scholarly journals Histopathologic Features of Intracranial Vascular Involvement in Fibromuscular Dysplasia, Ehlers-Danlos Type IV, and Neurofibromatosis I

2014 ◽  
Vol 73 (10) ◽  
pp. 916-932 ◽  
Author(s):  
Seth Lummus ◽  
Robert Breeze ◽  
M. Scott Lucia ◽  
Bette Kay Kleinschmidt-DeMasters
Keyword(s):  
Fibromuscular Dysplasia ◽  
Vascular Involvement ◽  
Neurofibromatosis I ◽  
Type Iv ◽  
Histopathologic Features

Fibromuscular dysplasia – results of a multicentre study in Flanders

VASA ◽  
2017 ◽  
Vol 46 (3) ◽  
pp. 211-218 ◽  
Author(s):  
Marie De Groote ◽  
Patricia Van der Niepen ◽  
Dimitri Hemelsoet ◽  
Bert Callewaert ◽  
Frank Vermassen ◽  
...  
Keyword(s):  
Multicentre Study ◽  
Fibromuscular Dysplasia ◽  
Real Life ◽  
Clinical Manifestations ◽  
Vascular Involvement ◽  
Interventional Treatment ◽  
Cerebrovascular Event ◽  
Atherosclerotic Vascular Disease ◽  
Imaging Results ◽  
The Us

Abstract. Background: Fibromuscular dysplasia (FMD) is an idiopathic, non-inflammatory, non-atherosclerotic vascular disease, resulting in focal narrowing of small and medium-sized arteries. Systematic recording of clinical data in central databases as in the US and France provided new insights into FMD. The main objectives of this multicentre study were to explore the epidemiology, pattern of vascular involvement, clinical manifestations, and management of FMD patients in Flanders. Patients and methods: Multicentre, retrospective registry of patients diagnosed with FMD based on medical imaging. Results: Hundred-twenty-three FMD patients (83.7 % female) were included. Mean age at FMD diagnosis was 57.3 years (SD 15.8). More than half of patients (59.5 %) were hypertensive at the time of diagnosis. Neurological complaints such as headache (26.4 %) and dizziness (23.1 %) were also frequently reported. FMD was discovered incidentally in 10 patients (8.3 %). Nearly one quarter (22.8 %) of patients experienced a cerebrovascular event. Aneurysms were found in one-fifth (20.3 %) of patients and 11.4 % had an arterial dissection. FMD affected most frequently the renal (85.3 %), carotid (74.7 %), and vertebral (39.8 %) arteries. Renovascular FMD was more prevalent in men, whereas cerebrovascular FMD was more frequent in women. Multiple affected sites were documented in 25 of 61 (41.0 %) patients, having two or more vascular beds imaged. Digital subtraction angiography was most frequently used for detecting FMD. One third (32.9 %) of patients received an interventional treatment, mainly patients with renovascular FMD (32.8 % underwent percutaneous transluminal angioplasty) and patients with an intracranial carotid aneurysm (36.4 % were treated by means of coiling). Conclusions: Although differences existed, results of the Flemish registry were broadly in line with the US and French registries. Patient databases help to learn more about the natural history, progression, and management of FMD, based on real life clinical evidence.

Ocular ischemic manifestations in fibromuscular dysplasia

2021 ◽  
pp. 112067212110270
Author(s):  
June Artaechevarria-Artieda ◽  
Laura Cabrejas-Martinez ◽  
Claudia Azpitarte Sánchez-Muros ◽  
Marta Oses Lara ◽  
Elena Quiroga-Caneiro ◽  
...  
Keyword(s):  
Visual Loss ◽  
Fibromuscular Dysplasia ◽  
Young Patients ◽  
Vascular Involvement ◽  
Chronic Hypertension ◽  
Optic Coherence Tomography ◽  
Ophthalmological Examination ◽  
Acute Visual Loss ◽  
Sd Oct ◽  
Cerebral Vascular

Purpose: To report visual, clinical and radiological outcomes of three patients with fibromuscular dysplasia (FMD) that presented different ocular ischemic events, supported by different multimodal imaging. Methods: A retrospective study including patients who attended a tertiary referral hospital from July 2016 to June 2019 referring acute visual loss and later diagnosed of FMD. Ophthalmological examination included best corrected visual acuity (BCVA), biomicroscopy, fundoscopy, fluorescein angiography (FA), and macular and retinal nerve fiber layer spectral domain optic coherence tomography (SD-OCT). Patients were admitted by the Neurology service, completing the study with blood and cerebrospinal fluid (CSF) test, serology and cultures, cerebral magnetic resonance angiography (angioMRI), and arteriography. Results: Three patients were included; one male and second females. All referred unilateral acute visual loss, with BCVA of counting fingers or hand-motion. Relative afferent pupilar defect was present in all patients. Two patients associated neurological focal signs and one had chronic hypertension. Fundoscopy revealed hemorrhages and exudates in all patients, and the typical sign of “string-of-beads” in one. FA showed dye filling delay and macular and peripheral hypofluorescence. SD-OCT revealed acute and chronic retinal ischemic signs. Blood and CSF tests, serologies and cultures resulted negative. All presented cerebral vascular involvement, objectified in the cerebral angioMRI or arteriography. Conclusions: FMD should be considered in cases with unilateral sudden visual loss associated to neurological focal deficits in young patients. The typical “string-of-beads” image is rare in small arteries such as the retinal vasculature. When suspected, a complete ophthalmological examination and cerebral vascular imaging is essential to confirm the diagnosis.

Correlation between laminin and Type IV collagen distribution in Breast Carcinomas, and Estrogen receptors expression, Lymph Node and Vascular involvement

1990 ◽  
Vol 7 (1) ◽  
pp. 43-54 ◽  
Author(s):  
colette Charpin ◽  
lucile Andrac ◽  
marie christine Habib ◽  
Hélène Vacheret ◽  
Marie Noëlle Lavaut ◽  
...  
Keyword(s):  
Lymph Node ◽  
Estrogen Receptors ◽  
Type Iv Collagen ◽  
Vascular Involvement ◽  
Breast Carcinomas ◽  
Type Iv

[PP.24.06] CARDIAC PHENOTYPE BASED ON ANGIOGRAPHIC SUBTYPES AND VASCULAR INVOLVEMENT IN PATIENTS WITH FIBROMUSCULAR DYSPLASIA –ARCADIA-POL STUDY

2017 ◽  
Vol 35 ◽  
pp. e289-e290
Author(s):  
P. Dobrowolski ◽  
E. Warchol-celinska ◽  
A. Klisiewicz ◽  
K. Hanus ◽  
A. Prejbisz ◽  
...  
Keyword(s):  
Fibromuscular Dysplasia ◽  
Vascular Involvement ◽  
Cardiac Phenotype

RELATIONSHIP BETWEEN SMOKING, ANGIOGRAPHIC SUBTYPES AND VASCULAR INVOLVEMENT IN PATIENTS WITH FIBROMUSCULAR DYSPLASIA - THE ARCADIA-POL STUDY

2018 ◽  
Vol 36 (Supplement 1) ◽  
pp. e35-e36
Author(s):  
H. Witowicz ◽  
E. Warchol-Celinska ◽  
J. Ziebka ◽  
K. Paschalis-Purtak ◽  
B. Pucilowska ◽  
...  
Keyword(s):  
Fibromuscular Dysplasia ◽  
Vascular Involvement

scholarly journals Subclinical involvement of common carotid arteries in patients with fibromuscular dysplasia – a case-control study

VASA ◽  
2019 ◽  
Vol 48 (6) ◽  
pp. 509-515
Author(s):  
Matthew Lippmann ◽  
Nicholas Isom ◽  
Tyler Buechler ◽  
Tarun Dalia ◽  
Reza Masoomi ◽  
...  
Keyword(s):  
Abdominal Aorta ◽  
Carotid Arteries ◽  
Fibromuscular Dysplasia ◽  
Vascular Involvement ◽  
Cross Sectional ◽  
Iliac Arteries ◽  
Thoracic And Abdominal ◽  
And Control ◽  
Common Carotid Arteries ◽  
Age And Sex

Summary: Background: Fibromuscular dysplasia (FMD) primarily involves medium-sized arteries, though the entire spectrum of vascular involvement is not fully understood. We hypothesized that larger arteries may also be affected, albeit sub-clinically. Patients and methods: We measured the cross-sectional diameter of the thoracic aorta, abdominal aorta, common iliac arteries (CIA) and common carotid arteries (CCA) in FMD subjects and compared them to matched controls. We retrospectively analyzed records of FMD subjects (n = 74) and of age- and sex- matched controls (n = 74) that underwent computed tomography of the neck, chest or abdomen. Cross-sectional diameters of the thoracic and abdominal aorta, CIA and CCA were measured in a standardized manner by two trained physicians. Results: The FMD group had a significantly greater diameter of the CIA and CCA bilaterally. The measurements (mm) in FMD and control groups were as follows: Right CIA: 10.85 + 1.75 vs. 10.23 + 1.36, p = 0.04, left CIA: 11.01 + 1.93 vs. 10.15 + 1.38, p = 0.007, right CCA: 7.70 + 0.81 vs. 6.80 + 1.10, p < 0.001 and left CCA: 7.70 + 1.10 vs. 6.80 + 1.0, p < 0.001). There was no difference in the diameter between the two groups in the ascending aorta, descending and the abdominal aorta. After adjusting for baseline differences, common carotid arteries (but not common iliac) were significantly larger in FMD group compared with controls. Conclusions: There is sub-clinical involvement of the common carotid arteries in patients with FMD and this manifests as a greater diameter of these arteries compared to age and sex matched controls.

The LDE-Type Flare Occurrence (1969-1993)

1994 ◽  
Vol 144 ◽  
pp. 279-282
Author(s):  
A. Antalová
Keyword(s):  
Time Series ◽  
Fourier Analysis ◽  
Sunspot Cycle ◽  
List Type ◽  
Type Number ◽  
Solar Cycles ◽  
Type Iv ◽  
Long Duration ◽  
Cycle Maximum ◽  
Flare Index

AbstractThe occurrence of LDE-type flares in the last three cycles has been investigated. The Fourier analysis spectrum was calculated for the time series of the LDE-type flare occurrence during the 20-th, the 21-st and the rising part of the 22-nd cycle. LDE-type flares (Long Duration Events in SXR) are associated with the interplanetary protons (SEP and STIP as well), energized coronal archs and radio type IV emission. Generally, in all the cycles considered, LDE-type flares mainly originated during a 6-year interval of the respective cycle (2 years before and 4 years after the sunspot cycle maximum). The following significant periodicities were found:• in the 20-th cycle: 1.4, 2.1, 2.9, 4.0, 10.7 and 54.2 of month,• in the 21-st cycle: 1.2, 1.6, 2.8, 4.9, 7.8 and 44.5 of month,• in the 22-nd cycle, till March 1992: 1.4, 1.8, 2.4, 7.2, 8.7, 11.8 and 29.1 of month,• in all interval (1969-1992):a)the longer periodicities: 232.1, 121.1 (the dominant at 10.1 of year), 80.7, 61.9 and 25.6 of month,b)the shorter periodicities: 4.7, 5.0, 6.8, 7.9, 9.1, 15.8 and 20.4 of month.Fourier analysis of the LDE-type flare index (FI) yields significant peaks at 2.3 - 2.9 months and 4.2 - 4.9 months. These short periodicities correspond remarkably in the all three last solar cycles. The larger periodicities are different in respective cycles.

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