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https://www.readbyqxmd.com/read/28549781/nucleostemin-dysregulation-contributes-to-ischemic-vulnerability-of-diabetic-hearts-role-of-ribosomal-biogenesis
#1
Shihao Zhao, Yunlong Xia, Fuyang Zhang, Zhenyu Xiong, Yueyang Li, Wenjun Yan, Xiyao Chen, Wei Wang, Helin Wang, Erhe Gao, Yan Lee, Congye Li, Shan Wang, Ling Zhang, Ling Tao
Diabetes is a major health problem worldwide. As well-known, diabetes greatly increases cardiac vulnerability to ischemia/reperfusion (I/R) injury, but the underlying mechanisms remain elusive. Nucleostemin (NS) is a nucleolar protein that controls ribosomal biogenesis and exerts cardioprotective effects against I/R injury. However, whether NS-mediated ribosomal biogenesis regulates ischemic vulnerability of diabetic hearts remains unanswered. Utilizing myocardial I/R mouse models, we found that cardiac NS expression significantly increased in response to I/R in normal diet (ND)-fed mice...
May 23, 2017: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/28524787/cerebral-hemorrhage-in-monozygotic-twins-with-hereditary-hemorrhagic-telangiectasia-case-report-and-hemorrhagic-risk-evaluation
#2
Abbas Rattani, Michael C Dewan, Vickie Hannig, Robert P Naftel, John C Wellons, Lori C Jordan
The authors present a case of monozygotic twins with hereditary hemorrhagic telangiectasia (HHT) who experienced cerebral arteriovenous malformation (AVM) hemorrhage at a very young age. The clinical variables influencing HHT-related AVM rupture are discussed, and questions surrounding the timing of screening and intervention are explored. This is only the second known case of monozygotic HHT twins published in the medical literature, and the youngest pair of first-degree relatives to experience AVM-related cerebral hemorrhage...
May 19, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28521822/central-nervous-system-manganese-induced-lesions-and-clinical-consequences-in-patients-with-hereditary-hemorrhagic-telangiectasia
#3
M M Serra, C H Besada, A Cabana Cal, A Saenz, C V Stefani, D Bauso, A B Golimstok, J C Bandi, D H Giunta, C M Elizondo
BACKGROUND: Around 47-74% of patients with hereditary hemorrhagic telangiectasia (HHT) have hepatic vascular malformations (HVMs); magnetic resonance images (MRI) of the central nervous system (CNS) might show in T1 sequences a hyper-intensity signal in different areas, mainly in the basal ganglia (BG) as consequence of manganese (Mn) deposits as observed in cirrhotic patients. These patients might suffer from different neuropsychiatric disorders (hepatic encephalopathy). In HHT patients, even in the presence of hepatic shunts, hepatocellular function is usually preserved...
May 18, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28494714/is-the-hepatic-factor-a-mirna-that-maintains-the-integrity-of-pulmonary-microvasculature-by-inhibiting-the-vascular-endothelial-growth-factor
#4
Joseph John Vettukattil
The "hepatic factor," a molecule or group of molecules present in the hepatic venous blood, essential for the prevention of the development of pulmonary arteriovenous malformations (PAVMs) and right-to-left shunting has been a conceptual enigma in the understanding of many related conditions. Patients with various forms of liver diseases including acute hepatic failure, and others with normal hepatic function like hereditary hemorrhagic telangiectasia (HHT), inflammatory and parasitic disorders, cardiogenic hepatopulmonary syndrome (cHPS) and skin disorders like Dyskeratosis congenita are all known to cause PAVMs...
May 9, 2017: Current Cardiology Reviews
https://www.readbyqxmd.com/read/28490409/endoscopic-guided-coblation-treatment-of-nasal-telangiectasias-in-hereditary-hemorrhagic-telangiectasia-how-i-do-it
#5
David M Poetker
INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease that leads to frequent epistaxis. It can have a significant impact on quality of life. Many reports exist regarding various therapies to address the epistaxis. MATERIALS AND METHODS: We presented our technique for addressing the epistaxis associated with HHT. RESULTS: Patients are treated in the operating room while they are under general anesthesia...
May 1, 2017: American Journal of Rhinology & Allergy
https://www.readbyqxmd.com/read/28487607/effects-of-hwangryunhaedok-tang-on-gastrointestinal-motility-function-in-mice
#6
Hyoyeon Kim, Iksung Kim, Min Cheol Lee, Hyun Jung Kim, Guem San Lee, Hyungwoo Kim, Byung Joo Kim
AIM: To investigate the effects of Hwangryunhaedok-tang (HHT) on gastrointestinal (GI) motility in mice. METHODS: The effects of a boiling water extract of HHT (HHTE) on GI motility were investigated by calculating percent intestinal transit rates (ITR%) and gastric emptying (GE) values using Evans Blue and phenol red, respectively, in normal mice and in mice with experimentally induced GI motility dysfunction (GMD). In addition, the effects of the four components of HHT, that is, Gardeniae Fructus (GF), Scutellariae Radix (SR), Coptidis Rhizoma (CR), and Phellodendri Cortex (PC), on GI motility were also investigated...
April 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28463768/homoharringtonine-enhances-bortezomib-antimyeloma-activity-in-myeloma-cells-adhesion-to-bone-marrow-stromal-cells-and-in-scid-mouse-xenografts
#7
Ping Chen, Qin Yuan, Hui Yang, Xiaofang Wen, Peidong You, Diyu Hou, Jieqiong Xie, Yu Cheng, Huifang Huang
Despite the great progress in the treatment, multiple myeloma (MM) still remains incurable. Bortezomib (BTZ), a reversible inhibitor of the 26S proteasome, is very effective against MM but unable to eradicate the MM cells in bone marrow niche eventually causing the disease relapse. Homoharringtonine (HHT) is a known anti-leukemia drug that inhibits MM both in vitro and in vivo. This study aimed to investigate whether HHT could potentiate the anti-tumor activity of BTZ in MM cells cocultured with bone marrow stromal cells and in vivo xenograft models...
April 25, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28461180/giant-symptomatic-aneurysm-secondary-to-hereditary-hemorrhagic-telangiectasia-of-a-main-hepatic-artery-with-aberrant-origin-in-superior-mesenteric-artery
#8
Francisco Javier Peinado Cebria N, Santiago Este Banez Seco, A Ngel Flores-Herrero, Ricardo Montoya Ching, Benito Mendez Feria, Diego Soto Valdés, Maria Pilar Lamarca Mendoza, Antonio Orgaz Pe Rez-Grueso
INTRODUCTION: Hereditary Hemorragic Telangiectasia (HHT) is a rare disease consistent in vascular dysplasias affecting different organs. Liver involvement includes telangiectases, arteriovenous shunting, and ischemic cholangitis, and its prevalence ranges from 8 to 31%. Spontaneous pseudo aneurysms have never been reported associated to HHT. Several variations in the origin of the main hepatic artery (MHA) have been described in large radiological series, and can be found in around 4% of general population...
April 28, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28454099/homoharringtonine-targets-smad3-and-tgf-%C3%AE-pathway-to-inhibit-the-proliferation-of-acute-myeloid-leukemia-cells
#9
Jian Chen, Qitian Mu, Xia Li, Xiufeng Yin, Mengxia Yu, Jing Jin, Chenying Li, Yile Zhou, Jiani Zhou, Shanshan Suo, Demin Lu, Jie Jin
Homoharringtonine (HHT) has long and widely been used in China for the treatment of acute myeloid leukemia (AML), the clinical therapeutic effect is significant but the working mechanism is poorly understood. The purpose of this study is to screen the possible target for HHT with virtual screening and verify the findings by cell experiments. Software including Autodock, Python, and MGL tools were used, with HHT being the ligand and proteins from PI3K-Akt pathway, Jak-stat pathway, TGF-β pathway and NK-κB pathway as the receptors...
April 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28446318/-research-progress-of-homoharringtonine-effect-on-im-resistant-chronic-myelogenous-leukemia-review
#10
Qian Wang, Yu-Feng Li
Homoharringtonine(HHT) is an alkaloid with anti-tumor activity, having a good therapeutic effect on chronic myeloid leukemia(CML), and its toxicity is much lower than other anti-cancer drugs. However, the remarkable therapeutic efficacy of imatinib on CML treatment made HHT to be forgotten gradually. Today, the omacetaxine mepesuccinate, a semisynthetic form of HHT, display a good clinical response to TKI-resistant CML patients. Therefore, the HHT again attracts more attention from the medical field. Here, the clinical effects of HHT on IM-resistant CML patients and its mechanism are briefly reviewed...
April 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28446303/-prognosis-and-survival-analysis-of-patients-with-essential-thrombocythemia
#11
Mo Chen, Li-Jun Hou, Zeng-Xiang Liu, Hong-Bing Liu, Qiao-Dan Liu
OBJECTIVE: To investigate the survival status and prognosis of patients with essential thrombocythemia(ET) and analyze the prognostic factors for the patients' survival, so as to provide a evidence for clinical treatment and prognosis evaluation. METHODS: A retrospective analysis of 118 patients with ET was conducted in the Fifth Affiliated Hospital of Sun Yat-Sen University and Zhongshan Municipale People's Hospital from December 2002 to December 2013. The clinical characteristics were summarized, such as the survival curve and multi-factor analysis, therefore looking for the disease characteristics and risk factors affecting the survival and prognosis...
April 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28446285/-effect-of-autophagy-on-homoharringtonine-treated-k562-cells
#12
Xue-Ying Lu, Li-Lin Ye, Yu-Ye Shi, Yi-Han Ding, Yu-Feng Li
OBJECTIVE: To study the effect of homoharringtonine (HHT) alone or combined with 3-methyladenine (3-MA) , an autophagy inhibitor, on the apoptosis and autophagy of K562 cells. METHODS: K562 cells were treated with HHT(10 ng/ml) or HHT(10 ng/ml) combined with 3-MA (1.5 mmol/L) for 1 to 8 days. The apoptosis of treated cells was tested by means of flow cytometry(FCM), and the autophagy levels were tested with RT-PCR, Western blot and electron microscopy. RESULTS: In the early stage of HHT-treated group, the apoptosis rate increased and decreased later...
April 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28446279/-effect-of-stromal-cell-co-culture-on-drug-resistance-of-acute-myeloid-leukemia-cells-and-its-mechanism
#13
Han Yue, Li-Ying Liu, Xiao-Qian Wang
OBJECTIVE: To study the effect of co-culture of stromal cells and acute myeloid leukemia(AML) cells on drug resistance of AML cells and its mechanism. METHODS: Stromal cells were co-cultured with acute myeloid leukemia cell HL-60 and then were treated with DNR, HHT and Ara-C for observing the sensitivity of HL-60 cells to drugs after incubation with HS-5. At the same time, the the inhibitor LY294002 of PI3K/AKT signaling pathway was used to treat the cells, so as to explore whether the changes of HL-60 sensitivity is associated with the activation of PI3K/AKT signal pathway after co-culture of cells...
April 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28427830/liver-transplantation-for-hereditary-hemorrhagic-telangiectasia-a-systematic-review
#14
REVIEW
Emanuele Felli, Pietro Addeo, François Faitot, Gennaro Nappo, Constantin Oncioiu, Philippe Bachellier
AIM: To evaluate the indications, timing and results of liver transplantation in patients affected by hereditary hemorrhagic telangiectasia (HHT), by undertaking a systematic review of the current literature. METHODS: Electronic bibliographical databases were searched on MEDLINE and Pubmed according to the PRISMA criteria. A total of 58 articles were initially found, 11 have been excluded because of single center series later included in the European Liver transplant Registry (ELTR), already reported in this study...
April 17, 2017: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
https://www.readbyqxmd.com/read/28420371/effect-of-pulmonary-arteriovenous-malformations-on-the-mechanical-properties-of-the-lungs
#15
Cécile Rotenberg, Marcel Bonay, Mostafa El Hajjam, Sandra Blivet, Alain Beauchet, Pascal Lacombe, Thierry Chinet
BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) are present in approximately 15-50% individuals with hereditary hemorrhagic telangiectasia (HHT). They may be isolated but more often are multiple. The goal of this study was to evaluate the influence of PAVMs on lung mechanical properties. METHODS: We reviewed the files of all adult patients (age ≥ 18 years) referred to our Center for evaluation of HHT between 2005 and 2013. The diagnosis of HHT was based on the Curacao criteria and/or the presence of a pathogenic mutation...
April 19, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28418273/thalidomide-for-epistaxis-in-patients-with-hereditary-hemorrhagic-telangiectasia-a-preliminary-study
#16
Jia Fang, Xiaomeng Chen, Bijun Zhu, Haibo Ye, Weitian Zhang, Jian Guan, Kaiming Su
To evaluate the effectiveness of thalidomide for epistaxis in hereditary hemorrhagic telangiectasia (HHT), 7 HHT patients with recurrent epistaxis were treated with thalidomide at an initial dose of 50 mg/d, gradually increasing to 100 mg/d if needed. The Epistaxis Severity Score (ESS) was used to evaluate the treatment effects. Patients reported that epistaxis improved 1 to 3 weeks after starting thalidomide. The mean ESS before treatment, at the end of treatment, and 3 months after stopping treatment was 5...
April 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28410239/homoharringtonine-suppresses-imatinib-resistance-via-the-bcl-6-p53-pathway-in-chronic-myeloid-leukemia-cell-lines
#17
Qian Wang, Wei Ding, Yihan Ding, Jingjing Ma, Zhaoye Qian, Jingxian Shao, Yufeng Li
BACKGROUND: The anti-leukemic mechanism of homoharringtonine (HHT) differs from that of IM, and HHT is one of the most useful agents for use in patients with IM resistance or intolerance. The Bcl-6/p53 pathway has been shown to regulate the sensitivity of tumor cells to antitumor drugs. We tested whether HHT blocked the Bcl-6/p53 pathway in order to promote the apoptosis of IM-resistant cells in vitro and in vivo. RESULTS: Ph+ acute lymphoblastic leukemia (ALL) cells and IM-resistant chronic myeloid leukemia (CML) cells showed high expression of Bcl-6 protein...
March 31, 2017: Oncotarget
https://www.readbyqxmd.com/read/28407366/screening-children-for-pulmonary-arteriovenous-malformations-evaluation-of-18-years-of-experience
#18
Anna E Hosman, Els M de Gussem, Walter A F Balemans, Andréanne Gauthier, Cees J J Westermann, Repke J Snijder, Marco C Post, Johannes J Mager
BACKGROUND: Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disease with multi-systemic vascular dysplasia. Early diagnosis through screening is important to prevent serious complications. How best to screen children of affected parents for pulmonary arteriovenous malformations (PAVMs) is often subject to debate. Transthoracic contrast echocardiogram (TTCE) is considered optimal in screening for PAVMs in adults. Guidelines for the screening of children are not specific, reflecting the lack of scientific evidence on the best method to use...
April 13, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28394685/a-case-report-of-hereditary-hemorrhagic-telangiectasia-diagnosed-in-a-podiatric-medical-patient
#19
Kunal Amin, Douglas Mckay
Hereditary hemorrhagic telangiectasia (HHT), which is also known as Osler-Weber-Rendu syndrome, is a group of related disorders characterized by the development of arteriovenous malformations. These malformations occur in almost all organs but predominantly in the skin, intestines, liver, lungs, and brain. This is a case report of a patient with cutaneous manifestations of HHT in the lower extremities as diagnosed by his podiatric physician. To our knowledge, the literature does not present any case reports in which cutaneous manifestations of the lower extremities followed by a further work-up allowed a diagnosis of HHT...
March 2017: Journal of the American Podiatric Medical Association
https://www.readbyqxmd.com/read/28339142/selective-effects-of-oral-antiangiogenic-tyrosine-kinase-inhibitors-on-an-animal-model-of-hereditary-hemorrhagic-telangiectasia
#20
Y H Kim, M-J Kim, S-W Choe, D Sprecher, Y J Lee, S P Oh
Essentials Antiangiogenic drugs are indicated as therapies for hereditary hemorrhagic telangiectasia. We interrogated the response to four antiangiogenic drugs for anemia and intestinal bleeding. Sorafenib and a pazopanib analog significantly improved while erlotinib worsened anemia. Some oral antiangiogenic drugs were effective in reducing intestinal bleeding. SUMMARY: Background Epistaxis and gastrointestinal (GI) tract hemorrhages are common symptoms of aged hereditary hemorrhagic telangiectasia (HHT) patients that result in anemia...
March 24, 2017: Journal of Thrombosis and Haemostasis: JTH
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