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Melanie Baxter, Lori Erby, Debra Roter, Barbara A Bernhardt, Peter Terry, Alan Guttmacher
PURPOSE: This study aimed to identify factors that influence screening behaviors of adults with hereditary hemorrhagic telangiectasia (HHT). METHODS: Participants with a self-reported diagnosis of HHT were recruited from the HHT Foundation International, Inc.; the "HHT Awareness" Facebook group; and six HHT clinics. A cross-sectional mixed methods survey was administered to investigate the relationships among the Health Belief model constructs, the domains of illness representations, and HHT-specific screening behaviors consistent with recommended guidelines...
October 13, 2016: Genetics in Medicine: Official Journal of the American College of Medical Genetics
Claire L Shovlin, Trishan Patel, James E Jackson
Pulmonary AVM embolisation appears to improve nosebleed severity for nearly one in six people with HHT
April 2016: ERJ Open Research
Trishan Patel, Amy Elphick, James E Jackson, Claire L Shovlin
OBJECTIVE: To evaluate if injection of intravenous particles may provoke migraines in subjects with right-to-left shunts due to pulmonary arteriovenous malformations (AVMs). BACKGROUND: Migraine headaches commonly affect people with hereditary hemorrhagic telangiectasia (HHT), especially those with pulmonary AVMs that provide right-to-left shunts. In our clinical practice, patients occasionally reported acute precipitation of migraine headaches following injection of technetium-labeled albumin macroaggregates for nuclear medicine scans...
October 11, 2016: Headache
Stephen S Y Lam, Eric S K Ho, Bai-Liang He, Wui-Wing Wong, Chae-Yin Cher, Nelson K L Ng, Cheuk-Him Man, Harinder Gill, Alice M S Cheung, Ho-Wan Ip, Chi-Chiu So, Jerome Tamburini, Chi Wai Eric So, Dona N Ho, Chun-Hang Au, Tsun-Leung Chan, Edmond S K Ma, Raymond Liang, Yok-Lam Kwong, Anskar Y H Leung
An in vitro drug-screening platform on patient samples was developed and validated to design personalized treatment for relapsed/refractory acute myeloid leukemia (AML). Unbiased clustering and correlation showed that homoharringtonine (HHT), also known as omacetaxine mepesuccinate, exhibited preferential antileukemia effect against AML carrying internal tandem duplication of fms-like tyrosine kinase 3 (FLT3-ITD). It worked synergistically with FLT3 inhibitors to suppress leukemia growth in vitro and in xenograft mouse models...
October 5, 2016: Science Translational Medicine
Misako Shigematsu, Tomoaki Koga, Ayako Ishimori, Kazuko Saeki, Yumiko Ishii, Yoshitaka Taketomi, Mai Ohba, Airi Jo-Watanabe, Toshiaki Okuno, Norihiro Harada, Takeshi Harayama, Hideo Shindou, Jian-Dong Li, Makoto Murakami, Sumio Hoka, Takehiko Yokomizo
Although pneumococcal infection is a serious problem worldwide and has a high mortality rate, the molecular mechanisms underlying the lethality caused by pneumococcus remain elusive. Here, we show that BLT2, a G protein-coupled receptor for leukotriene B4 and 12(S)-hydroxyheptadecatrienoic acid (12-HHT), protects mice from lung injury caused by a pneumococcal toxin, pneumolysin (PLY). Intratracheal injection of PLY caused lethal acute lung injury (ALI) in BLT2-deficient mice, with evident vascular leakage and bronchoconstriction...
October 5, 2016: Scientific Reports
Meiyu Chen, Fei Xiong, Liang Ma, Hong Yao, Qinrong Wang, Lijun Wen, Qian Wang, Ning Gu, Suning Chen
Homoharringtonine (HHT), a natural cephalotaxine alkaloid, has been used in the People's Republic of China for treatment of leukemia for >3 decades. Here, we employed magnetic Fe3O4 nanoparticles (MNP-Fe3O4) to improve the therapeutic effect of HHT and investigated its biological effects. Within a certain range of concentrations, the HHT-MNP-Fe3O4 showed a more enhanced inhibitory effect on the selected myeloid leukemia cell lines than HHT alone. Compared with HHT, HHT-MNP-Fe3O4 could induce more extensive apoptosis in leukemia cells, which also showed more pronounced cell arrests at G0/G1 phase...
2016: International Journal of Nanomedicine
Nicolas Baeyens, Bruno Larrivée, Roxana Ola, Brielle Hayward-Piatkowskyi, Alexandre Dubrac, Billy Huang, Tyler D Ross, Brian G Coon, Elizabeth Min, Maya Tsarfati, Haibin Tong, Anne Eichmann, Martin A Schwartz
Morphogenesis of the vascular system is strongly modulated by mechanical forces from blood flow. Hereditary hemorrhagic telangiectasia (HHT) is an inherited autosomal-dominant disease in which arteriovenous malformations and telangiectasias accumulate with age. Most cases are linked to heterozygous mutations in Alk1 or Endoglin, receptors for bone morphogenetic proteins (BMPs) 9 and 10. Evidence suggests that a second hit results in clonal expansion of endothelial cells to form lesions with poor mural cell coverage that spontaneously rupture and bleed...
September 26, 2016: Journal of Cell Biology
Christine W Duarte, Adam W Black, F Lee Lucas, Calvin P H Vary
PURPOSE: Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder characterized by deficiency in endoglin, an angiogenic protein. We previously showed that HHT, in which systemic endoglin expression is reduced, was associated with better survival outcomes in cancer patients (Duarte et al. in Cancer Epidemiol Biomarkers Prev 23:117-125, 2014). Here, we evaluated whether HHT was associated with reduced cancer incidence. METHODS: A matched case-control analysis using SEER Medicare was conducted to evaluate the effect of HHT on diagnosis with breast, colorectal, lung, or prostate cancer between 2000 and 2007 (n = 633,162)...
September 16, 2016: Journal of Cancer Research and Clinical Oncology
Jong Woo Choi, Yeon Hee Joo, Woo Shik Jeong, Yong Ju Jang
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant multi-systemic disease that exhibits increasing penetrance with age. Some patients present with severe life-threatening epistaxis which is intractable to all common treatment modalities. A 63-year-old female patient with hereditary hemorrhagic telangiectasia (HHT) presented with recurrent life-threatening epistaxis that required repeated transfusions despite multiple embolizations and septodermoplasty. Previous septodermoplasty failed due to septal perforation...
September 8, 2016: Auris, Nasus, Larynx
Baran Yılmaz, Zafer Orkun Toktaş, Akın Akakın, Semra Işık, Kaya Bilguvar, Türker Kılıç, Murat Günel
OBJECTIVE Brain arteriovenous malformations (AVMs) can occur in patients with hereditary hemorrhagic telangiectasia (HHT). However, brain AVM without HHT has also been reported. Using whole exome sequencing, the authors performed comprehensive genomic characterization of a 6-person Turkish family with 3 cases of brain AVM without HHT. METHODS Three siblings with brain AVM, one of whom also had spinal AVM, were evaluated. The parents and the fourth sibling had no AVM on cranial MRI. The authors performed a whole exome capture and Illumina sequencing on blood samples from 2 siblings with AVM...
September 9, 2016: Journal of Neurosurgery
Junichi Miyamoto, Kyo Niijima
An intracranial dural arteriovenous fistula(dAVF)was incidentally detected in a 39-year-old man during a medical checkup. Except for a mild episode of pneumonia at the age of 22 years, his medical history was unremarkable. He had no family history of hereditary hemorrhagic telangiectasia(HHT). The dAVF was treated radically via ligation of the fistula, without any complications. Postoperative angiography demonstrated that the dAVF had completely healed, but showed an aberrant, dilatated, and tortuous internal mammary artery...
September 2016: No Shinkei Geka. Neurological Surgery
Kevin J Whitehead, Nathan B Sautter, Justin P McWilliams, Murali M Chakinala, Christian A Merlo, Maribeth H Johnson, Melissa James, Eric M Everett, Marianne S Clancy, Marie E Faughnan, S Paul Oh, Scott E Olitsky, Reed E Pyeritz, James R Gossage
IMPORTANCE: Epistaxis is a major factor negatively affecting quality of life in patients with hereditary hemorrhagic telangiectasia (HHT; also known as Osler-Weber-Rendu disease). Optimal treatment for HHT-related epistaxis is uncertain. OBJECTIVE: To determine whether topical therapy with any of 3 drugs with differing mechanisms of action is effective in reducing HHT-related epistaxis. DESIGN, SETTING, AND PARTICIPANTS: The North American Study of Epistaxis in HHT was a double-blind, placebo-controlled randomized clinical trial performed at 6 HHT centers of excellence...
September 6, 2016: JAMA: the Journal of the American Medical Association
Sophie Dupuis-Girod, Alexis Ambrun, Evelyne Decullier, Anne-Emmanuelle Fargeton, Adeline Roux, Valentine Bréant, Bettina Colombet, Sophie Rivière, César Cartier, Pascal Lacombe, Thierry Chinet, Sandra Blivet, Jean-Hugues Blondel, Brigitte Gilbert-Dussardier, Xavier Dufour, Justin Michel, Jean-Robert Harle, Patrick Dessi, Frédéric Faure
BACKGROUND: Epistaxis is the most frequent and disabling manifestation of hereditary hemorrhagic telangiectasia (HHT). The efficacy of intravenous bevacizumab (an anti-vascular endothelial growth factor monoclonal antibody) for epistaxis has been shown. However, the efficacy of intranasal bevacizumab has yet to be evaluated. OBJECTIVE: To evaluate the efficacy of 3 different doses of bevacizumab administered as a nasal spray in a repeated manner for the duration of nosebleeds in patients with HHT...
September 6, 2016: JAMA: the Journal of the American Medical Association
Candice A Myers, Jeffrey N Keller, H Raymond Allen, Robert M Brouillette, Heather Foil, Allison B Davis, Frank L Greenway, William D Johnson, Corby K Martin
Dementia is a chronic condition in the elderly and depression is often a concurrent symptom. As populations continue to age, accessible and useful tools to screen for cognitive function and its associated symptoms in elderly populations are needed. The aim of this study was to test the reliability and validity of a new internet-based assessment battery for screening mood and cognitive function in an elderly population. Specifically, the Helping Hand Technology (HHT) assessments for depression (HHT-D) and global cognitive function (HHT-G) were evaluated in a sample of 57 elderly participants (22 male, 35 female) aged 59-85 years...
August 31, 2016: Journal of Alzheimer's Disease: JAD
Wei Li, Richard M Salmon, He Jiang, Nicholas W Morrell
Bone morphogenetic protein (BMP)9 and BMP10 are high affinity ligands for activin receptor-like kinase 1 (ALK1), a type I BMP receptor mainly expressed on vascular endothelial cells (ECs). ALK1-mediated BMP9/BMP10 signalling pathways have emerged as essential in EC biology and in angiogenesis. Several genetic mutations in the genes encoding the ligands and receptors of this pathway have been reported in two cardiovascular diseases, pulmonary arterial hypertension (PAH) and hereditary haemorrhagic telangiectasia (HHT)...
August 15, 2016: Biochemical Society Transactions
Joseph G Parambil
Hereditary hemorrhagic telangiectasia (HHT) is an underrecognized and underdiagnosed autosomal-dominant angiodysplasia that has an estimated prevalence of 1 in 5000 individuals, with variable clinical presentations even within family members with identical mutations. The most common manifestations are telangiectasias of the skin and nasal mucosa. However, HHT can often be complicated by the presence of arteriovenous malformations and telangiectasias in the lungs, brain, gastrointestinal tract, and liver that are often silent and can lead to life-threatening complications of stroke and hemorrhage...
September 2016: Clinics in Chest Medicine
Fang-Xia Wang, Wang-Gang Zhang, Ai-Li He, Xin-Mei Cao, Yin-Xia Chen, Wan-Hong Zhao, Yun Yang, Jian-Li Wang, Peng-Yu Zhang, Liu-Fang Gu
As sensitization of leukemia cells with granulocyte colony-stimulating factor (G-CSF) can enhance the cytotoxicity of chemotherapy in myeloid malignancies, a pilot study was conducted in order to evaluate the effect of G-CSF priming combined with low-dose chemotherapy in patients with higher risk myelodysplastic syndrome (MDS). The regimen, G-HA, consisted of cytarabine (Ara-C) 7.5mg/m(2)/12h by subcutaneous injection, days 1-14, homoharringtonine (HHT) 1.5mg/m(2)/day by intravenous continuous infusion, days 1-14, and G-CSF 150mg/m(2)/day by subcutaneous injection, days 0-14...
September 2016: Leukemia Research
Giorgos Papaspyrou, Bernhard Schick, Basel Al Kadah
BACKGROUND/AIMS: Extranasal telangiectasias are common amongst hereditary hemorrhagic telangiectasia (HHT) patients. Telangiectasias can be found at sites like the external nose, lips, oral cavity and fingers. Although not life threatening, they can be annoying for patients and lead to bleeding in some cases, necessitating treatment. METHODS: The data of 38 HHT patients treated for extranasal telangiectasias during a period of 10 years by means of Nd:YAG laser were retrospectively analyzed...
August 4, 2016: ORL; Journal for Oto-rhino-laryngology and its related Specialties
Kornelia E C Wirsching, Thomas S Kühnel
Objectives: Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of vascular malformations with an absence of capillaries between arteries and veins. One major manifestation site is the nasal mucous membrane where recurrent nosebleeds occur. Our clinical strategy to treat patients with HHT has the aim to reduce nasal bleeding long-term with minimal local and general side effects. Methods: We describe staged diagnosis and therapy including individual medical treatments of 97 patients with HHT...
July 21, 2016: Clinical and Experimental Otorhinolaryngology
Agnieszka A Książek, Katharyn J Mitchell, Laurent Morax, Colin C Schwarzwald, Simon P Hoerstrup, Benedikt Weber
BACKGROUND: Heterotopic heart transplantation (HHT) in rodent animal models represents an important technique enabling studies on organ transplantation immunology and pharmaceutical development. Recent investigations used nonworking HHT designs, with the left ventricle (LV) bypassed in the anastomosis system. In spite of their principal success, the lack of orthogonal ventricular filling leads to myocardial atrophy. However, when focusing on the cellular and molecular mechanisms involved in the in vivo remodeling of the myocardium or cell-based cardiovascular implants, a nonworking model is suboptimal as it lacks the native-analogous hemodynamic and metabolic situation...
July 20, 2016: European Surgical Research. Europäische Chirurgische Forschung. Recherches Chirurgicales Européennes
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