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hereditary hemorrhagic telangiectasia

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https://www.readbyqxmd.com/read/28070641/influence-of-temporary-nasal-occlusion-tno-on-epistaxis-frequency-in-patients-with-hereditary-hemorrhagic-telangiectasia-hht
#1
Kornelia E C Wirsching, Frank Haubner, Thomas S Kühnel
The objective of the study was to evaluate the influence of temporary nasal occlusion (tNO) with hypoallergenic tape on the frequency and severity of epistaxis in patients with hereditary hemorrhagic telangiectasia (HHT). This prospective observational study included 20 HHT patients who were undergoing Nd:YAG laser therapy at regular intervals. Over a 3-month period, laser therapy was supplemented by tNO with hypoallergenic tape for 5 h/day on average. On a 0-10 numeric rating scale, the patients reported significantly greater satisfaction in epistaxis terms after tNO treatment, with mean scores of 5 before and 7 after 3-month tNO (p = 0...
January 9, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28070235/bevacizumab-and-gastrointestinal-bleeding-in-hereditary-hemorrhagic-telangiectasia
#2
George Ou, Cherry Galorport, Robert Enns
We report a case of severe, refractory gastrointestinal (GI) bleeding in a patient with hereditary hemorrhagic telangiectasia (HHT) whose massive transfusion dependence was lifted shortly after treatment with bevacizumab, an anti-vascular endothelial growth factor. The patient's bleeding had been refractory to repeated endoscopic interventions, tranexamic acid, and tamoxifen. However, following treatment with bevacizumab at 5 mg/kg every other week, nearly 300 units of packed red blood cell transfusions were avoided in one year's time...
December 27, 2016: World Journal of Gastrointestinal Surgery
https://www.readbyqxmd.com/read/28059706/the-prevalence-of-malformations-of-cortical-development-in-a-pediatric-hereditary-hemorrhagic-telangiectasia-population
#3
G J Palagallo, S R McWilliams, L A Sekarski, A Sharma, M S Goyal, A J White
BACKGROUND AND PURPOSE: Brain AVM, cerebral abscess, and ischemic stroke are among the well known neurologic manifestations of hereditary hemorrhagic telangiectasia. However, recently reported data suggest an additional association with malformations of cortical development. The purpose of this study was to determine the prevalence of malformations of cortical development in a population of pediatric patients with hereditary hemorrhagic telangiectasia. MATERIALS AND METHODS: A retrospective review of brain MRIs from 116 pediatric patients was performed...
November 10, 2016: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28032956/efficacy-and-safety-of-propranolol-for-epistaxis-in-hereditary-hemorrhagic-telangiectasia-hht-retrospective-then-prospective-study-in-a-total-of-21-patients
#4
A Contis, N Gensous, J F Viallard, C Goizet, C Léauté-Labrèze, P Duffau
Hereditary Hemorrhagic Telangiectasia (HHT) is a genetic disorder of angiogenesis associated with disabling epistaxis. Propranolol, a beta-blocker, exerts anti-angiogenic properties. In the retrospective study, nine of ten HHT patients receiving propranolol significantly improved their Epistaxis Severity Score. In the prospective study, after three months of propranolol treatment, the median duration of epistaxis per month significantly decreased as did the number of epistaxis episodes per month in 11 HHT patients...
December 29, 2016: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28031662/hereditary-hemorrhagic-telangiectasia-and-myocardial-infarction
#5
Efrén Martínez-Quintana, Fayna Rodríguez-González, Silvia Gopar-Gopar
Hereditary hemorrhagic telangiectasia, also known as Osler-Weber-Rendu syndrome, is an autosomal dominant genetic disorder that leads to epistaxis, gastrointestinal bleeding, iron deficiency anemia, and arteriovenous malformations at the lungs, the liver, and the brain. However, due to its rarity and its unspecific findings on routine examinations, diagnosis is not easy unless suspected due to hypoxemia or paradoxical embolism. We present a case of a 46-year-old-woman with hereditary hemorrhagic telangiectasia and hypoxemia who presented a myocardial infarction secondary to paradoxical embolism through pulmonary arteriovenous malformations...
December 2016: International Journal of Angiology: Official Publication of the International College of Angiology, Inc
https://www.readbyqxmd.com/read/28008625/arterial-ligation-versus-embolization-in-epistaxis-management-counterintuitive-national-trends
#6
Michael J Sylvester, Sei Y Chung, Luis A Guinand, Aparna Govindan, Soly Baredes, Jean Anderson Eloy
OBJECTIVES/HYPOTHESIS: Arterial ligation and embolization are treatment modalities indicated in severe and refractory epistaxis. The purpose of this study was to examine temporal trends and compare outcomes in treatment of hospitalized epistaxis patients with ligation or embolization. METHODS: This retrospective cohort analysis utilized the 2008 to 2013 National Inpatient Sample to identify patients admitted with a primary diagnosis of epistaxis, and an associated procedure code for ligation or embolization...
December 23, 2016: Laryngoscope
https://www.readbyqxmd.com/read/28004998/de-novo-vascular-lesions-after-liver-transplant-in-a-case-with-hereditary-hemorrhagic-telangiectasia-and-fibropolycystic-liver-disease-long-term-follow-up-with-magnetic-resonance-imaging-and-magnetic-resonance-angiography
#7
Sila Ulus, Aydan Arslan, Ercan Karaarslan, Yilmaz Cakaloglu
We report a case of a 62-year-old woman who received a liver transplant 19 years previously for end-stage liver disease due to hereditary hemorrhagic telangiectasia and fibropolycystic liver disease. During long-term follow-up 8 years after the liver transplant, de novo vascular lesions were detected with magnetic resonance imaging and magnetic resonance angiography. Hepatic vascular lesions had slowly progressed, despite no symptoms. To our knowledge, there are few reports in the English literature of de novo vascular lesions after liver transplant in patients with hepatic telangiectasias...
December 22, 2016: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/27997001/-a-rare-cardiomegaly
#8
Bruno De Piccoli, Marino Cibin, Marta Possamai, Giuseppe Favretto
We present the case of a patient who came to our attention for enlargement of the cardiac silhouette on chest X-ray. Echocardiography showed moderate diastolic overload of both ventricles with enhanced cardiac output without valvular disease or cardiac shunt that could account for this cardiomegaly. A subsequent abdominal echocardiographic exploration showed an angiomatous transformation of the liver due to diffuse lacunar enlargement of hepatic portal vein branches and arterial-venous shunts. Computed tomography and magnetic resonance imaging confirmed the echocardiographic findings...
November 2016: Giornale Italiano di Cardiologia
https://www.readbyqxmd.com/read/27895866/liver-findings-in-patients-with-hereditary-hemorrhagic-telangiectasia
#9
Wenyan Song, Dawei Zhao, Hongjun Li, Jinli Ding, Ning He, Yu Chen
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare autosomal dominant genetic vascular dysplasia. OBJECTIVES: The aim is to characterize the liver involvement of HHT by multi-detector helical computed tomography. PATIENTS AND METHODS: Through the use of multi-detector helical computed tomography, scan data derived from 12 cases of HHT were retrospectively studied, and an abdominal scan was performed on all of the patients...
October 2016: Iranian Journal of Radiology: a Quarterly Journal Published By the Iranian Radiological Society
https://www.readbyqxmd.com/read/27878613/intermittent-low-dose-bevacizumab-in-hereditary-hemorrhagic-telangiectasia-a%C3%A2-case-report
#10
Florian Huemer, Martin Dejaco, Christoph Grabmer, Thomas Melchardt, Daniel Neureiter, Georg Mayer, Alexander Egle, Richard Greil, Lukas Weiss
BACKGROUND: Hereditary hemorrhagic telangiectasia is an inherited autosomal dominant disease presenting with recurrent bleeding episodes and iron deficiency anemia due to vascular malformations. Hereditary hemorrhagic telangiectasia is associated with an increased risk of stroke, gastrointestinal bleeding and pulmonary hypertension and life expectancy is significantly reduced. Excess vascular endothelial growth factor (VEGF) plays a key role in the pathophysiology of the disease. CASE PRESENTATION: Here we report about a male patient with hereditary hemorrhagic telangiectasia presenting with pulmonary and central nervous system involvement experiencing repetitive nosebleeds, necessitating frequent local cauterization and transfusion of more than 100 units of packed red blood cells...
November 23, 2016: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/27874028/a-mouse-model-of-hereditary-hemorrhagic-telangiectasia-generated-by-transmammary-delivered-immunoblocking-of-bmp9-and-bmp10
#11
Santiago Ruiz, Haitian Zhao, Pallavi Chandakkar, Prodyot K Chatterjee, Julien Papoin, Lionel Blanc, Christine N Metz, Fabien Campagne, Philippe Marambaud
Hereditary hemorrhagic telangiectasia (HHT) is a potentially life-threatening genetic vascular disorder caused by loss-of-function mutations in the genes encoding activin receptor-like kinase 1 (ALK1), endoglin, Smad4, and bone morphogenetic protein 9 (BMP9). Injections of mouse neonates with BMP9/10 blocking antibodies lead to HHT-like vascular defects in the postnatal retinal angiogenesis model. Mothers and their newborns share the same immunity through the transfer of maternal antibodies during lactation...
November 22, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27864871/hereditary-haemorrhagic-telangiectasia-to-transplant-or-not-to-transplant
#12
Carlo Sabbà
The Association of hereditary hemorrhagic telangiectasia (HHT) and type I hereditary angioedema is a very rare condition in medicine. The case reported by Muller et al., describes the coexistence of the two diseases and emphasizes the need for listing HHT patients for liver transplantation in case indications occurs, such as the presence of HCC, abnormally increased cardiac output, and gastrointestinal bleeding as reported for this case. The case described by Mueller et al. is anecdotal for the usefulness of liver transplantation in HHT patients and shows that liver transplantation may be the best supportive care and, sometimes, the unique and final therapeutic option for these patients...
December 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27864870/hereditary-hemorrhagic-telangiectasia-to-transplant-or-not-to-transplant
#13
Sophie Dupuis-Girod, Elisabetta Buscarini
This report comments the case reported by Muller et al. which describes a combination of at least two different indications for orthotopic liver transplant (OLT) in a same patient: hepatocarcinoma and HHT complicated with severe liver involvement and high output cardiac failure. This case report highlighted that the clear time for OLT in HHT can be difficult to determine. In HHT, if intensive medical approach is not efficient, OLT, has to be considered. In the case of Muller et al., the patient was correctly listed for OLT for a single hepatocellular carcinoma, however, he did not receive a sufficient priority so as to avoid worsening of liver vascular malformations complications...
December 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27863666/nasal-powders-of-thalidomide-for-local-treatment-of-nose-bleeding-in-persons-affected-by-hereditary-hemorrhagic-telangiectasia
#14
G Colombo, F Bortolotti, V Chiapponi, F Buttini, F Sonvico, R Invernizzi, F Quaglia, C Danesino, F Pagella, P Russo, R Bettini, P Colombo, A Rossi
In this work nasal powder formulations of thalidomide were designed and studied to be used by persons affected by hereditary hemorrhagic telangiectasia as a complementary anti-epistaxis therapy, with the goal of sustaining the effect obtained with thalidomide oral treatment after its discontinuation for adverse effects. Three nasal powders were prepared using as carriers β-CD or its more hydrophilic derivatives such as hydropropyl-β-CD and sulphobutylether-β-CD and tested with respect to technological and biopharmaceutical features after emission with active and passive nasal powder devices...
November 30, 2016: International Journal of Pharmaceutics
https://www.readbyqxmd.com/read/27861387/open-arterial-reconstruction-of-multiple-hepatic-artery-aneurysms-in-a-patient-with-hereditary-hemorrhagic-telangiectasia-a-case-report
#15
Hirotsugu Ozawa, Takao Ohki, Yuji Kanaoka, Koji Maeda, Shin Hagiwara
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is characterized by mucocutaneous telangiectasia and visceral vascular malformations (VMs). Liver involvement with VMs may lead to high-output cardiac failure, portal hypertension, and biliary disease. There is no curative treatment for the disease, and liver transplantation is indicated for life-threatening complications. Herein, we report a case of multiple hepatic artery aneurysms (HAAs) in a patient with HHT in which open arterial reconstruction was performed...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27860447/fluorescein-guided-intraoperative-endoscopy-in-patients-with-hereditary-hemorrhagic-telangiectasia-first-impressions
#16
Fabio Pagella, Alessandro Pusateri, Dario Zaccari, Daniele Bongetta, Cesare Zoia, Giuseppe Spinozzi, Carla Olivieri, Elina Matti
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic disease that results in mucocutaneous telangiectasias and arteriovenous visceral malformations. Nasal telangiectasias lead to recurrent epistaxis, which affects up to 96% of patients. Different morphologic classifications and methods of visualization of nasal lesions have been described in the literature. We developed a new method of intraoperative endoscopy based on the intravenous administration of fluorescein...
November 11, 2016: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/27814895/effect-of-center-volume-on-outcomes-in-hospitalized-patients-with-hereditary-hemorrhagic-telangiectasia
#17
Vivek N Iyer, Waleed Brinjikji, Bibek S Pannu, Dinesh R Apala, Giuseppe Lanzino, Harry J Cloft, Sanjay Misra, Michael J Krowka, Christopher P Wood, Karen L Swanson
OBJECTIVE: To determine whether hospitalized patients with hereditary hemorrhagic telangiectasia (HHT) had better outcomes at high-volume treatment centers (HVCs). PATIENTS AND METHODS: The Nationwide Inpatient Sample (2000-2011) was used to identify HHT-related hospitalizations. Hospitals were classified based on quartiles of annual HHT discharge volume. The 75th percentile cutoff value (third quartile) was used to classify hospitals as low-volume centers (1-7 HHT discharges per year) or as HVCs (≥8 discharges per year...
December 2016: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/27807449/splenic-involvement-in-hereditary-hemorrhagic-telangiectasia
#18
Susumu Takamatsu, Kota Sato, Shunsuke Kato, Hiroto Nagano, Shunro Ohtsukasa, Yasuyuki Kawachi
A 33-year-old man who presented with prolonged epigastric pain was referred to our hospital. He had experienced recurrent epistaxis and had a family history of hereditary hemorrhagic telangiectasia. Computed tomography and magnetic resonance imaging revealed splenomegaly and a 9 cm hypervascular mass in his spleen. Computed tomography also showed a pulmonary arteriovenous malformation and heterogeneous enhancement of the liver parenchyma, suggesting the presence of arteriosystemic shunts and telangiectases...
2016: Case Reports in Medicine
https://www.readbyqxmd.com/read/27801754/intratumoral-vasculopathy-in-leiomyoma-treated-with-tranexamic-acid
#19
Satoru Kudose, Hannah R Krigman
Although intravascular thrombi and infarct-type necrosis have been reported in leiomyomas following with tranexamic acid therapy, intratumoral vasculopathy resembling acute atherosis has not been reported to date in patients without exposure to gonadotropin receptor agonist. We describe a case of intratumoral vasculopathy resembling acute atherosis in a leiomyoma in a 49-year-old woman, with hereditary hemorrhagic telangiectasia and menorrhagia, treated with tranexamic acid. The patient had no exposure to gonadotropin receptor agonists...
October 31, 2016: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/27796677/the-subjective-experience-of-patients-diagnosed-with-hereditary-hemorrhagic-telangiectasia-a-qualitative-study
#20
Laura Geerts, Carole Fantini-Hauwel, Elodie Brugallé, Odile Boute, Frédéric Frénois, Lydie Defrance, Sylvie Manouvrier-Hanu, Florence Petit, Pascal Antoine
The aim of the present study was to understand the context and psychological impact for patients diagnosed with hereditary hemorrhagic telangiectasia (HHT). Semi-structured interviews were conducted with 9 patients affected by HHT, and the transcripts were analyzed using interpretative phenomenological analysis. The results of this study allowed us to propose a new hypothesis to explain the delay in diagnosis: the trivialization of symptoms associated with HHT. Moreover, the results showed that a genetic diagnosis of HHT results in emotional shock, uncertainty about the future, and worry about one's children in parents who are confronted with the dilemma of facing the reality of the diagnosis or delaying dealing with the diagnosis until disease onset...
October 28, 2016: Journal of Genetic Counseling
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