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https://www.readbyqxmd.com/read/28427830/liver-transplantation-for-hereditary-hemorrhagic-telangiectasia-a-systematic-review
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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-anti-angiogenic-tyrosine-kinase-inhibitors-on-an-animal-model-of-hereditary-hemorrhagic-telangiectasia
#7
Yong Hwan Kim, Mi-Jung Kim, Se-Woon Choe, Dennis Sprecher, Young Jae Lee, S Paul Oh
BACKGROUND: Epistaxis and gastrointestinal (GI) tract hemorrhages are common symptoms of aged hereditary hemorrhagic telangiectasia (HHT) patients that result in anemia. Clinical as well as animal studies have suggested that vascular endothelial growth factor (VEGF) neutralizing antibodies lessen hemorrhage associated with adult onset arteriovenous malformations (AVMs). OBJECTIVES: The goal of this study is to evaluate potential therapeutic effects of oral delivery of four anti-angiogenic tyrosine-kinase inhibitors (TKIs) in the development of adult onset AVMs in a murine model of HHT...
March 24, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28316639/feature-extraction-and-classification-of-ehg-between-pregnancy-and-labour-group-using-hilbert-huang-transform-and-extreme-learning-machine
#8
Lili Chen, Yaru Hao
Preterm birth (PTB) is the leading cause of perinatal mortality and long-term morbidity, which results in significant health and economic problems. The early detection of PTB has great significance for its prevention. The electrohysterogram (EHG) related to uterine contraction is a noninvasive, real-time, and automatic novel technology which can be used to detect, diagnose, or predict PTB. This paper presents a method for feature extraction and classification of EHG between pregnancy and labour group, based on Hilbert-Huang transform (HHT) and extreme learning machine (ELM)...
2017: Computational and Mathematical Methods in Medicine
https://www.readbyqxmd.com/read/28314138/depression-and-post-traumatic-stress-disorder-in-individuals-with-hereditary-hemorrhagic-telangiectasia-a-cross-sectional-survey
#9
Shruti Chaturvedi, Marianne Clancy, Nicole Schaefer, Olalekan Oluwole, Keith R McCrae
INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is characterized by frequent severe bleeding, particularly epistaxis, and life-threatening complications including stroke, brain abscess and heart failure. The psychological impact of HHT is not known. We conducted this cross sectional study to determine the prevalence of depression and post-traumatic stress disorder (PTSD) related to HHT. METHODS: A survey tool comprising demographic and clinical information and two validated self-administered questionnaires, the PTSD checklist for DSM-5 (PCL-5) and Beck Depression Inventory-II (BDI-II), was distributed to individuals with HHT...
March 9, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28298578/hereditary-hemorrhagic-telangiectasia-management
#10
Jeffrey Peterson
Hereditary hemorrhagic telangiectasia (HHT) is an inherited disorder that can lead to frequent and severe sequelae. Although the condition has no cure, many of its physical symptoms can be managed to improve the quality of life and lower the risk of life-threatening complications. This article discusses HHT presentation, genetic screening, diagnosis, and management. The management of HHT can involve interventional radiology techniques, particularly for patients with pulmonary arteriovenous malformations.
January 2017: Radiologic Technology
https://www.readbyqxmd.com/read/28297555/epistaxis-grading-in-osler-s-disease-comparison-of-comprehensive-scores-with-detailed-bleeding-diaries
#11
Thomas Parzefall, Axel Wolf, Klemens Frei, Alexandra Kaider, Dominik Riss
BACKGROUND: Use of reliable grading scores to measure epistaxis severity in hereditary hemorrhagic telangiectasia (HHT) is essential in clinical routine and for scientific purposes. For practical reasons, visual analog scale (VAS) scoring and the Epistaxis Severity Score (ESS) are widely used. VAS scores are purely subjective, and a potential shortcoming of the ESS is that it is based on self-reported anamnestic bleeding data. The aim of this study was to validate the level of correlation between VAS scores, the ESS, and actual bleeding events, based on detailed epistaxis diaries of patients...
March 2017: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/28282453/the-use-of-a-novel-signal-analysis-to-identify-the-origin-of-idiopathic-right-ventricular-outflow-tract-ventricular-tachycardia-during-sinus-rhythm-simultaneous-amplitude-frequency-electrogram-transformation-mapping
#12
Abigail Louise D Te, Satoshi Higa, Fa-Po Chung, Chin-Yu Lin, Men-Tzung Lo, Che-An Liu, Chen Lin, Yi-Chung Chang, Shih-Lin Chang, Li-Wei Lo, Yu-Feng Hu, Ta-Chuan Tuan, Tze-Fan Chao, Jonan Liao, Yao-Ting Chang, Chung-Hsing Lin, Yuan Hung, Shinya Yamada, Kuo-Li Pan, Yenn-Jiang Lin, Shih-Ann Chen
INTRODUCTION: The signal characteristics of intracardiac bipolar electrograms at the origin of idiopathic RVOT-VT during sinus rhythm remain unclear. OBJECTIVE: The study sought to develop a novel real-time/online technique, simultaneous amplitude frequency electrogram transformation (SAFE-T), to quantify and localize the diseased ventricular substrate in idiopathic RVOT-VT. METHODS: We retrospectively investigated the intracardiac bipolar recordings in 70 consecutive patients (26% male, mean age 42±12 years) who underwent successful radiofrequency catheter ablation of idiopathic RVOT-VT...
2017: PloS One
https://www.readbyqxmd.com/read/28268686/using-hilbert-huang-transform-to-assess-eeg-slow-wave-activity-during-anesthesia-in-post-cardiac-arrest-patients
#13
Jukka Kortelainen, Eero Vayrynen, Usko Huuskonen, Jouko Laurila, Juha Koskenkari, Janne T Backman, Seppo Alahuhta, Tapio Seppanen, Tero Ala-Kokko
Hypoxic ischemic encephalopathy (HIE) is a severe consequence of cardiac arrest (CA) representing a substantial diagnostic challenge. We have recently designed a novel method for the assessment of HIE after CA. The method is based on estimating the severity of the brain injury by analyzing changes in the electroencephalogram (EEG) slow wave activity while the patient is exposed to an anesthetic drug propofol in a controlled manner. In this paper, Hilbert-Huang Transform (HHT) was used to analyze EEG slow wave activity during anesthesia in ten post-CA patients...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28268498/phase-amplitude-coupling-analysis-for-seizure-evolvement-using-hilbert-huang-transform
#14
Hanyue Zhou, Ying Li, Yue-Loong Hsin, Wentai Liu
Recent studies show that the phase-amplitude coupling (PAC) may play a functional role in information processing and cognition. The conventional method for PAC analysis is implemented by using Fourier-based filters, which assumes the signal is stationary and linear. However, as biological signals are nonstationary and nonlinear, this method introduces inaccuracy. To solve this problem, a new method based on Hilbert Huang Transform (HHT) is proposed in this paper, and is applied to analyze intracranial signals from two epilepsy patients...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28267932/hemoglobin-is-a-vital-determinant-of-arterial-oxygen-content-in-hypoxemic-patients-with-pulmonary-arteriovenous-malformations
#15
Abeer Rizvi, Patricia Macedo, Lydia Babawale, Hannah C Tighe, J Michael B Hughes, James E Jackson, Claire L Shovlin
RATIONALE: Arterial partial pressure of oxygen (PaO2), and oxygen saturation (SaO2) are commonly measured in respiratory practice, but arterial oxygen content (CaO2) refers to the volume of oxygen delivered to the tissues per unit blood volume. CaO2 is calculated from SaO2 and the hemoglobin concentration in blood, recognizing that each gram of hemoglobin can transport approximately 1.34mls of oxygen when fully saturated. OBJECTIVES: To prospectively evaluate serial changes in CaO2 in man, incorporating and excluding dynamic changes to oxygenation and hemoglobin parameters that may occur during life...
March 7, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28261074/frequency-clustering-analysis-for-resting-state-functional-magnetic-resonance-imaging-based-on-hilbert-huang-transform
#16
Xia Wu, Tong Wu, Chenghua Liu, Xiaotong Wen, Li Yao
Objective: Exploring resting-state functional networks using functional magnetic resonance imaging (fMRI) is a hot topic in the field of brain functions. Previous studies suggested that the frequency dependence between blood oxygen level dependent (BOLD) signals may convey meaningful information regarding interactions between brain regions. Methods: In this article, we introduced a novel frequency clustering analysis method based on Hilbert-Huang Transform (HHT) and a label-replacement procedure. First, the time series from multiple predefined regions of interest (ROIs) were extracted...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28256158/treatment-outcomes-and-patterns-of-radiologic-appearance-after-hypofractionated-image-guided-radiotherapy-delivered-with-helical-tomotherapy-hht-for-lung-tumours
#17
Stefano Arcangeli, Lorenzo Falcinelli, Stefano Bracci, Alessandro Greco, Alessia Monaco, Jessica Dognini, Cinzia Chiostrini, Rita Bellavita, Cynthia Aristei, Vittorio Donato
OBJECTIVE: To evaluate treatment outcomes and patterns of CT lung injury after hypofractionated image-guided radiotherapy delivered with helical tomotherapy (HHT) in a series of inoperable lung lesions. METHODS: 68 patients who were medically inoperable (69 lesions) without evidence of viable extrathoracic disease were included. Dose prescription was driven by tumour location (hilar/pericentral vs peripheral) and/or target volume. 52% of the lesions received a biological equivalent dose (BED10) ≥100 Gy...
March 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28248153/hepatic-angiodynamic-profile-in-paediatric-patients-with-hereditary-haemorrhagic-telangiectasia-type-1-and-type-2
#18
Paola Giordano, Mariantonietta Francavilla, Paolo Buonamico, Patrizia Suppressa, Patrizia Lastella, Maria Sangerardi, Vito L Miniello, Arnaldo Scardapane, Gennaro M Lenato, Carlo Sabbà
Liver involvement is a common manifestation of hereditary haemorrhagic telangiectasia (HHT). Although a number of studies have been carried out in adult patients, no study has ever been focused on investigating HHT-related hepatic involvement in paediatric patients. The present study aimed for the first time to systematically estimate the prevalence of HHT-associated liver involvement and to characterize HHT-associated hepatic angiodynamic features in paediatric age. Patients and methods: The study was designed as a cross-sectional survey in an HHT paediatric cohort, subclassified as HHT1 and HHT2 according to the mutated gene...
March 1, 2017: VASA. Zeitschrift Für Gefässkrankheiten
https://www.readbyqxmd.com/read/28245379/-effect-of-homoharringtonine-combined-with-imatinib-on-the-k562-g01-cells-and-its-mechanism
#19
Jing-Jing Wu, Yi-Han Ding, Zhi-Kui Deng, Yu-Ye Shi, Xue-Ying Lu, Yu-Feng Li
OBJECTIVE: To explore the effect of homoharringtonine(HHT) combined with imatinib(IM) on proliferation and apoptosis of K562/G01 cells and its potential mechanism. METHODS: K562/G01 cells were cultured with HHT and/or IM. CCK-8 assay was used to detect cell proliferation. Cell apoptosis and phosphorylated tyrosine levels were analyzed by flow cytometry. The expression levels of p210, PI3K, p-Akt and Akt protein were determined by Western blot. RESULTS: Compared with HHT or IM alone, drug combination significantly inhibited cell proliferation and induced apoptosis of K562/G01 cells (both P< 0...
February 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28226873/using-hilbert-huang-transform-to-assess-eeg-slow-wave-activity-during-anesthesia-in-post-cardiac-arrest-patients
#20
Jukka Kortelainen, Eero Vayrynen, Usko Huuskonen, Jouko Laurila, Juha Koskenkari, Janne T Backman, Seppo Alahuhta, Tapio Seppanen, Tero Ala-Kokko, Jukka Kortelainen, Eero Vayrynen, Usko Huuskonen, Jouko Laurila, Juha Koskenkari, Janne T Backman, Seppo Alahuhta, Tapio Seppanen, Tero Ala-Kokko, Eero Vayrynen, Janne T Backman, Jukka Kortelainen, Tapio Seppanen, Jouko Laurila, Usko Huuskonen, Seppo Alahuhta, Tero Ala-Kokko, Juha Koskenkari
Hypoxic ischemic encephalopathy (HIE) is a severe consequence of cardiac arrest (CA) representing a substantial diagnostic challenge. We have recently designed a novel method for the assessment of HIE after CA. The method is based on estimating the severity of the brain injury by analyzing changes in the electroencephalogram (EEG) slow wave activity while the patient is exposed to an anesthetic drug propofol in a controlled manner. In this paper, Hilbert-Huang Transform (HHT) was used to analyze EEG slow wave activity during anesthesia in ten post-CA patients...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
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