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Fibromuscular dysplasia

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https://www.readbyqxmd.com/read/29650765/prospective-cardiovascular-genetics-evaluation-in-spontaneous-coronary-artery-dissection
#1
M Ihsan Kaadan, Carolyn MacDonald, Francesca Ponzini, Jessica Duran, Kelsey Newell, Linda Pitler, Angela Lin, Ido Weinberg, Malissa J Wood, Mark E Lindsay
BACKGROUND: Previous studies describing genetics evaluation in spontaneous coronary artery dissection (SCAD) have been retrospective in nature or presented as single case reports. As part of a dedicated clinical program, we evaluated patients in cardiovascular genetics clinic to determine the role of genetically triggered vascular disease and genetic testing in SCAD. METHODS AND RESULTS: Patient data were entered prospectively into the Massachusetts General Hospital SCAD registry database from July 2013 to September 2017...
April 2018: Circulation. Genomic and precision medicine
https://www.readbyqxmd.com/read/29628950/safety-assessment-of-endovascular-treatment-of-cerebral-aneurysms-in-patients-with-fibromuscular-dysplasia
#2
Matthew T Bender, Christopher Hurtado, Bowen Jiang, Jessica K Campos, Judy Huang, Rafael J Tamargo, Li-Mei Lin, Alexander L Coon, Geoffrey P Colby
Background: The prevalence of cerebral aneurysms is increased in fibromuscular dysplasia (FMD). The presence of FMD may serve as discouragement to elective endovascular aneurysm treatment. Outcomes of endovascular intervention for aneurysms through vessels affected by FMD have not been reported. Methods: A prospectively maintained database of patients undergoing intracranial embolization was reviewed for patients with FMD who underwent endovascular aneurysm treatment...
February 2018: Interventional Neurology
https://www.readbyqxmd.com/read/29568781/renal-artery-rupture-in-association-with-fibromuscular-dysplasia
#3
Tamer Akel, Suzanne Elsayegh
Fibromuscular dysplasia is a noninflammatory arteriopathy of unknown etiology that affects medium-sized arteries. Although patients affected with it are often asymptomatic, some might have recurrent catastrophic events that depend mainly on the arterial bed involved. The most worrisome vascular complications of the disease are aneurysmal rupture and arterial dissection. Herein, we report a case of a 49-year-old woman who presented with sudden-onset abdominal pain without any inciting factors. She was found to have active blood extravasation from a capsular branch of the renal artery that happened spontaneously...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29560003/a-rare-case-of-carotid-web-presenting-with-ischemic-stroke-in-a-young-woman-and-a-brief-review-of-the-literature
#4
Kyaw Kyaw, Htun Latt, Sammy San Myint Aung, Jay Babu, Rajesh Rangaswamy
Carotid web is a radiological description of a shelf-like intraluminal filling defect in the carotid bulb. It is histologically defined as atypical fibromuscular dysplasia (FMD), with abnormal fibrosis and smooth muscle cell hyperplasia in the tunica intima . The spur-like intraluminal protrusion can serve as a nidus for thrombus formation, which could cause systemic embolism and ischemic strokes. We report a case of a 20-year-old female patient presenting with acute ischemic stroke in the ipsilateral middle cerebral artery (MCA) territory...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29535868/factors-associated-with-outcomes-of-percutaneous-transluminal-renal-angioplasty-in-patients-with-renal-artery-stenosis-a-retrospective-analysis-of-50-consecutive-cases
#5
Tetsutaro Matayoshi, Kei Kamide, Ryoichi Tanaka, Tetsuya Fukuda, Takeshi Horio, Yoshio Iwashima, Fumiki Yoshihara, Satoko Nakamura, Hajime Nakahama, Yusuke Ohya, Yuhei Kawano
Background: The results of recent trials have brought some confusion to the treatment strategy for renal artery stenosis (RAS). To evaluate the applicability of percutaneous transluminal renal angioplasty (PTRA) for RAS, we extracted the factors that may affect the effectiveness of PTRA from cases experienced at a hypertension center. Methods and Results: We retrospectively assessed the blood pressure (BP) lowering effects and renoprotective effects in 50 consecutive patients that had hemodynamically significant RAS and had undergone PTRA and stenting during 2001-2005...
2018: International Journal of Hypertension
https://www.readbyqxmd.com/read/29534984/fibromuscular-dysplasia-contemporary-concepts-and-future-directions
#6
REVIEW
Nupoor Narula, Daniella Kadian-Dodov, Jeffrey W Olin
Fibromuscular dyplasia (FMD) is an under-recognized non-atherosclerotic, non-inflammatory arteriopathy that occurs most commonly in middle-aged women, but may affect individuals of all age groups. FMD may result in stenosis, aneurysm, dissection, occlusion, or arterial tortuosity. Recently published data demonstrated a genetic association of FMD with a variant in the phosphatase and actin regulator 1 gene (PHACTR1), substantiating that the pathogenesis of this condition has genetic contribution. The renal and extracranial carotid and vertebral arteries are most often involved, although any arterial bed may be affected...
March 10, 2018: Progress in Cardiovascular Diseases
https://www.readbyqxmd.com/read/29528871/echocardiographic-assessment-of-left-ventricular-morphology-and-function-in-patients-with-fibromuscular-dysplasia-the-arcadia-pol-study
#7
Piotr Dobrowolski, Magdalena Januszewicz, Anna Klisiewicz, Aleksander Prejbisz, Ewa Warchoł-Celińska, Ilona Michałowska, Elżbieta Florczak, Katarzyna Kożuch, Katarzyna Hanus, Anna Aniszczuk-Hybiak, Helena Witowicz, Adam Witkowski, Jacek Kądziela, Marek Kabat, Krzysztof Madej, Sławomir Nazarewski, Andrzej Tykarski, Łukasz Stryczyński, Małgorzata Szczerbo-Trojanowska, Łukasz Światłowski, Przemysław Kosiński, Krystyna Widecka, Andrzej Januszewicz, Piotr Hoffman
OBJECTIVE: To provide a comprehensive assessment of left ventricle (LV) structure, and function and to detect alterations in cardiac properties in relationship to presence, subtypes and extent of fibromuscular dysplasia (FMD). METHODS: We studied 144 patients with FMD. The control group consisted of 50 matched individuals. Office and ambulatory blood pressure levels were evaluated. Echocardiography was employed to assess: left ventricular mass index (LVMI), systolic function including speckle tracking echocardiography and diastolic function assessed by mitral flow and tissue Doppler imaging...
March 9, 2018: Journal of Hypertension
https://www.readbyqxmd.com/read/29525759/spontaneous-renal-infarct-heralding-bowel-ischaemia-in-an-adult-male-lessons-to-learn-from-a-rare-clinical-association
#8
Siddharth Pandey, Ajay Aggarwal, Manoj Kumar, Satyanarayan Sankhwar
Spontaneous renal artery dissection (SRAD) is a rare entity with less than 200 cases reported in literature. It usually affects middle-aged men and the clinical presentation is non-specific. Many times it is associated with conditions such as hypertension, fibromuscular dysplasia or vasculitides. We report the case of a patient who initially had renal infarct due to SRAD and then progressed to have bowel ischaemia. The renal infarct preceded bowel ischaemia in this patient and to the best of our knowledge this is the first such association reported in literature...
March 9, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29483231/management-of-a-pregnant-woman-with-fibromuscular-dysplasia
#9
Elena Berra, Anna F Dominiczak, Rhian M Touyz, Sophie Pierard, Frank Hammer, Gian Paolo Rossi, Ruben Gabriel Micali, Jan A Staessen, Michael Bursztyn, Thomas Kahan, Alexandre Persu
No abstract text is available yet for this article.
February 26, 2018: Hypertension
https://www.readbyqxmd.com/read/29472380/spontaneous-coronary-artery-dissection-current-state-of-the-science-a-scientific-statement-from-the-american-heart-association
#10
REVIEW
Sharonne N Hayes, Esther S H Kim, Jacqueline Saw, David Adlam, Cynthia Arslanian-Engoren, Katherine E Economy, Santhi K Ganesh, Rajiv Gulati, Mark E Lindsay, Jennifer H Mieres, Sahar Naderi, Svati Shah, David E Thaler, Marysia S Tweet, Malissa J Wood
Spontaneous coronary artery dissection (SCAD) has emerged as an important cause of acute coronary syndrome, myocardial infarction, and sudden death, particularly among young women and individuals with few conventional atherosclerotic risk factors. Patient-initiated research has spurred increased awareness of SCAD, and improved diagnostic capabilities and findings from large case series have led to changes in approaches to initial and long-term management and increasing evidence that SCAD not only is more common than previously believed but also must be evaluated and treated differently from atherosclerotic myocardial infarction...
February 22, 2018: Circulation
https://www.readbyqxmd.com/read/29459079/anti-hypertensive-treatment-in-peripheral-artery-disease
#11
REVIEW
Costas Tsioufis, Ioannis Andrikou, Gerasimos Siasos, Konstantinos Filis, Dimitrios Tousoulis
Peripheral artery disease (PAD) affects more than 200 million people worldwide. Hypertension has been related to increased risk of PAD. The treatment of elevated blood pressure (BP) in these patients is indicated to lower the cardiovascular risk with a BP goal of less than 130/80mmHg. Although there is no evidence that one class of antihypertensive medication or strategy is superior for BP lowering in PAD, the use of renin-angiotensin-system (RAS) inhibitors can be effective to reduce the cardiovascular risk...
February 16, 2018: Current Opinion in Pharmacology
https://www.readbyqxmd.com/read/29448245/a-genetic-variant-associated-with-the-five-vascular-diseases-is-a-distal-regulator-of-endothelin-1-gene-expression
#12
Ernesto L Schiffrin
Gupta et al. [Cell 2017; 170: 522-533] have shown that a genetic variant associated with the 5 vascular diseases is a distal regulator of endothelin (ET)-1 gene (EDN1) expression. A common single nucleotide polymorphism (SNP) in the third intron of the PHACTR1 gene, rs9349379, is the potential variant responsible for increased risk of coronary artery disease, and lower risk of migraine, carotid dissection, fibromuscular dysplasia, and hypertension. Since ET-1 acts through ET type B receptors (ETBR) on endothelial cells to stimulate the production of nitric oxide and prostacyclin and induce vasodilation, this may contribute to these findings...
February 15, 2018: Nephron
https://www.readbyqxmd.com/read/29421412/right-subclavian-artery-aneurysms-with-fibromuscular-dysplasia
#13
Daisuke Kaneyuki, Hideki Ueda, Goro Matsumiya
Subclavian artery aneurysms are rare and even more uncommon in patients with fibromuscular dysplasia. A 20-year-old man with a past medical history of moyamoya disease, presented with an abnormal mass shadow in the apex of the right lung on chest x-ray. Based on computed tomography imaging, a diagnosis of right subclavian artery aneurysm was established and an excision of the aneurysm was performed. Since the left vertebral artery is dominant, the proximal and distal right subclavian artery and right vertebral artery were clamped...
February 5, 2018: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/29398271/tread-carefully-a-functional-variant-in-the-human-nadph-oxidase-4-nox4-is-not-disease-causing
#14
Michael Nafisinia, Minal Juliet Menezes, Wendy Anne Gold, Lisa Riley, Joshua Hatch, John Cardinal, David Coman, John Christodoulou
In this study, we report a paediatric patient with a lethal phenotype of respiratory distress, failure to thrive, pancreatic insufficiency, liver dysfunction, hypertrophic cardiomyopathy, bone marrow suppression, humoral and cellular immune deficiency. To identify the genetic basis of this unusual clinical phenotype and potentially make available the option of future prenatal testing, whole exome sequencing (WES) was used followed by functional studies in a bid to confirm pathogenicity. The WES we identified a homozygous novel variant, AK298328; c...
February 2, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29390277/a-rare-case-report-of-bilateral-common-and-internal-iliac-arterial-fibromuscular-dysplasia-coexisted-dissection-aneurysm-and-stenosis
#15
Luyun Fan, Wenjun Ma, Huimin Zhang, Jun Cai
RATIONALE: Iliac arterial fibromuscular dysplasia (FMD) was rarely reported and its demographic, clinical, and imaging features have not been precisely described resulting in uncertain therapeutic methods. PATIENT CONCERNS: A 31-year-old man was referred because of 3-month-ago onset hypertension, low serum potassium, and a small-sized right kidney with normal renal artery under ultrasound examination. This patient was suspected of primary aldosteronism, whereas spirolactone was poorly effective...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29359368/b-flow-and-b-mode-ultrasound-imaging-in-carotid-fibromuscular-dysplasia
#16
Joshua J Cutler, Nelly Campo, Sebastian Koch
BACKGROUND AND PURPOSE: Previous ultrasound studies in fibromuscular dysplasia (FMD) have largely reported on color flow imaging, power Doppler, and Doppler flow augmentation. We here report on arterial wall imaging findings by B-flow and B-mode in patients with carotid FMD. METHODS: We retrospectively reviewed ultrasonographic findings in subjects with known or suspected FMD. All patients were evaluated with a standardized imaging protocol including Doppler, B-mode, and B-flow...
January 23, 2018: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
https://www.readbyqxmd.com/read/29331613/internal-carotid-artery-web-as-the-cause-of-recurrent-cryptogenic-ischemic-stroke
#17
Jon Antigüedad-Muñoz, Patricia de la Riva, Gorka Arenaza Choperena, Amaia Muñoz Lopetegi, Naiara Andrés Marín, Gorka Fernández-Eulate, Manuel Moreno Valladares, Maite Martínez Zabaleta
Carotid artery web is considered an exceptional cause of recurrent ischemic strokes in the affected arterial territory. The underlying pathology proposed for this entity is an atypical fibromuscular dysplasia. We present the case of a 43-year-old woman with no cardiovascular risk factors who had experienced 2 cryptogenic ischemic strokes in the same arterial territory within an 11-month period. Although all diagnostic tests initially yielded normal results, detailed analysis of the computed tomography angiography images revealed a carotid web; catheter angiography subsequently confirmed the diagnosis...
May 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29317976/postpartum-sudden-cardiac-death-after-spontaneous-coronary-artery-dissection-in-a-patient-with-fibromuscular-dysplasia
#18
Tannaz Shoja, Craig Basman, Suresh Jain, Aditya Mangla, Zoran Lasic
Spontaneous coronary artery dissection (SCAD) is a rare entity that can cause acute myocardial infarction and sudden cardiac death (SCD) which often goes unrecognized. We report a case of SCAD in a young postpartum female who presented with sudden cardiac arrest. The patient was managed medically and found to have fibromuscular dysplasia (FMD). After being stabilized in the critical care unit, coronary angiography was performed which showed dissection of the left main artery, intramural hematoma, and the culprit lesion...
December 2017: Cardiology Research
https://www.readbyqxmd.com/read/29275928/spontaneous-coronary-artery-dissection-scad-the-underdiagnosed-cardiac-condition-that-plagues-women
#19
REVIEW
Sandrine Lebrun, Rachel M Bond
Coronary heart disease (CHD) continues to be understudied, underdiagnosed, and undertreated in women. Gender and age bias complicate the evaluation of women with acute coronary syndrome (ACS). As a result, conditions like spontaneous coronary artery dissection (SCAD) are often missed. SCAD is an infrequent yet important cause of myocardial infarction (MI) with a predilection for young to middle aged women. The condition is thought to be under-reported, likely a result of both low index of suspicion as well as an unfamiliarity with SCAD's angiographic variants...
December 11, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29274451/treatment-of-progressive-herpes-zoster-induced-vasculopathy-with-surgical-revascularization-effects-on-cerebral-hemodynamics
#20
Sarah K Lants, Jennifer M Watchmaker, Meher R Juttukonda, Larry T Davis, Manus J Donahue, Matthew R Fusco
BACKGROUND: Herpes zoster ophthalmicus (HZO) is caused by reactivation of the herpes simplex virus in the trigeminal nerve. HZO-initiated cerebral vasculopathy is well characterized; however, there are no documented cases that report the efficacy of surgical revascularization for improving cerebral hemodynamics following progressive HZO-induced vasculopathy. We present a case in which quantitative anatomic and hemodynamic imaging were performed longitudinally before and after surgical revascularization in a patient with HZO and vasculopathic changes...
March 2018: World Neurosurgery
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