Presentation
also known as Rienhoff (pronounced REENhoff) syndrome, is characterized by syndromic presentation of aortic aneurysms involving the thoracic and/or abdominal aorta, with risk of dissection and rupture. [ebi.ac.uk]
CD-68, a macrophage marker that is normally absent in aorta tissue, is weakly present in the adventitia of thoracic aorta (arrow, K) and strongly present in the media and adventitia of abdominal aorta (arrows, L). [pediatrics.aappublications.org]
Four of the 6 (67%) patients presented with sudden hematemesis. [interventions.onlinejacc.org]
Patients have increased risk of presenting life threatening aortic rupture. The documents contained in this web site are presented for information purposes only. [orpha.net]
Workup
Making a distinction between TAD/TAA and acute coronary syndrome (ACS) is especially important as the workup of ACS is significantly different. [wjes.biomedcentral.com]
Clinical Testing and Workup Microscopic (histologic) examination of affected arteries can reveal disruption of elastic fibers of affected arterial walls. [rarediseases.org]
Treatment
Search: name, specialism, conditions, treatments, tests or services Search: name, specialism, conditions, treatments, tests or services Search: name, specialism, conditions, treatments, tests or services Royal Brompton & Harefield NHS Foundation Trust [rbht.nhs.uk]
New therapeutic strategies such as endovascular treatment and surgical techniques have appeared to improve conventional surgery in advanced phases of the disease, but until recently, medical treatment has focused on the control of cardiovascular risk [books.google.com]
The size of the aneurysm, presence and severity of symptoms, and the risk of surgery help determine the treatment approach. REGULAR MONITORING is standard treatment for smaller aneurysms that do not require surgery. [vascular.org]
Research Brings New Treatment Options for Aortic Disease We continue to forge the way in finding new treatment options for aortic diseases. [umcvc.org]
Endovascular stenting remains an option for treatment of some type B dissections. [emedicine.medscape.com]
Prognosis
CT scanning may be used to confirm the diagnosis but is less helpful in complications and prognosis. [patient.info]
Mayo Clinic Proceedings, 84, 465-481. http://dx.doi.org/10.1016/S0025-6196(11)60566-1 Clouse, W.D., Hallett, J.W., Schaff, H.V., et al. (1998) Improved Prognosis of Thoracic Aortic Aneurysms. A Population-Based Study. JAMA, 280, 1926-1929. [file.scirp.org]
If the individual has managed to survive this window period, his prognosis is improved. About 66% of all dissections present in the acute phase. [en.wikipedia.org]
Parents should talk to their children’s physician and medical team about their specific case, associated symptoms and overall prognosis. [rarediseases.org]
Prognosis Depending on the nature and extent of the dissection, death can occur within a few hours of the start of a dissection. Approximately 75% of untreated people die within two weeks of the start of a dissection. [medical-dictionary.thefreedictionary.com]
Etiology
The University of Michigan is home to the International Registry of Aortic Dissection, and several research laboratories that are exploring the molecular and genetic etiologies of aortic diseases. [umcvc.org]
In young patients, an underlying etiology (i.e. Marfan’s) should be explored. E. Common Pitfalls and Side-Effects of Management. [clinicaladvisor.com]
The etiology may differ depending on the location of the aneurysm. [omicsonline.org]
Both twins had deep vein thrombosis of unclear etiology, possibly attributed entirely to immobility after surgery. [pediatrics.aappublications.org]
Both interventionalists and general physicians are generally unaware of AEF as an important but rather rare differential diagnosis of hematemesis or other symptoms among other more common etiologies in patients with prior TEVAR. [interventions.onlinejacc.org]
Epidemiology
Review of epidemiological studies on the occupational risk of tuberculosis in low-incidence areas. Respiration 2005;72:431-46. [ Crossref ] [ PubMed ] Teo DT, Lim TW. [jtd.amegroups.com]
Epidemiology The incidence is approximately 3-4 per 100,000 per year. [ 2 ] Aortic dissection is the most common emergency affecting the aorta. The male:female ratio varies - in one study, almost 70% of cases were in males. [patient.info]
EPIDEMIOLOGY, NATURAL HISTORY, AND TYPES OF AORTIC ANEURYSMS Abdominal aortic aneurysms (AAAs), involving the infradiaphragmatic abdominal area, are the most common type of aneurysms. [nap.edu]
Mészáros I, Mórocz J, Szlávi J, Schmidt J, Tornóci L, et al. (2000) Epidemiology and clinicopathology of aortic dissection. Chest 117: 1271-1278. [omicsonline.org]
Epidemiology [ edit ] Establishing the incidence of aortic dissection has been difficult because many cases are only diagnosed after death (which may have been attributed to another cause), and is often initially misdiagnosed. [en.wikipedia.org]
Pathophysiology
This book is driven by the considerable progress made by novel imaging techniques in understanding the pathophysiology and evolution of aortic disease and its diagnosis. [books.google.com]
The exact pathophysiologic mechanisms of AEF formation secondary to TEVAR are unknown, so far. We were not able to identify anatomic or procedural similarities among our patients that may have predisposed to AEF. [interventions.onlinejacc.org]
Familial thoracic aortic aneurysm/dissection with patent ductus arteriosus: genetic arguments for a particular pathophysiological entity. Eur J Hum Genet 2004;12:173-80. Loeys BL, Dietz HC, Braverman AC, et al. [annalscts.com]
Syphilis only potentially causes aortic dissection in its tertiary stage. [13] Pathophysiology [ edit ] Blood penetrates the intima and enters the media layer. [en.wikipedia.org]
Tadros TM, Klein MD, Shapira OM (2009) Ascending aortic dilatation associated with bicuspid aortic valve: pathophysiology, molecular biology, and clinical implications. [journals.plos.org]
Prevention
Medication Cholesterol-lowering medications (statins) and blood-pressure medications may help slow aneurysm growth and prevent complications. [dukehealth.org]
Prevention The chance of developing an ascending aortic aneurysm may depend on preventative lifestyle changes. [medicalnewstoday.com]
JRRP at UTHealth are working to prevent premature deaths due to thoracic aortic disease by improving diagnosis, treatment, and public awareness of this disease. [aortadissektion.com]
Smidt Heart Institute offers preventive, rehabilitative, diagnostic and both nonsurgical and surgical treatments for heart disease, including angioplasty, bypass surgery and heart transplantation. [cedars-sinai.edu]
It also emphasizes the role of imaging in diagnosis and follows up of ascending aortic aneurysm particularly where prevention is key to prevent the high mortality associated with Thoracic aneurysymal rupture. [file.scirp.org]