin-in-c-elegans
#6
Huan-Da Chen, Cheng-Yuan Kao, Bang-Yu Liu, Shin-Whei Huang, Cheng-Ju Kuo, Jhen-Wei Ruan, Yen-Hung Lin, Cheng-Rung Huang, Yu-Hung Chen, Horng-Dar Wang, Raffi V Aroian, Chang-Shi Chen
Autophagy is an evolutionarily conserved intracellular system that maintains cellular homeostasis by degrading and recycling damaged cellular components. The transcription factor HLH-30/TFEB-mediated autophagy has been reported to regulate tolerance to bacterial infection, but less is known about the bona fide bacterial effector that activates HLH-30 and autophagy. Here, we unveil that bacterial membrane pore-forming toxin (PFT) induces autophagy in an HLH-30-dependent manner in Caenorhabditis elegans. Moreover, autophagy controls the susceptibility of animals to PFT toxicity through xenophagic degradation of PFT and repair of membrane-pore cell-autonomously in the PFT-targeted intestinal cells in C...
November 22, 2016: Autophagy
https://www.readbyqxmd.com/read/27862115/pediatric-acute-liver-failure-of-undetermined-cause-a-research-workshop
#7
Estella M Alonso, Simon P Horslen, Edward M Behrens, Edward Doo
: Pediatric Acute liver failure (PALF) is a potentially devastating condition which occurs in previously healthy children of all ages and frequently leads to a rapid clinical deterioration. An identified cause for liver injury is lacking in approximately 30% of cases. Children with undetermined diagnosis have lower spontaneous survival and higher rates of transplantation and death than other diagnostic groups. A single day workshop sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases brought together clinicians and basic scientists to integrate aligned research findings and develop a foundation for new mechanistic studies and future treatment trials...
November 14, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27859590/miz-1-and-max-compete-to-engage-c-myc-implication-for-the-mechanism-of-inhibition-of-c-myc-transcriptional-activity-by-miz-1
#8
Mikaël Bédard, Loïka Maltais, Martin Montagne, Pierre Lavigne
c-Myc is a basic Helix-Loop-Helix Leucine Zipper (b-HLH-LZ) transcription factor deregulated in the majority of human cancers. As a heterodimer with Max, another b-HLH-LZ transcription factor, deregulated and persistent c-Myc accumulates at transcriptionally active promoters and enhancers and amplifies transcription. This leads to the so-called transcriptional addiction of tumor cells. Recent studies have showed that the c-Myc transcriptional activities can be reversed by its association with Miz-1, a POZ transcription factor containing 13 classical zinc fingers...
November 16, 2016: Proteins
https://www.readbyqxmd.com/read/27853859/hemophagocytic-lymphohistiocytosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome-case-report-and-review
#9
REVIEW
L García-Montoya, C N Sáenz-Tenorio, I Janta, J Menárguez, F J López-Longo, I Monteagudo, E Naredo
Hemophagocytic lymphohistiocytosis (HLH) is a very rare syndrome with a mortality up to 95% of cases if not treated. It is characterised by an excessive activation of the immune system that leads to a disproportionate and destructive inflammatory response. The high mortality rates are in part due to a delay in the diagnosis, and therefore clinicians must maintain a high index of suspicion. When the treatment is started early, the survival rate reaches around 55% of cases. HLH usually presents with persistent fever, pancytopenia, and organomegaly and is associated with very high levels of serum ferritin...
November 16, 2016: Rheumatology International
https://www.readbyqxmd.com/read/27851371/1736-looking-beyond-acute-chest-syndrome-hlh-due-to-granulomatosis-polyangiitis-in-sickle-cell-crisis
#10
Daniel Lercher, Adam Bensimhon, Keia Sanderson, Afsaneh Pirzadeh
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27851370/1735-acute-onset-malignant-hypertension-and-atrial-fibrillation-in-a-patient-with-ebv-associated-hlh
#11
Whitney Marvin, Helen Harvey, Jenny Kim, Matt Williams
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27850984/1348-immune-memory-alters-cytokine-storm-and-treatment-response-in-the-perforin-deficient-model-of-hlh
#12
Matthew Taylor, Edward Behrens
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27847518/adult-onset-still-s-disease-associated-with-mycoplasma-pneumoniae-infection-and-hemophagocytic-lymphohistiocytosis
#13
Abhishek Agnihotri, Allison Ruff, Lauren Gotterer, Addie Walker, Amy H McKenney, Andrei Brateanu
Adult Onset Still's Disease (AOSD) is a systemic inflammatory disorder that can be associated with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease of overactive histiocytes and lymphocytes. We present a unique case of AOSD complicated by Mycoplasma pneumonia infection and HLH. A 28-year-old female developed joint pains followed by a diffuse, erythematous, pruritic skin rash that quickly spread throughout the body. The patient deteriorated and developed fever, chills, cough, and dyspnea and had to be intubated...
2016: Case Reports in Medicine
https://www.readbyqxmd.com/read/27846659/the-non-langerhans-cell-histiocytoses-rare-histiocytoses-clinical-aspects-and-therapeutic-approaches
#14
C F Classen, M Minkov, T Lehrnbecher
Rare histiocytoses, also called non-Langerhans cell histiocytoses, include all proliferative disorders of histiocytes, macrophages and dendritic cells that are not classified as Langerhans cell histiocytosis (LCH) and do not belong to the hemophagocytic lymphohistiocytosis (HLH) group of diseases. Thus, the term includes numerous benign or malignant, localized or systemic, adult or pediatric diseases. The classification of the histiocytic disorders has been revised several times. Here, we follow the classification recently published by Jean Francois Emile and an international expert panel, defining subgroups of histiocytoses described as L-Group, C-Group, M-Group, R-Group, and H-Group, which stands for LCH-like, cutaneous or mucocutaneous, malignant, Rosai-Dorfman-Disease like and HLH like...
November 2016: Klinische Pädiatrie
https://www.readbyqxmd.com/read/27831908/diagnosing-haemophagocytic-syndrome
#15
REVIEW
Ethan S Sen, Colin G Steward, Athimalaipet V Ramanan
Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a hyperinflammatory disorder characterised by uncontrolled activation of the immune system. It can result from mutations in multiple genes involved in cytotoxicity or occur secondary to a range of infections, malignancies or autoimmune rheumatic diseases. In the latter case, it is also known as macrophage activation syndrome (MAS). Characteristic features are persistent fever, hepatosplenomegaly, petechial/purpuric rash, progressive cytopenias, coagulopathy, transaminitis, raised C reactive protein, falling erythrocyte sedimentation rate, hypertriglyceridaemia, hypofibrinogenaemia and extreme hyperferritinaemia often associated with multi-organ impairment...
October 24, 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27830097/histoplasmosis-induced-hemophagocytic-lymphohistiocytosis-in-an-adult-patient-a-case-report-and-review-of-the-literature
#16
Ramona Vesna Untanu, Syed Akbar, Stephen Graziano, Neerja Vajpayee
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threating immune dysregulation syndrome characterized by persistent activation of the mononuclear phagocytic system leading to uncontrolled systemic hyperinflammatory response. The proliferation and activation of histiocytes and lymphocytes lead to production of large amounts of cytokines, also called cytokine storm. Hematopoietic and lymphoid tissues are directly involved while other organs are damaged by circulating cytokines. Primary HLH is attributed to genetic defects of the immune system and secondary HLH is usually seen in adults secondary to malignancy, infection, or autoimmune diseases...
2016: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/27826329/recurrent-macrophage-activation-syndrome-since-toddler-age-in-an-adolescent-boy-with-hla-b27-positive-juvenile-ankylosing-spondylitis
#17
Joon Hyeong Park, Yu Mi Seo, Seung Beom Han, Ki Hwan Kim, Jung Woo Rhim, Nack Gyun Chung, Myung Shin Kim, Jin Han Kang, Dae Chul Jeong
Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis (HLH) 4 different times, which was well controlled with intravenous immunoglobulin and steroids since the age of 3...
October 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/27815752/hematopoietic-stem-cell-transplantation-for-xiap-deficiency-in-japan
#18
Shintaro Ono, Tsubasa Okano, Akihiro Hoshino, Masakatsu Yanagimachi, Kazuko Hamamoto, Yozo Nakazawa, Toshihiko Imamura, Masaei Onuma, Hidetaka Niizuma, Yoji Sasahara, Hiroshi Tsujimoto, Taizo Wada, Reiko Kunisaki, Masatoshi Takagi, Kohsuke Imai, Tomohiro Morio, Hirokazu Kanegane
BACKGROUND: X-linked inhibitor of apoptosis protein (XIAP) deficiency is a rare immunodeficiency that is characterized by recurrent hemophagocytic lymphohistiocytosis (HLH) and splenomegaly and sometimes associated with refractory inflammatory bowel disease (IBD). Although hematopoietic stem cell transplantation (HSCT) is the only curative therapy, the outcomes of HSCT for XIAP deficiency remain unsatisfactory compared with those for SLAM-associated protein deficiency and familial HLH...
November 4, 2016: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27813134/establishment-and-characterization-of-a-novel-hodgkin-lymphoma-cell-line-am-hlh-carrying-the-epstein-barr-virus-genome-integrated-into-the-host-chromosome
#19
Masahiko Hayashida, Masanori Daibata, Erika Tagami, Takahiro Taguchi, Fumiyo Maekawa, Kayo Takeoka, Katsuhiro Fukutsuka, Daiki Shimomura, Takamasa Hayashi, Yoshinori Iwatani, Hitoshi Ohno
We describe the establishment and characterization of a cell line, AM-HLH, obtained from a patient with Epstein-Barr virus-positive (EBV(+) ) nodular sclerosis-type Hodgkin lymphoma (HL). The cells were positive for CD2 and CD30 and negative for CD15. The immunoglobulin heavy- and κ light-chain genes were rearranged. The karyotype was of the triploid range. Southern blotting using the EBV terminal repeat probe detected 3 hybridizing bands that were identical to those of the parental HL material. The cells expressed EBV-encoded RNAs as well as latent genes (EBNA1, EBNA2, LMP1, and LMP2A) and lytic genes (BZLF1 and BALF2)...
November 4, 2016: Hematological Oncology
https://www.readbyqxmd.com/read/27803821/hemophagocytic-lymphohistiocytosis-in-a-patient-with-classical-hodgkin-lymphoma
#20
G Hyun, K J Robbins, N Wilgus, L Grosso, S D Goyal
Introduction. Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome that can be associated with inherited genetic mutations, malignancy, autoimmune disorders, and viral infections. Though the pathogenesis is not fully known, HLH is understood to be a reactive process in the setting of uncontrolled activation of macrophages, CD8+ cytotoxic lymphocytes, and other immune cells. Hallmark clinicopathological features of HLH include fevers, cytopenias, hepatosplenomegaly, and hemophagocytosis in the bone marrow...
2016: Case Reports in Hematology
keyword
keyword
23588
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"