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Dissection and vasculitis

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https://www.readbyqxmd.com/read/28367219/alpha-1-antitrypsin-deficiency-presenting-with-mpo-anca-associated-vasculitis-and-aortic-dissection
#1
Bram M Voorzaat, Jan van Schaik, Stijn L P Crobach, Catharina S P van Rijswijk, Joris I Rotmans
The combination of alpha-1 antitrypsin (AAT) deficiency, ANCA-vasculitis, and aortic aneurysm has been rarely described in literature. We report an eventually fatal case in a 70-year-old patient who initially presented with giant cell arteritis and ANCA associated glomerulonephritis. Several years later, he presented with aortic dissection due to large vessel vasculitis, raising the suspicion of AAT deficiency, as two first-line relatives had chronic obstructive pulmonary disease, while they never smoked. This diagnosis was confirmed by AAT electrophoresis and immunohistochemistry on a temporal artery biopsy...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28361331/anca-associated-vasculitis-pathogenesis-a-commentary
#2
REVIEW
Eric J Gapud, Philip Seo, Brendan Antiochos
PURPOSE OF REVIEW: The ANCA-associated vasculitides are a group of small vessel vasculitides characterized by autoantibodies recognizing the neutrophil cytoplasmic antigens PR3 and MPO. We examine the current clinical and molecular immunology understanding of ANCA-associated vasculitides and discuss the current needs in our understanding of the pathogenic mechanisms of these rare diseases. RECENT FINDINGS: The majority of efforts to understand the pathogenesis of these diseases have focused on dissecting neutrophil biology because the neutrophil is the primary expressor of ANCA autoantigens...
April 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28330435/idiopathic-carotidynia
#3
Aleksandra Policha, David Williams, Mark Adelman, Frank Veith, Neal S Cayne
Idiopathic carotidynia is a syndrome characterized by pain and tenderness over the carotid artery without an associated structural luminal abnormality. Controversy exists over whether this is a distinct disease entity or merely a symptom attributable to other causes of neck pain, such as carotid dissection or vasculitis. A 50-year-old woman presented with sudden-onset right neck pain. Imaging studies demonstrated transmural inflammation of the proximal internal carotid artery, without evidence of intraluminal pathology...
April 2017: Vascular and Endovascular Surgery
https://www.readbyqxmd.com/read/28317622/aortic-dissection-in-takayasu-arteritis
#4
Kun-Qi Yang, Yan-Kun Yang, Xu Meng, Ying Zhang, Hui-Min Zhang, Hai-Ying Wu, Ya-Xin Liu, Xiong-Jing Jiang, Jun Cai, Xian-Liang Zhou, Ru-Tai Hui, De-Yu Zheng, Li-Sheng Liu
BACKGROUND: Aortic dissection (AD) is a rare complication of Takayasu arteritis (TA). The clinical presentation and long-term management outcomes of AD in patients with TA have not been well described. MATERIALS AND METHODS: We conducted a retrospective study of patients with TA along with AD admitted to Fuwai Hospital between January 1985 and March 2016. Clinical data and follow-up data were collected and analyzed. RESULTS: Of the 1,154 patients with TA, we identified 10 patients (0...
April 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28286188/endovascular-repair-of-internal-mammary-artery-aneurysms-in-two-sisters-with-smad3-mutation
#5
Daiva Nevidomskyte, Sherene Shalhub, Gabriel S Aldea, Peter H Byers, Ulrike Schwarze, Mitzi L Murray, Benjamin Starnes
True aneurysms of the internal mammary artery are rare and have been described in association with vasculitis or connective tissue disorders. Herein we describe two cases of familial internal mammary artery aneurysms in two sisters with SMAD3 mutation. The older sister presented at the age of 54 with an incidental diagnosis of a multilobed right internal mammary artery aneurysm (IMA) and the younger sister presented several years earlier with a ruptured left IMA aneurysm at the age of 49. Both sisters had Debakey type I aortic dissections prior to the IMA aneurysm presentation...
March 7, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28188400/-juvenile-stroke-what-is-important
#6
M Fischer, B Eckert, J Röther
Stroke in young adults is a diagnostic and therapeutic challenge for all persons involved. Approximately 15% of ischemic strokes occur in young adults. Lack of awareness of the symptoms in emergency departments often results in delayed diagnosis and access to specific therapeutic options, such as revascularization. The causes are often heterogeneous and necessitate specific investigations. The etiology of juvenile stroke includes drug abuse, vasculitis and arteriopathies, such as reversible vasoconstriction syndrome and posterior reversible encephalopathy syndrome, although the prevalence of classical vascular risk factors is high...
February 10, 2017: Der Nervenarzt
https://www.readbyqxmd.com/read/28116728/isolation-and-culture-of-endothelial-cells-from-large-vessels
#7
Augusto C Montezano, Karla B Neves, Rheure A M Lopes, Francisco Rios
The endothelium, which is at the interface between circulating blood and the vascular wall, comprises a simple squamous layer of cells that lines the inner surface of all blood vessels. Endothelial cells are highly metabolically active and play an important role in many physiological functions, including control of vasomotor tone, blood cell trafficking, vascular permeability, and maintenance of vascular integrity (Mensah, Vascul Pharmacol 46(5):310-314, 2007; Yetik-Anacak and Catravas, Vascul Pharmacol 45(5):268-276, 2006)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28076021/radiologic-assessment-of-native-renal-vasculature-a-multimodality-review
#8
Sayf Al-Katib, Monisha Shetty, Syed Mohammad A Jafri, Syed Zafar H Jafri
A wide range of clinically important anatomic variants and pathologic conditions may affect the renal vasculature, and radiologists have a pivotal role in the diagnosis and management of these processes. Because many of these entities may not be suspected clinically, renal artery and vein assessment is an essential application of all imaging modalities. An understanding of the normal vascular anatomy is essential for recognizing clinically important anatomic variants. An understanding of the protocols used to optimize imaging modalities also is necessary...
January 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/27960184/vessel-and-vessel-wall-imaging
#9
REVIEW
Seung Chai Jung, Dong-Wha Kang, Tanya N Turan
Angiography is a useful, important, common imaging method, with digital subtraction angiography (DSA) remaining the gold standard for luminal imaging. Computed tomography angiography (CTA) is minimally invasive and quite accurate in the evaluation of stenosis. Magnetic resonance angiography (MRA) is a good screening tool with the least invasiveness. Angiography mostly represents intracranial artery disease as luminal stenosis, which is often not sufficient to evaluate intracranial vascular pathology. The modalities provide indirect information about vascular pathology because luminal change, such as stenosis, results from the changes of vessel walls...
2016: Frontiers of Neurology and Neuroscience
https://www.readbyqxmd.com/read/27766271/complete-resolution-of-wegener-s-granulomatosis-lung-granuloma-after-aortic-root-replacement
#10
Sarah-Jayne Edmondson, Saina Attaran, Ulrich P Rosendahl
Wegner's granulomatosis (WG) is an autoimmune systemic vasculitis that results in necrotizing granulomas. We report a WG patient with a lung granuloma and aortic root dilatation, who underwent aortic root replacement on cardiopulmonary bypass (CPB). Intraoperatively, the patient suffered an aortic dissection, which was repaired immediately under deep hypothermic circulatory arrest (DHCA). Follow-up imaging showed complete granuloma resolution, despite absence of immunosuppressive therapy. Immune stimulation following CPB is well described; here, the opposite was observed and DHCA effects are discussed...
February 2016: Aorta (Stamford, Conn.)
https://www.readbyqxmd.com/read/27713202/-unplanned-ascending-aortic-graft-replacement-at-valvuler-surgery-in-a-patient-with-systemic-lupus-erythematosus-report-of-a-case
#11
Koichi Muramatsu, Noriyasu Kawada, Toshiyuki Hoshina, Hirokuni Naganuma, Kazuhiro Hasimoto
A 65-year-old woman with severe mitral stenosis was admitted to our hospital. She had been previously diagnosed with systemic lupus erythematosus (SLE) and had been taking prednisolone (5 mg/day) for 19 years. As SLE patients with prolonged steroid use are known to be at risk of an aortic dissection and aneurysm, femoral artery was chosen for arterial perfusion to reduce the risk of a dissection of the ascending aorta. However, hemostasis was difficult at the insertion point of the catheter to infuse the antegrade cardioplegic solution...
October 2016: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/27672552/retroperitoneal-fibrosis-with-periaortitis-a-case-report-of-an-unusual-form-of-presentation-of-granulomatosis-with-polyangiitis
#12
Elizabeth Manuely González Revilla, Araceli Abad Fernandez, María Teresa Río Ramirez, Sara Calero Pardo, María Antonia Juretschke Moragues
Granulomatosis with polyangiitis (GPA) is the name that has been used in recent years for Wegener's granulomatosis. This condition is a systemic inflammatory disease characterised by necrotizing vasculitis that affects small and medium-sized blood vessels (capillaries, arterioles, venules and arteries). The granulomatous inflammation affects the respiratory system; it also commonly affects the kidney and can very rarely affect large vessels such as the aorta and the surrounding retroperitoneal tissue. Early diagnosis and treatment is of vital importance because of the high risk of dissection and of obstruction of retroperitoneal structures...
2016: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27658465/arterial-dissection-in-childhood-takayasu-arteritis-not-as-rare-as-thought
#13
Florence A Aeschlimann, Lars Grosse-Wortmann, Susanne M Benseler, Ronald M Laxer, Diane Hebert, Rae S M Yeung
BACKGROUND: Arterial vessel wall dissection is a rare, life-threatening and rarely described complication in childhood Takayasu Arteritis (cTA). Prevalence and risk factors for arterial dissection in cTA are unknown. We sought to study the prevalence and analyse risk factors for arterial dissection in cTA. FINDINGS: A single center retrospective review of all children with cTA was performed. Patients with arterial dissection at cTA diagnosis were reported in detail and compared to the remaining single center retrospective cohort of children without dissection...
September 22, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27637962/cerebrovascular-disease
#14
REVIEW
M L P Portegies, P J Koudstaal, M A Ikram
With 16.9 million people who suffered a first-ever stroke in 2010 worldwide, stroke is a very common vascular disease. Epidemiologic studies have played an essential role in assessing this burden and in detecting the risk factors for stroke. Primary prevention of these risk factors, primarily hypertension, smoking, diabetes, and atrial fibrillation, has reduced the incidence in high-income countries. However, stroke remains a major cause of death and disability, and therefore research should be continued. Subarachnoid hemorrhages are less prevalent than strokes but have an even higher risk of death...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27615054/hypertensive-emergency-presenting-with-an-isolated-celiac-artery-dissection-a-rare-case-study
#15
Natalie Swergold, Steven Kozusko, Carlos Rivera, Cindy Sturt
INTRODUCTION: To our knowledge the most recent article on celiac dissection was published in 2015 and reported 24 known cases of spontaneous isolated celiac trunk dissection [2]. While some of those cases reported hypertension as a risk factor, no other case presents as hypertensive emergency with an isolated celiac artery dissection. PRESENTATION OF CASE: A 43 year-old man with a past medical history of uncontrolled hypertension, for which he had reportedly been non-compliant with follow-up, presented with complaints of severe, sudden-onset epigastric pain which was non-radiating and constant for 1 hour prior to arrival...
2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27507861/nonatheroscleotic-isolated-middle-cerebral-artery-disease-may-be-early-manifestation-of-moyamoya-disease
#16
Yeon-Jung Kim, Joo Kyung Lee, Sung-Ho Ahn, Bum Joon Kim, Dong-Wha Kang, Jong S Kim, Sun U Kwon
BACKGROUND AND PURPOSE: Middle cerebral artery steno-occlusive disease (MCAD) is not an uncommon cause of ischemic stroke in young Asians. Aside from atherosclerosis, the pathogenesis of MCAD include various nonatherosclerotic vasculopathies, most of which are yet to be defined. This study investigated the pathogenesis of symptomatic isolated MCAD in young Asian patients using high-resolution magnetic resonance imaging (HR-MRI) and mutation analysis of RNF213. METHODS: Patients aged <60 years with stroke or transient ischemic attack caused by MCAD were prospectively enrolled...
September 2016: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/27498818/a-case-of-possible-paraneoplastic-neurological-syndrome-presenting-as-multiple-cranial-nerve-palsies-associated-with-gallbladder-cancer
#17
Misako Kaido, Yoshihito Yuasa, Tameyoshi Yamamoto, Satoru Munakata, Naohiro Tagawa, Keiko Tanaka
We report the case of a patient who had developed multiple cranial nerve palsies in the course of possible paraneoplastic neurological syndrome (PNS) associated with gallbladder cancer. Twelve days prior to visiting our hospital, a 69-year-old man began experiencing neurological symptoms, beginning with diplopia and progressing to ptosis of the left palpebra and subsequent complete closure of the eye within 8 days. Results of the initial medical examination indicated paresis of left oculomotor (III) and abducens (VI) nerves...
September 29, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/27428181/large-vessel-involvement-by-igg4-related-disease
#18
Cory A Perugino, Zachary S Wallace, Nandini Meyersohn, George Oliveira, James R Stone, John H Stone
OBJECTIVES: IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs and lead to tumefactive, tissue-destructive lesions. Reports have described inflammatory aortitis and periaortitis, the latter in the setting of retroperitoneal fibrosis (RPF), but have not distinguished adequately between these 2 manifestations. The frequency, radiologic features, and response of vascular complications to B cell depletion remain poorly defined...
July 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27393479/novel-percutaneous-coronary-intervention-techniques-for-revascularizing-chronically-occluded-giant-coronary-aneurysms-in-a-patient-with-kawasaki-disease
#19
Zachary L Steinberg, Thomas K Jones, William L Lombardi
Kawasaki disease is a self-limiting vasculitis presenting in childhood that can lead to aneurysms of the coronary arteries. Patients who develop giant coronary aneurysms have a high incidence of coronary stenoses and occlusions resulting in myocardial ischemia. The mainstay of treatment for these lesions is surgical bypass due to complex coronary anatomy and a high rate of chronic total occlusions precluding traditional percutaneous coronary intervention techniques. We report the first successful percutaneous revascularization of two chronically occluded giant coronary aneurysms using the Hybrid Interventional Strategy for approaching chronic total occlusions...
October 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27367985/18f-fluorodeoxyglucose-positron-emission-tomography-and-the-risk-of-subsequent-aortic-complications-in-giant-cell-arteritis-a-multicenter-cohort-of-130-patients
#20
MULTICENTER STUDY
Hubert de Boysson, Eric Liozon, Marc Lambert, Jean-Jacques Parienti, Nicolas Artigues, Loïk Geffray, Jonathan Boutemy, Yann Ollivier, Gwénola Maigné, Kim Ly, Damien Huglo, Eric Hachulla, Pierre-Yves Hatron, Achille Aouba, Alain Manrique, Boris Bienvenu
Previous studies reported a 2- to 17-fold higher risk of aortic complications (dilation or dissection) in patients with giant-cell arteritis (GCA). We aimed to determine whether or not GCA patients with large-vessel involvement demonstrated by positron emission tomography with F-fluorodeoxyglucose combined with computed tomography (FDG-PET/CT) have a higher risk of aortic complications. We conducted a retrospective multicenter study between 1995 and 2014. Patients were included if they fulfilled at least 3 American College of Rheumatology criteria for GCA, or 2 criteria associated with extratemporal biopsy-proven giant-cell vasculitis; they underwent at least 1 FDG-PET/CT scan at diagnosis or during follow-up; and the morphology of the aorta was assessed by medical imaging at diagnosis...
June 2016: Medicine (Baltimore)
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