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https://www.readbyqxmd.com/read/29771035/-analysis-of-clinical-characteristics-of-141-patients-with-postviral-olfactory-dysfunction
#1
J Tian, Y X Wei, L Li, Z F Sun, B Q Wang
Objective: The aim of this study is to investigate the clinical features of the patients with postviral olfactory dysfunction (PVOD). Method: Clinical information of 141 consecutive outpatients with PVOD were collected and analyzed based on a questionnaire, including age, gender, months of onset, and symptoms. Sniffin'Sticks Test was used as a psychophysical olfactory test to evaluate olfactory function. Result: Retrospective analysis of 141 patients with PVOD, the male to female ratio of patients was 1.0:2...
May 20, 2017: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
https://www.readbyqxmd.com/read/29770201/recent-advances-in-managing-septal-defects-ventricular-septal-defects-and-atrioventricular-septal-defects
#2
REVIEW
P Syamasundar Rao, Andrea D Harris
This review discusses the management of ventricular septal defects (VSDs) and atrioventricular septal defects (AVSDs). There are several types of VSDs: perimembranous, supracristal, atrioventricular septal, and muscular. The indications for closure are moderate to large VSDs with enlarged left atrium and left ventricle or elevated pulmonary artery pressure (or both) and a pulmonary-to-systemic flow ratio greater than 2:1. Surgical closure is recommended for large perimembranous VSDs, supracristal VSDs, and VSDs with aortic valve prolapse...
2018: F1000Research
https://www.readbyqxmd.com/read/29743074/genetic-analyses-in-a-cohort-of-191-pulmonary-arterial-hypertension-patients
#3
Hang Yang, Qixian Zeng, Yanyun Ma, Bingyang Liu, Qianlong Chen, Wenke Li, Changming Xiong, Zhou Zhou
BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive and fatal disorder associated with high pulmonary artery pressure. Genetic testing enables early diagnosis and offers an opportunity for family screening. To identify genetic mutations and help make a precise diagnosis, we performed genetic testing in 191 probands with PAH and tried to analyze the genotype-phenotype correlation. METHODS: Initially, PAH samples (n = 119) were submitted to BMPR2 screening using Sanger sequencing...
May 9, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29548663/clinical-prediction-score-for-identifying-patients-with-pulmonary-veno-occlusive-disease-pulmonary-capillary-hemangiomatosis
#4
Aiko Ogawa, Yukari Takahashi, Hiromi Matsubara
BACKGROUND: Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension. Although diagnosis is based on pathological findings, an early diagnosis is crucial because of poor prognosis compared to other types of pulmonary hypertension. Furthermore, vasodilators may cause fatal pulmonary edema in patients with PVOD/PCH. This study aimed to identify specific characteristics for patients with PVOD/PCH to clinically diagnose PVOD/PCH...
March 13, 2018: Journal of Cardiology
https://www.readbyqxmd.com/read/29501213/-pulmonary-veno-occlusive-disease
#5
REVIEW
A Boucly, B Girerd, D Bourlier, S Nemlaghi, J Caliez, L Savale, X Jaïs, P Dorfmüller, G Simonneau, O Sitbon, M Humbert, D Montani
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterized by preferential remodelling of pulmonary venules and angioproliferation. PVOD term includes idiopathic, heritable (biallelic mutations of EIF2AK4 gene), drugs and toxins induced (alkylating agents, organic solvents) and connectivite-associated forms (especially systemic-sclerosis associated form). PVOD and pulmonary arterial hypertension (PAH) share a similar clinical presentation. Lung biopsy is contraindicated in PVOD due to high risk of life-threatening bleeding...
February 2018: Revue des Maladies Respiratoires
https://www.readbyqxmd.com/read/29343015/-clinical-and-imaging-features-of-pulmonary-veno-occlusive-disease-and-pulmonary-capillary-hemangioma
#6
Q H Zhao, W H Wu, S G Gong, R Jiang, J He, C J Luo, H L Qiu, J L Li, J M Liu, L Wang
Objective: To improve the diagnosis and treatment of the pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangioma (PCH). Methods: The clinical features, radiological findings, laboratory testing and treatment in 8 cases of PVOD/PCH which was diagnosed from 2013 to 2017 were described. Results: PVOD/PCH was rare. The clinical symptoms were easily confused with IPAH, but the decrease of hypoxemia, clubbing, D(L)CO were more obvious, and the imaging features of HRCT were helpful for PVOD/PCH diagnosis...
January 12, 2018: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/29246894/global-pulmonary-vascular-remodeling-in-pulmonary-hypertension-associated-with-heart-failure-and-preserved-or-reduced-ejection-fraction
#7
Ahmed U Fayyaz, William D Edwards, Joseph J Maleszewski, Ewa A Konik, Hilary M DuBrock, Barry A Borlaug, Robert P Frantz, Sarah M Jenkins, Margaret M Redfield
Background -We hypothesized that pulmonary venous hypertension in heart failure (HF) leads to predominate remodeling of pulmonary veins and that the severity of venous remodeling is associated with the severity of pulmonary hypertension (PH) in HF. Methods -Patients with HF (n=108; 53 preserved and 55 reduced ejection fraction) with PH (HF-PH; pulmonary artery systolic pressure (PASP) ≥ 40 mmHg) were compared to normal Controls (n=12) and patients with primary pulmonary veno-occlusive disease (PVOD; n=17)...
December 15, 2017: Circulation
https://www.readbyqxmd.com/read/29116138/vascular-endothelial-growth-factor-signaling-requires-glycine-to-promote-angiogenesis
#8
Dongqing Guo, Colin E Murdoch, Hao Xu, Hui Shi, Dayue Darrel Duan, Asif Ahmed, Yuchun Gu
Peripheral vascular occlusive disease (PVOD) is a common manifestation of atherosclerosis, and it has a high rate of morbidity. Therapeutic angiogenesis would re-establish blood perfusion and rescue ischemic tissue. Vascular endothelial growth factor (VEGF) induces angiogenesis and can potentially be used to treat ischemic diseases, yet in clinical trials VEGF has not fulfilled its full potential with side effects. Whether amino acids promote angiogenesis and the molecular mechanisms are largely unknown. Here we showed that (1) Glycine significantly promoted angiogenesis both in vitro and in vivo and effectively protected mitochondrial function...
November 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29108819/pulmonary-vascular-remodeling-patterns-and-expression-of-general-control-nonderepressible-2-gcn2-in-pulmonary-veno-occlusive-disease
#9
Esther J Nossent, Fabrice Antigny, David Montani, Harm Jan Bogaard, Maria Rosa Ghigna, Mélanie Lambert, Vincent Thomas de Montpréville, Barbara Girerd, Xavier Jaïs, Laurent Savale, Olaf Mercier, Elie Fadel, Florent Soubrier, Olivier Sitbon, Gérald Simonneau, Anton Vonk Noordegraaf, Marc Humbert, Frédéric Perros, Peter Dorfmüller
BACKGROUND: Heritable pulmonary veno-occlusive disease (PVOD) is linked to mutations in the eukaryotic initiation factor 2 alpha kinase 4 (EIF2AK4) gene, leading to a loss of general control nonderepressible 2 (GCN2). The role of GCN2 expression in pulmonary vascular remodeling remains obscure. We sought to identify specific histologic and biologic features in heritable PVOD. METHODS: Clinical data and lung histology of 24 PVOD patients (12 EIF2AK4 mutation carriers, 12 non-carriers) were submitted to systematic histologic analysis and semiautomated morphometry...
October 4, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29045954/-pulmonary-arterial-hypertension-as-leading-manifestation-of-methylmalonic-aciduria-clinical-characteristics-and-gene-testing-in-15-cases
#10
X Q Liu, H Yan, J X Qiu, C Y Zhang, J G Qi, X Zhang, H J Xiao, Y L Yang, Y H Chen, J B DU
OBJECTIVE: To deepen our understanding of Methylmalonic aciduria (MMA) associated pulmonary hypertension (PH) by analyzing the characteristics of clinical presentation, pulmonary high resolusion CT(HRCT), treatment response and gene mutation. METHODS: This study includes 15 cases of pediatric patients with MMA associated PH diagnosed and treated in Peking University First Hospital pediatric department between May 2012 and May 2016 with symptoms of PH as their leading presentation...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29029203/prognostic-significance-of-computed-tomography-criteria-for-pulmonary-veno-occlusive-disease-in-systemic-sclerosis-pulmonary-arterial-hypertension
#11
Michelle J Connolly, Sharif Abdullah, Deborah A Ridout, Benjamin E Schreiber, Jamanda A Haddock, J Gerry Coghlan
Objectives: SSc-pulmonary arterial hypertension (SSc-PAH) is associated with worse response to therapy and survival when compared with idiopathic PAH. It is suggested that the vasculopathy in SSc may involve postcapillary pulmonary venules resulting in pulmonary veno-occlusive disease (PVOD). This may underlie the lower gas transfer and worse outcome on therapy. We sought to test whether CT signs of PVOD (CTS-PVOD) were frequent in SSc-PAH and whether they were associated with pulmonary oedema on therapy and worse survival...
December 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28972005/phenotypic-characterization-of-eif2ak4-mutation-carriers-in-a-large-cohort-of-patients-diagnosed-clinically-with-pulmonary-arterial-hypertension
#12
MULTICENTER STUDY
Charaka Hadinnapola, Marta Bleda, Matthias Haimel, Nicholas Screaton, Andrew Swift, Peter Dorfmüller, Stephen D Preston, Mark Southwood, Jules Hernandez-Sanchez, Jennifer Martin, Carmen Treacy, Katherine Yates, Harm Bogaard, Colin Church, Gerry Coghlan, Robin Condliffe, Paul A Corris, Simon Gibbs, Barbara Girerd, Simon Holden, Marc Humbert, David G Kiely, Allan Lawrie, Rajiv Machado, Robert MacKenzie Ross, Shahin Moledina, David Montani, Michael Newnham, Andrew Peacock, Joanna Pepke-Zaba, Paula Rayner-Matthews, Olga Shamardina, Florent Soubrier, Laura Southgate, Jay Suntharalingam, Mark Toshner, Richard Trembath, Anton Vonk Noordegraaf, Martin R Wilkins, Stephen J Wort, John Wharton, Stefan Gräf, Nicholas W Morrell
BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare disease with an emerging genetic basis. Heterozygous mutations in the gene encoding the bone morphogenetic protein receptor type 2 ( BMPR2 ) are the commonest genetic cause of PAH, whereas biallelic mutations in the eukaryotic translation initiation factor 2 alpha kinase 4 gene ( EIF2AK4 ) are described in pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis. Here, we determine the frequency of these mutations and define the genotype-phenotype characteristics in a large cohort of patients diagnosed clinically with PAH...
November 21, 2017: Circulation
https://www.readbyqxmd.com/read/28947033/efficacy-and-safety-of-long-term-imatinib-therapy-for-patients-with-pulmonary-veno-occlusive-disease-and-pulmonary-capillary-hemangiomatosis
#13
Aiko Ogawa, Katsumasa Miyaji, Hiromi Matsubara
BACKGROUND: Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are categorized as Group 1' in the clinical classification of pulmonary hypertension. No medical therapy has been proven to be effective in patients with PVOD/PCH. Imatinib is a molecular targeted drug and was expected to be effective in patients with pulmonary arterial hypertension. We evaluated its efficacy and safety in patients with PVOD/PCH. METHODS: In the present observational study, 9 patients with PVOD/PCH received imatinib...
October 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28768975/sudden-death-in-a-patient-with-pulmonary-veno-occlusive-disease-pvod-and-severe-pulmonary-hypertension
#14
Minako Saito, Nobuharu Ohshima, Hirotoshi Matsui, Akira Hebisawa, Ken Ohta
A 58-year-old woman was referred to our hospital with a chief complaint of exertional dyspnea. Bronchoscopy failed to establish a diagnosis, and the patient subsequently died suddenly due to respiratory insufficiency because of advanced pulmonary hypertension (PH). The pathological diagnosis at autopsy was pulmonary veno-occlusive disease (PVOD). PVOD is difficult to diagnose antemortem and has a poor prognosis. Lung transplantation is the only curative treatment for PVOD.
2017: Internal Medicine
https://www.readbyqxmd.com/read/28754081/pulmonary-capillary-hemangiomatosis-and-hypertrophic-cardiomyopathy-in-a-persian-cat
#15
Tiffany L Jenkins, Ryan N Jennings
Pulmonary capillary hemangiomatosis (PCH) and pulmonary veno-occlusive disease (PVOD) are rare causes of primary pulmonary hypertension in humans, and, in 2016, were reported in dogs. A 1-y-old, neutered male Persian cat was presented for autopsy after sudden death several hours after grooming. Grossly, the lungs were mottled red-to-pink, contained rubbery-to-firm nodular foci, and there was moderate-to-marked left-sided cardiomegaly and left atrial dilation, consistent with hypertrophic cardiomyopathy. Microscopically, there was multifocal to regionally extensive capillary proliferation within pulmonary alveolar septa and around respiratory bronchioles, with nodular aggregates of densely arranged capillaries that replaced pulmonary alveolar spaces...
November 2017: Journal of Veterinary Diagnostic Investigation
https://www.readbyqxmd.com/read/28697925/variable-expressivity-of-a-founder-mutation-in-the-eif2ak4-gene-in-hereditary-pulmonary-veno-occlusive-disease-and-its-impact-on-survival
#16
Paula Navas Tejedor, Julián Palomino Doza, Jair Antonio Tenorio Castaño, Ana Belén Enguita Valls, José Julián Rodríguez Reguero, Amaya Martínez Meñaca, Ignacio Hernández González, Héctor Bueno Zamora, Pablo Daniel Lapunzina Badía, Pilar Escribano Subías
INTRODUCTION AND OBJECTIVES: Hereditary pulmonary veno-occlusive disease (PVOD) has been associated with biallelic mutations in EIF2AK4 with the recent discovery of a founder mutation in Iberian Romani patients with familial PVOD. The aims of this study were phenotypical characterization and survival analysis of Iberian Romani patients with familial PVOD carrying the founder p.Pro1115Leu mutation in EIF2AK4, according to their tolerance to pulmonary vasodilators (PVD). Familial genetic screening was conducted, as well as assessment of sociocultural determinants with a potential influence on disease course...
February 2018: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28668489/pulmonary-clinicopathological-correlation-after-allogeneic-hematopoietic-stem-cell-transplantation-an-autopsy-series
#17
Lee Gazourian, Laura Spring, Emily Meserve, David Hwang, Alejandro A Diaz, Samuel Y Ash, Vincent T Ho, Lynette M Sholl, George R Washko
Pulmonary complications are a significant cause of morbidity, mortality, and resource utilization after hematopoietic stem cell transplantation (HSCT). The objective of this study was to compare antemortem clinical suspicion of pulmonary complications and postmortem findings in a modern HSCT cohort. All patients who underwent allogeneic HSCT at our institution (n = 1854) between January 1, 2000 and June 30, 2010 were reviewed and patients who died of any cause greater than 1 year after HSCT and had an unrestricted autopsy available for analysis were included...
October 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28661905/genetics-of-pulmonary-hypertension-in-the-clinic
#18
Barbara Girerd, Edmund Lau, David Montani, Marc Humbert
PURPOSE OF REVIEW: Heritable pulmonary arterial hypertension (PAH) is an autosomal dominant disease with incomplete penetrance because of mutations in bone morphogenetic protein receptor-II (BMPR2), activin A receptor type II-like kinase 1, endoglin, caveolin-1, potassium channel subfamily K, member 3, and T-box gene 4 genes. Heritable pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PVOD/PCH) is an autosomal recessive disease because of biallelic mutations in the eukaryotic translation initiation factor 2 alpha kinase 4 gene...
September 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28353604/a-case-report-of-pvod-patient-combined-with-pulmonary-embolism-anticoagulation-or-not
#19
Xiaoling Yuan, Xianghe Hou, Weihong Guo, Haiming Jiang, Junmeng Zheng, Stuti Paudyal, Yanhua Lyu
RATIONALE: Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary arterial hypertension (PAH). Oral anticoagulation is confined to patients with idiopathic PAH (IPAH), but no oral anticoagulation has been recommended for PVOD, because occult pulmonary hemorrhage was a common finding in PVOD. PATIENT CONCERNS: We report a case of PVOD, who was misdiagnosed as IPAH for 5 years with worsening dyspnea and two episodes of pulmonary embolism (PE). DIAGNOSES: He was confirmed as PVOD combined with PE by biopsy of the explanted lung specimen...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28265532/corrigendum-to-a-simple-method-to-assess-in-vivo-proliferation-in-lung-vasculature-with-edu-the-case-of-mmc-induced-pvod-in-rat
#20
Fabrice Antigny, Benoît Ranchoux, Valérie Nadeau, Edmund Lau, Sébastien Bonnet, Frédéric Perros
[This corrects the article DOI: 10.1155/2015/326385.].
2017: Analytical Cellular Pathology (Amsterdam)
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