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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
#1
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/28480722/hepatopulmonary-syndrome-with-large-pulmonary-arteriovenous-malformations-ct-findings-with-emphasis-on-its-association-with-a-mosaic-pattern-of-the-lung-parenchyma
#2
Luis Gorospe Sarasúa, Andreína Olavarría-Delgado, Frank Eric Farfán-Leal, Josefa Pérez-Templado Ladrón de Guevara
We present a case of a 54-year-old patient with cirrhosis, progressive dyspnea, and platypnea. Thoracic computed tomography (CT) showed multiple pulmonary arteriovenous malformations (PAVM), confirming the diagnosis of hepatopulmonary syndrome (HPS). Besides precisely identifying the number and location of PAVM, CT also demonstrated a striking mosaic pattern of the lung parenchyma, characterized by the presence of alternating geographic areas of low attenuation (showing pulmonary vessels with a decreased diameter) with regions of relatively increased attenuation (showing pulmonary vessels with a normal diameter)...
May 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28430880/cerebral-abscess-associated-with-odontogenic-bacteremias-hypoxemia-and-iron-loading-in-immunocompetent-patients-with-right-to-left-shunting-through-pulmonary-arteriovenous-malformations
#3
E J Boother, S Brownlow, H C Tighe, K B Bamford, J E Jackson, C L Shovlin
Background: Cerebral abscess is a recognised complication of pulmonary arteriovenous malformations (PAVMs) that allow systemic venous blood to bypass the pulmonary capillary bed through anatomic right-to-left shunts. Broader implications and mechanisms remain poorly explored. Methods: Between June 2005 and December 2016, at a single institution, 445 consecutive adult patients with CT-scan confirmed PAVMs (including 403 (90.5%) with hereditary haemorrhagic telangiectasia) were recruited to a prospective series...
April 19, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28421188/pulmonary-arteriovenous-malformations-evidence-of-physician-under-education
#4
Claire L Shovlin, James R Gossage
PAVMs pose unique management challenges; publication patterns indicate their importance remains poorly recognised http://ow.ly/7iIT304WYl2.
April 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28420371/effect-of-pulmonary-arteriovenous-malformations-on-the-mechanical-properties-of-the-lungs
#5
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/28407366/screening-children-for-pulmonary-arteriovenous-malformations-evaluation-of-18-years-of-experience
#6
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/28375020/pulmonary-hypertension-prevalence-and-prognosis-in-a-cohort-of-patients-with-hereditary-hemorrhagic-telangiectasia-undergoing-embolization-of-pavms
#7
Mwelwa Chizinga, Anastasiia A Rudkovskaia, Katharine Henderson, Jeffrey Pollak, Guadalupe Garcia-Tsao, Lawrence H Young, Wassim H Fares
No abstract text is available yet for this article.
April 4, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28324193/hemodynamic-characterization-of-peripheral-arterio-venous-malformations
#8
Sabrina Frey, A Haine, R Kammer, H von Tengg-Kobligk, D Obrist, I Baumgartner
Peripheral arterio-venous malformations (pAVMs) are congenital vascular anomalies that require treatment, due to their severe clinical consequences. The complexity of lesions often leads to misdiagnosis and ill-planned treatments. To improve disease management, we developed a computational model to quantify the hemodynamic effects of key angioarchitectural features of pAVMs. Hemodynamic results were used to predict the transport of contrast agent (CA), which allowed us to compare our findings to digital subtraction angiography (DSA) recordings of patients...
June 2017: Annals of Biomedical Engineering
https://www.readbyqxmd.com/read/28267932/hemoglobin-is-a-vital-determinant-of-arterial-oxygen-content-in-hypoxemic-patients-with-pulmonary-arteriovenous-malformations
#9
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/28260780/pulmonary-arterio-venous-malformation-pavm-a-rare-case-report
#10
A H Mollah, M S Islam, N Ghafoor, S S Morshed, T K Kar, A L Kabir, N Tabassum
A 6-year-old boy from Comilla, was admitted in Dhaka Medical College Hospital with exertional dyspnea, central cyanosis, clubbing and was finally diagnosed as pulmonary Arterio-Venous Malformation (PAVM) by bubble contrast echocardiography, and pulmonary CT angiography. As PAVM is rare in children, it is often not thought of in differential diagnoses and the diagnosis remains in disguise. In this report, we described the clinical presentation of 6-year-old child with PAVM and also how to investigate the case to get the diagnosis...
January 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28188364/a-survey-of-pulmonary-arteriovenous-malformation-screening-management-and-follow-up-in-hereditary-hemorrhagic-telangiectasia-centers-of-excellence
#11
Jeffrey Forris Beecham Chick, Shilpa N Reddy, Reed E Pyeritz, Scott O Trerotola
PURPOSE: To determine current practice regarding screening, management, and follow-up of pulmonary arteriovenous malformations (PAVMs), particularly those with feeding arteries <3 mm in diameter, in those patients with hereditary hemorrhagic telangiectasia (HHT) at HHT Centers of Excellence. MATERIALS AND METHODS: A 32-question survey focusing on PAVM screening, management, and follow-up was sent to 147 participants at HHT Centers worldwide. RESULTS: Thirty-three responses were received (22%); 60% from interventional radiologists and 20% from pulmonologists; 67% from the USA, 21% European, and 6% Asian...
February 10, 2017: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/28165669/non-invasive-ct-screening-for-pulmonary-arteriovenous-malformations-in-children-with-confirmed-hereditary-hemorrhagic-telangiectasia-results-from-two-pediatric-centers
#12
Nurcan Soysal, Mélanie Eyries, Suzanne Verlhac, Virginie Escabasse, Natascha Remus, Aline Tamalet, Jean-Yves Rioux, Stéphanie Franchi-Abella, Manuela Vasile, Sarah Robert, Céline Delestrain, Isabelle Hau, Hubert Ducou-Le Pointe, Florent Soubrier, Marie-France Carette, Ralph Epaud
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disorder that is caused by mutations in mainly two genes, that is ENG, encoding endoglin (HHT1), or ACVRL1, encoding activin receptor-like kinase 1 (ALK-1/HHT2). HHT is characterized by recurrent epistaxis, mucocutaneous telangiectasia, and vascular visceral dysplasia responsible for visceral arteriovenous malformations (AVM). AIM: to report the experience of two university hospitals (Trousseau, Paris, and CHIC, Creteil) with screening children for HHT and pulmonary AVM (PAVM) using high resolution computed tomography (HRCT)...
May 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28132749/persistence-in-coil-embolized-pulmonary-arteriovenous-malformations-with-feeding-artery-diameters-of-3-mm-or-less-a-retrospective-single-center-observational-study
#13
Elliot J Stein, Jesse L Chittams, Mary Miller, Scott O Trerotola
PURPOSE: To assess persistence in coil-embolized pulmonary arteriovenous malformations (PAVMs) with feeding artery diameters ≤ 3 mm. MATERIALS AND METHODS: Fifty-eight patients (21 male, 37 female; mean age, 43 y; range, 13-71 y) with 141 simple-type PAVMs treated from 2004 to 2014 were analyzed retrospectively. Inclusion criteria were one or more PAVMs with feeding artery diameters ≤ 3 mm ("small PAVMs"), treatment with coil embolization, and presence of a follow-up chest computed tomographic (CT) angiogram at approximately 6 months...
March 2017: Journal of Vascular and Interventional Radiology: JVIR
https://www.readbyqxmd.com/read/28040200/follow-up-interval-for-small-untreated-pulmonary-arteriovenous-malformations-in-hereditary-haemorrhagic-telangiectasia
#14
D J Ryan, T M O'Connor, M M Murphy, A P Brady
AIM: To investigate the natural history of untreated small (<3 mm) and microscopic pulmonary arteriovenous malformations (PAVMs) in hereditary haemorrhagic telangiectasia (HHT) in order to discern the optimal frequency of follow-up thoracic computed tomography (CT). MATERIALS AND METHODS: A retrospective analysis was performed on the follow-up data for definite and suspected HHT patients with untreated PAVMs. RESULTS: For small PAVMs in definite HHT (n=13), PAVM enlargement was identified in one patient (1/13, 7...
March 2017: Clinical Radiology
https://www.readbyqxmd.com/read/27856403/does-the-type-and-size-of-amplatzer-vascular-plug-affect-the-occlusion-time-of-pulmonary-arteriovenous-malformations
#15
Ahmed Kamel Abdel Aal, Moustafa Omar Massoud, Dina Mahmoud Elantably
PURPOSE: Occlusion time (OT) is an important factor in the treatment of pulmonary arteriovenous malformations (PAVMs) since it can lead to serious complications. The purpose of our study is to calculate the OT of Amplatzer vascular plug (AVP, St Jude Medical), and correlate it to the type of the device used (AVP or AVP 2) and the percent of device oversizing. Technical success rates and complications were also recorded. METHODS: We retrospectively studied a total of 19 patients with 47 PAVMs who received percutaneous transcatheter embolization therapy using either AVP or AVP 2...
January 2017: Diagnostic and Interventional Radiology: Official Journal of the Turkish Society of Radiology
https://www.readbyqxmd.com/read/27835862/a-novel-mutation-in-nuclear-prelamin-a-recognition-factor-like-causes-diffuse-pulmonary-arteriovenous-malformations
#16
Hong-Zhou Liu, Chun-Xian Du, Jing Luo, Xue-Ping Qiu, Zu-Hua Li, Qi-Yong Lou, Zhan Yin, Fang Zheng
Two daughters in a Chinese consanguineous family were diagnosed as diffuse pulmonary arteriovenous malformations (PAVMs) and screened using whole exome sequencing (WES) and copy number variations (CNVs) chips. Though no mutation was found in the established causative genes of capillary malformation-AVMs (CM-AVMs) or PAVMs, Ser161Ile (hg19 NM_022493 c.482G>T) mutation in nuclear prelamin A recognition factor-like (NARFL) was identified. Ser161Ile mutation in NARFL conservation region was predicted to be deleterious and absent in 500 population controls and Exome Aggregation Consortium (ExAC) Database...
January 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/27812780/pulmonary-arteriovenous-malformations-embolized-using-a-micro-vascular-plug-system-technical-note-on-a-preliminary-experience
#17
Emanuele Boatta, Christine Jahn, Matthieu Canuet, Julien Garnon, Nitin Ramamurthy, Roberto Luigi Cazzato, Afshin Gangi
AIM: To report our preliminary experience using a Micro Vascular Plug (MVP) deployed through a 2.8Fr micro-catheter for the treatment of pulmonary arteriovenous malformations (PAVMs) in a cohort of patients affected by Hereditary Haemorrhagic Telangiectasia (HHT). MATERIALS AND METHODS: Four consecutive female patients (mean age 38.0 years; range 25-55 years) with PAVMs diagnosed on echocardiogram/bubble test and contrast-enhanced CT (CECT) underwent MVP embolization...
February 2017: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/27730189/embolisation-of-pavms-reported-to-improve-nosebleeds-by-a-subgroup-of-patients-with-hereditary-haemorrhagic-telangiectasia
#18
Claire L Shovlin, Trishan Patel, James E Jackson
Pulmonary AVM embolisation appears to improve nosebleed severity for nearly one in six people with HHT http://ow.ly/4mJqip.
April 2016: ERJ Open Research
https://www.readbyqxmd.com/read/27559714/endovascular-occlusion-of-pulmonary-arteriovenous-malformations-with-the-artventive-endoluminal-occlusion-system%C3%A2
#19
Fabio Corvino, Mattia Silvestre, Amedeo Cervo, Francesco Giurazza, Antonio Corvino, Franco Maglione
Pulmonary arteriovenous malformations (PAVMs) are vascular anomalies of the lung and carry the risk of cerebral thromboembolism, brain abscess, or pulmonary hemorrhage. We describe a 64-year-old male with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) who presented with a five-year history of progressive effort dyspnea and a PAVM in the right upper lobe successfully treated by transcatheter embolization of feeding arteries using a new occlusion device, the ArtVentive Endoluminal Occlusion System™...
September 2016: Diagnostic and Interventional Radiology: Official Journal of the Turkish Society of Radiology
https://www.readbyqxmd.com/read/27400664/thrombosis-of-pancreatic-arteriovenous-malformation-induced-by-diagnostic-angiography-case-report
#20
Jernej Vidmar, Mirko Omejc, Rok Dežman, Peter Popovič
BACKGROUND: We report on a case of pancreatic arteriovenous malformation (PAVM) that obliterated shortly after diagnostic angiography (DSA). PAVM is a rare anomaly that presents with upper abdominal pain, signs of acute pancreatitis and massive gastrointestinal bleeding. The management of PAVM is rather complex, with complete treatment usually accomplished only by a total extirpation of the affected organ or at least its involved portion. DSA prior to treatment decisions is helpful for characterizing symptomatic PAVM, since it can clearly depict the related vascular networks...
July 11, 2016: BMC Gastroenterology
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