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Fibromuscular dysplasia(FMDA)

MedGen UID:
376945
Concept ID:
C1851111
Disease or Syndrome
Synonyms: Fibromuscular dysplasia of arteries; FMDA
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Sources: HPO, OMIM, Orphanet
Autosomal dominant inheritance refers to genetic conditions that occur when a mutation is present in one copy of a given gene (i.e., the person is heterozygous).
Autosomal dominant inheritance (HPO, OMIM, Orphanet)
 
OMIM®: 135580

Definition

Fibromuscular dysplasia (FMDA) is a nonatherosclerotic, noninflammatory arterial disease that most commonly involves the renal and carotid arteries. The prevalence of symptomatic renal artery FMDA is about 4 in 1,000 and the prevalence of cervicocranial FMDA is about half of that. Histologic classification includes 3 main subtypes, intimal, medial, and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string of beads' appearance that is related to medial FMDA, and tubular and focal types, which are not clearly related to specific histologic lesions (summary by Plouin et al., 2007) [from OMIM]

Clinical features

Arterial fibromuscular dysplasia
MedGen UID:
4700
Concept ID:
C0016052
Disease or Syndrome
An idiopathic, segmental, nonatheromatous disease of the musculature of arterial walls, leading to STENOSIS of small and medium-sized arteries. There is true proliferation of SMOOTH MUSCLE CELLS and fibrous tissue. Fibromuscular dysplasia lesions are smooth stenosis and occur most often in the renal and carotid arteries. They may also occur in other peripheral arteries of the extremity.
Renovascular hypertension
MedGen UID:
43786
Concept ID:
C0020545
Disease or Syndrome
Hypertension due to RENAL ARTERY OBSTRUCTION or compression.
Intermittent claudication
MedGen UID:
7115
Concept ID:
C0021775
Disease or Syndrome
A symptom complex characterized by pain and weakness in SKELETAL MUSCLE group associated with exercise, such as leg pain and weakness brought on by walking. Such muscle limpness disappears after a brief rest and is often relates to arterial STENOSIS; muscle ISCHEMIA; and accumulation of LACTATE.
Myocardial infarction
MedGen UID:
10150
Concept ID:
C0027051
Disease or Syndrome
Each year over a million people in the U.S. have a heart attack. About half of them die. Many people have permanent heart damage or die because they don't get help immediately. It's important to know the symptoms of a heart attack and call 9-1-1 if someone is having them. Those symptoms include. -Chest discomfort - pressure, squeezing, or pain . -Shortness of breath . -Discomfort in the upper body - arms, shoulder, neck, back . -Nausea, vomiting, dizziness, lightheadedness, sweating . These symptoms can sometimes be different in women. What exactly is a heart attack? Most heart attacks happen when a clot in the coronary artery blocks the supply of blood and oxygen to the heart. Often this leads to an irregular heartbeat - called an arrhythmia - that causes a severe decrease in the pumping function of the heart. A blockage that is not treated within a few hours causes the affected heart muscle to die. NIH: National Heart, Lung, and Blood Institute.
Stroke
MedGen UID:
52522
Concept ID:
C0038454
Disease or Syndrome
A stroke is a medical emergency. Strokes happen when blood flow to your brain stops. Within minutes, brain cells begin to die. There are two kinds of stroke. The more common kind, called ischemic stroke, is caused by a blood clot that blocks or plugs a blood vessel in the brain. The other kind, called hemorrhagic stroke, is caused by a blood vessel that breaks and bleeds into the brain. Mini-strokes or transient ischemic attacks (TIAs), occur when the blood supply to the brain is briefly interrupted. Symptoms of stroke are . -Sudden numbness or weakness of the face, arm or leg (especially on one side of the body). -Sudden confusion, trouble speaking or understanding speech. -Sudden trouble seeing in one or both eyes. -Sudden trouble walking, dizziness, loss of balance or coordination. -Sudden severe headache with no known cause. If you have any of these symptoms, you must get to a hospital quickly to begin treatment. Acute stroke therapies try to stop a stroke while it is happening by quickly dissolving the blood clot or by stopping the bleeding. Post-stroke rehabilitation helps individuals overcome disabilities that result from stroke damage. Drug therapy with blood thinners is the most common treatment for stroke. . NIH: National Institute of Neurological Disorders and Stroke.

Professional guidelines

PubMed

Meschia JF, Bushnell C, Boden-Albala B, Braun LT, Bravata DM, Chaturvedi S, Creager MA, Eckel RH, Elkind MS, Fornage M, Goldstein LB, Greenberg SM, Horvath SE, Iadecola C, Jauch EC, Moore WS, Wilson JA; American Heart Association Stroke Council.; Council on Cardiovascular and Stroke Nursing.; Council on Clinical Cardiology.; Council on Functional Genomics and Translational Biology.; Council on Hypertension.
Stroke 2014 Dec;45(12):3754-832. Epub 2014 Oct 28 doi: 10.1161/STR.0000000000000046. PMID: 25355838Free PMC Article

Recent clinical studies

Etiology

Cotroneo AR, Amoroso L, Giammarino A, Galasso D, Taglialatela F, Timpani C, Gabrielli D
Cardiovasc Intervent Radiol 2017 Apr;40(4):546-552. Epub 2016 Dec 29 doi: 10.1007/s00270-016-1536-6. PMID: 28035431
Yang YK, Zhang Y, Meng X, Yang KQ, Jiang XJ, Wu HY, Zhang HM, Song L, Wang LP, Gao LG, Zhou XL
Clin Res Cardiol 2016 Nov;105(11):930-937. Epub 2016 Jun 6 doi: 10.1007/s00392-016-1001-1. PMID: 27270759
Green R, Gu X, Kline-Rogers E, Froehlich J, Mace P, Gray B, Katzen B, Olin J, Gornik HL, Cahill AM, Meyers KE
Pediatr Nephrol 2016 Apr;31(4):641-50. Epub 2015 Nov 2 doi: 10.1007/s00467-015-3234-z. PMID: 26525198
Pasquini M, Trystram D, Nokam G, Gobin-Metteil MP, Oppenheim C, Touzé E
Rev Neurol (Paris) 2015 Sep;171(8-9):616-23. Epub 2015 Apr 7 doi: 10.1016/j.neurol.2015.02.011. PMID: 25857462
O'Connor SC, Gornik HL
J Am Heart Assoc 2014 Dec;3(6):e001259. doi: 10.1161/JAHA.114.001259. PMID: 25527625Free PMC Article

Diagnosis

Yang YK, Zhang Y, Meng X, Yang KQ, Jiang XJ, Wu HY, Zhang HM, Song L, Wang LP, Gao LG, Zhou XL
Clin Res Cardiol 2016 Nov;105(11):930-937. Epub 2016 Jun 6 doi: 10.1007/s00392-016-1001-1. PMID: 27270759
Vasuri F, Freyrie A, Pasquinelli G
Ultrastruct Pathol 2016;40(2):116-20. doi: 10.3109/01913123.2016.1156797. PMID: 27031177
Green R, Gu X, Kline-Rogers E, Froehlich J, Mace P, Gray B, Katzen B, Olin J, Gornik HL, Cahill AM, Meyers KE
Pediatr Nephrol 2016 Apr;31(4):641-50. Epub 2015 Nov 2 doi: 10.1007/s00467-015-3234-z. PMID: 26525198
Rostambeigi N, Goldfarb R, Hunter DW, Anderson JK
Vasc Endovascular Surg 2015 Oct;49(7):206-9. Epub 2015 Oct 13 doi: 10.1177/1538574415610006. PMID: 26462977
Pasquini M, Trystram D, Nokam G, Gobin-Metteil MP, Oppenheim C, Touzé E
Rev Neurol (Paris) 2015 Sep;171(8-9):616-23. Epub 2015 Apr 7 doi: 10.1016/j.neurol.2015.02.011. PMID: 25857462

Therapy

Yang YK, Zhang Y, Meng X, Yang KQ, Jiang XJ, Wu HY, Zhang HM, Song L, Wang LP, Gao LG, Zhou XL
Clin Res Cardiol 2016 Nov;105(11):930-937. Epub 2016 Jun 6 doi: 10.1007/s00392-016-1001-1. PMID: 27270759
Weinberg I, Gu X, Giri J, Kim SE, Bacharach MJ, Gray BH, Katzen BT, Matsumoto AH, Chi YW, Rogers KR, Froehlich J, Olin JW, Gornik HL, Jaff MR
Vasc Med 2015 Oct;20(5):447-53. Epub 2015 May 11 doi: 10.1177/1358863X15584982. PMID: 25964292
Lou X, Mitter SS, Blair JE, Benzuly K, Gambardella I, Malaisrie SC
Ann Thorac Surg 2015 Apr;99(4):1442-4. doi: 10.1016/j.athoracsur.2014.06.107. PMID: 25841833
Gavalas M, Meisner R, Labropoulos N, Gasparis A, Tassiopoulos A
Vasc Endovascular Surg 2014 Oct-Nov;48(7-8):445-51. Epub 2014 Sep 15 doi: 10.1177/1538574414551206. PMID: 25227972
Savard S, Azarine A, Jeunemaitre X, Azizi M, Plouin PF, Steichen O
Hypertension 2013 Jun;61(6):1227-32. Epub 2013 Apr 22 doi: 10.1161/HYPERTENSIONAHA.111.00838. PMID: 23608656

Prognosis

Cotroneo AR, Amoroso L, Giammarino A, Galasso D, Taglialatela F, Timpani C, Gabrielli D
Cardiovasc Intervent Radiol 2017 Apr;40(4):546-552. Epub 2016 Dec 29 doi: 10.1007/s00270-016-1536-6. PMID: 28035431
Green R, Gu X, Kline-Rogers E, Froehlich J, Mace P, Gray B, Katzen B, Olin J, Gornik HL, Cahill AM, Meyers KE
Pediatr Nephrol 2016 Apr;31(4):641-50. Epub 2015 Nov 2 doi: 10.1007/s00467-015-3234-z. PMID: 26525198
Rostambeigi N, Goldfarb R, Hunter DW, Anderson JK
Vasc Endovascular Surg 2015 Oct;49(7):206-9. Epub 2015 Oct 13 doi: 10.1177/1538574415610006. PMID: 26462977
Pasquini M, Trystram D, Nokam G, Gobin-Metteil MP, Oppenheim C, Touzé E
Rev Neurol (Paris) 2015 Sep;171(8-9):616-23. Epub 2015 Apr 7 doi: 10.1016/j.neurol.2015.02.011. PMID: 25857462
O'Connor SC, Gornik HL
J Am Heart Assoc 2014 Dec;3(6):e001259. doi: 10.1161/JAHA.114.001259. PMID: 25527625Free PMC Article

Clinical prediction guides

Iampreechakul P, Siriwimonmas S
Interv Neuroradiol 2016 Dec;22(6):717-727. Epub 2016 Aug 1 doi: 10.1177/1591019916659263. PMID: 27481913
Saw J, Bezerra H, Gornik HL, Machan L, Mancini GB
Circulation 2016 Apr 19;133(16):1548-59. Epub 2016 Mar 8 doi: 10.1161/CIRCULATIONAHA.115.020282. PMID: 26957531
Green R, Gu X, Kline-Rogers E, Froehlich J, Mace P, Gray B, Katzen B, Olin J, Gornik HL, Cahill AM, Meyers KE
Pediatr Nephrol 2016 Apr;31(4):641-50. Epub 2015 Nov 2 doi: 10.1007/s00467-015-3234-z. PMID: 26525198
Rostambeigi N, Goldfarb R, Hunter DW, Anderson JK
Vasc Endovascular Surg 2015 Oct;49(7):206-9. Epub 2015 Oct 13 doi: 10.1177/1538574415610006. PMID: 26462977
O'Connor SC, Gornik HL
J Am Heart Assoc 2014 Dec;3(6):e001259. doi: 10.1161/JAHA.114.001259. PMID: 25527625Free PMC Article

Recent systematic reviews

Ham SW, Kumar SR, Wang BR, Rowe VL, Weaver FA
Arch Surg 2010 Sep;145(9):832-9. doi: 10.1001/archsurg.2010.183. PMID: 20855752
Trinquart L, Mounier-Vehier C, Sapoval M, Gagnon N, Plouin PF
Hypertension 2010 Sep;56(3):525-32. Epub 2010 Jul 12 doi: 10.1161/HYPERTENSIONAHA.110.152918. PMID: 20625080
MacGinley R, Kumar SK, Mantha M, Mount P, Roberts M, Mangos G; CARI.
Nephrology (Carlton) 2010 Apr;15 Suppl 1:S210-7. doi: 10.1111/j.1440-1797.2009.01243.x. PMID: 20591034
Bloch MJ, Basile J
Minerva Med 2004 Oct;95(5):357-73. PMID: 15467512
Cloft HJ, Kallmes DF, Kallmes MH, Goldstein JH, Jensen ME, Dion JE
J Neurosurg 1998 Mar;88(3):436-40. doi: 10.3171/jns.1998.88.3.0436. PMID: 9488296

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