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https://www.readbyqxmd.com/read/28768975/sudden-death-in-a-patient-with-pulmonary-veno-occlusive-disease-pvod-and-severe-pulmonary-hypertension
#1
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
#2
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...
July 1, 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
#3
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...
July 8, 2017: Revista Española de Cardiología
https://www.readbyqxmd.com/read/28668489/pulmonary-clinicopathological-correlation-after-allogeneic-hematopoietic-stem-cell-transplantation-an-autopsy-series
#4
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...
June 28, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28661905/genetics-of-pulmonary-hypertension-in-the-clinic
#5
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
#6
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
#7
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)
https://www.readbyqxmd.com/read/28087362/clinical-phenotypes-and-outcomes-of-heritable-and-sporadic-pulmonary-veno-occlusive-disease-a-population-based-study
#8
David Montani, Barbara Girerd, Xavier Jaïs, Marilyne Levy, David Amar, Laurent Savale, Peter Dorfmüller, Andrei Seferian, Edmund M Lau, Mélanie Eyries, Jérôme Le Pavec, Florence Parent, Damien Bonnet, Florent Soubrier, Elie Fadel, Olivier Sitbon, Gérald Simonneau, Marc Humbert
BACKGROUND: Bi-allelic mutations of the EIF2AK4 gene cause heritable pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis (PVOD/PCH). We aimed to assess the effect of EIF2AK4 mutations on the clinical phenotypes and outcomes of PVOD/PCH. METHODS: We did a population-based study using clinical, functional, and haemodynamic data from the registry of the French Pulmonary Hypertension Network. We reviewed the clinical data and outcomes from all patients referred to the French Referral Centre (Pulmonary Department, Hospital Kremlin-Bicêtre, University Paris-Sud) with either confirmed or highly probable PVOD/PCH with DNA available for mutation screening (excluding patients with other risk factors of pulmonary hypertension, such as chronic respiratory diseases)...
February 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27884767/eif2ak4-mutations-in-patients-diagnosed-with-pulmonary-arterial-hypertension
#9
D Hunter Best, Kelli L Sumner, Benjamin P Smith, Kristy Damjanovich-Colmenares, Ikue Nakayama, Lynette M Brown, Youna Ha, Eleri Paul, Ashley Morris, Mohamed A Jama, Mark W Dodson, Pinar Bayrak-Toydemir, C Gregory Elliott
BACKGROUND: Differentiating pulmonary venoocclusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) from idiopathic pulmonary arterial hypertension (IPAH) or heritable pulmonary arterial hypertension (HPAH) is important clinically. Mutations in eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) cause heritable PVOD and PCH, whereas mutations in other genes cause HPAH. The aim of this study was to describe the frequency of pathogenic EIF2AK4 mutations in patients diagnosed clinically with IPAH or HPAH...
April 2017: Chest
https://www.readbyqxmd.com/read/27872804/pulmonary-hypertension-secondary-to-pulmonary-veno-occlusive-disease-complicated-by-right-heart-failure-hypotension-and-acute-kidney-injury
#10
Nima Golzy, Stuti Fernandes, Justin Sharim, Rikin Tank, Henry D Tazelaar, Howard E Epstein, Victor Tapson, Antoine Hage
Pulmonary veno-occlusive disease (PVOD) is rare condition which can lead to severe pulmonary hypertension, right ventricular dysfunction, and cardiopulmonary failure. The diagnosis of PVOD can be challenging due to its nonspecific symptoms and its similarity to idiopathic pulmonary arterial hypertension and interstitial lung disease in terms of diagnostic findings. This case describes a 57 year old female patient who presented with a 5-month history of progressive dyspnea on exertion and nonproductive cough...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27684876/eif2ak4-mutation-in-pulmonary-veno-occlusive-disease-a-case-report-and-review-of-the-literature
#11
REVIEW
Li Liang, Guofeng Ma, Kai Chen, Yangxiang Liu, Xiaohong Wu, Kejing Ying, Ruifeng Zhang
BACKGROUND: Pulmonary veno-occlusive disease (PVOD) is a rare and devastating cause of pulmonary arterial hypertension with a non-specific clinical presentation and a relatively specific presentation in high-resolution thoracic CT scan images. Definitive diagnosis is made by histological examination in previous. According to the 2015 ESC/ERS Guidelines, detection of a mutation in the eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) without histological confirmation is recommended to validate the diagnosis of PVOD...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27672068/centrilobular-nodules-in-high-resolution-computed-tomography-of-the-lung-in-ipah-patients-preliminary-data-concerning-clinico-radiological-correlates
#12
Monika Szturmowicz, Aneta Kacprzak, Barbara Burakowska, Agnieszka Skoczylas, Iwona Bestry, Jan Kuś, Anna Fijałkowska, Adam Torbicki, Marcin Kurzyna
INTRODUCTION: Inhomogeneity of lung attenuation pattern is observed in high resolution chest computed tomography (HRCT) in some IPAH patients despite lack of interstitial lung disease. Such radiological changes are described either as ill-defined centrilobular nodules (CN) or as focal ground glass opacities (FGGO). There is no consensus in the literature, whether they indicate the distinct type of IPAH, or pulmonary venoocclusive disease (PVOD) with subtle radiological changes. Thus the aim of the present pilot study was to assess the frequency and clinical significance of inhomogenic lung attenuation pattern in IPAH...
2016: Pneumonologia i Alergologia Polska
https://www.readbyqxmd.com/read/27623416/small-sample-lung-biopsy-findings-in-patients-with-clinicoradiologic-suspicion-of-pulmonary-venoocclusive-disease-pulmonary-capillary-hemangiomatosis
#13
Shakti K Bal, Balamugesh Thangakunam, Aparna Irodi, Mayank Gupta, Devasahayam J Christopher
Pulmonary venoocclusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) form a sinister subgroup of pulmonary arterial hypertension where the predominant pathology lies in the pulmonary veins and capillaries, thus making the use of pulmonary vasodilators potentially dangerous in these patients. Radiologically, the presence of centrilobular nodules, interlobular septal thickening, and significant mediastinal adenopathy are useful in identifying this subgroup from patients with pulmonary arterial hypertension...
October 2016: Journal of Bronchology & Interventional Pulmonology
https://www.readbyqxmd.com/read/27587546/genetic-analyses-in-a-cohort-of-children-with-pulmonary-hypertension
#14
Marilyne Levy, Mélanie Eyries, Isabelle Szezepanski, Magalie Ladouceur, Sophie Nadaud, Damien Bonnet, Florent Soubrier
The prevalence of germline mutations in paediatric pulmonary hypertension (PH) is poorly documented. The objective of this study was to determine the mutation frequency in PH genes in a paediatric cohort and describe the clinical characteristics of mutation carriers.The study involved 66 index cases with PH: 35 children with idiopathic pulmonary arterial hypertension (IPAH); five children with familial PAH (FPAH); three children with pulmonary veno-occlusive disease (PVOD); and 23 children with PAH associated with congenital heart disease (APAH-CHD)...
October 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/27514598/pulmonary-capillary-hemangiomatosis-and-pulmonary-veno-occlusive-disease
#15
REVIEW
Neal F Chaisson, Mark W Dodson, Charles Gregory Elliott
This article provides an overview of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH), two disorders that challenge clinicians, radiologists, and pathologists because they often mimic pulmonary arterial hypertension (PAH). The article reviews the features that differentiate PVOD and PCH from PAH. The article also describes the overlap of PVOD and PCH, highlighted by recent reports of families diagnosed with PVOD or PCH caused by EIF2AK4 mutations. In addition, the article outlines current approaches to the diagnosis and treatment of PVOD and PCH...
2016: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/27428110/sendai-virus-induces-persistent-olfactory-dysfunction-in-a-murine-model-of-pvod-via-effects-on-apoptosis-cell-proliferation-and-response-to-odorants
#16
Jun Tian, Jayant M Pinto, Xiaolan Cui, Henghui Zhang, Li Li, Yulong Liu, Chan Wu, Yongxiang Wei
BACKGROUND: Viral infection is a common cause of olfactory dysfunction. The complexities of studying post-viral olfactory loss in humans have impaired further progress in understanding the underlying mechanism. Recently, evidence from clinical studies has implicated Parainfluenza virus 3 as a causal agent. An animal model of post viral olfactory disorders (PVOD) would allow better understanding of disease pathogenesis and represent a major advance in the field. OBJECTIVE: To develop a mouse model of PVOD by evaluating the effects of Sendai virus (SeV), the murine counterpart of Parainfluenza virus, on olfactory function and regenerative ability of the olfactory epithelium...
2016: PloS One
https://www.readbyqxmd.com/read/27118446/gender-difference-in-chinese-adults-with-post-viral-olfactory-disorder-a-hospital-based-study
#17
MULTICENTER STUDY
Jia Liu, Jayant M Pinto, Ling Yang, Li Li, Jingwu Sun, Xutao Miao, Kunyan Li, Guowei Chen, Yongxiang Wei
CONCLUSIONS: This hospital-based study found a gender differences in Chinese subjects with post-viral olfactory disorder (PVOD). Women appear to face higher susceptibility to PVOD, a finding that appears to be magnified with age and duration of olfactory loss. OBJECTIVES: PVOD is one of the most common reasons for acquired olfactory dysfunction, but there is little data in Chinese populations. We analyzed Chinese subjects with PVOD in order to determine demographic risk factors and clinical features in this major population group...
September 2016: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/27069695/reactive-pulmonary-capillary-hemangiomatosis-and-pulmonary-veno-occlusive-disease-in-a-patient-with-repaired-scimitar-syndrome
#18
Eva Güttinger, Bart Vrugt, Rudolf Speich, Silvia Ulrich, Fabienne Schwitz, Mattia Arrigo, Lars C Huber
Pulmonary capillary hemangiomatosis (PCH) is a rare histological substrate within the spectrum of pulmonary arterial hypertension that possibly represents an unusual manifestation of pulmonary veno-occlusive disease (PVOD). One of the histological hallmarks of PCH is the proliferation of pulmonary capillaries in the alveolar septa that infiltrate adjacent structures such as bronchioles, vessels, and visceral pleura. The hyperplastic process involving the smallest vessels of the pulmonary vascular bed might reflect uncontrolled angiogenesis, but whether this vascular proliferation is idiopathic or, conversely, a reactive process remains to be elucidated...
2016: Case Reports in Cardiology
https://www.readbyqxmd.com/read/27055781/-drug-induced-pulmonary-hypertension-a-current-review
#19
REVIEW
S Günther, J Behr, H Knoop
BACKGROUND: The current classification of pulmonary Hypertension (PH) consists of five clinical groups, and drug-induced pulmonary arterial hypertension (PAH) is classified under Group 1 as a distinct entity. Our present work encompasses the available data concerning the association between the intake of a wide range of drugs and development of PAH. METHODS: A selective literature search was performed in Pubmed to include published work between the years 1960 - 2015...
May 2016: Pneumologie
https://www.readbyqxmd.com/read/27034689/recovery-of-olfactory-function-in-postviral-olfactory-dysfunction-patients-after-acupuncture-treatment
#20
Qi Dai, Zhihui Pang, Hongmeng Yu
Introduction. The aims of this study were to assess the impact of traditional Chinese acupuncture (TCA) in postviral olfactory dysfunction (PVOD) patients who were refractory to standardized treatment and to compare the results with the impact observed in an observation group. Methods. Fifty patients who presented to the outpatient clinic with PVOD and were refractory to standardized treatment were included: 25 were treated with TCA and 25 patients were simply observed. A subjective olfactory test was performed using the University of Pennsylvania Smell Identification Test (UPSIT)...
2016: Evidence-based Complementary and Alternative Medicine: ECAM
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