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Anderson-Fabry disease (FD) is a rare genetic, progressive, and multi-systemic condition, with X-linked inheritance. This is caused by pathogenic variants in the GLA gene, coding for the lysosomal enzyme called alpha-galactosidase A (aGLA), responsible for the cleavage of globotriaosylceramide (Gb3). The reduced or absent activity of aGLA causes the intracellular accumulation of Gb3, particularly in smooth and endothelial muscle cells, which causes cellular dysfunction. The main organs involved are the central nervous system, heart, and kidneys. However, being a ubiquitous enzyme, FD disease must be considered a systemic disease involving the peripheral nervous system, ocular and audio-vestibular systems. Also, the vascular district is damaged but the pathophysiology of vasculopathy in FD is not yet entirely understood. In literature, many vascular diagnostic tests were used to evaluate this specific involvement in FD, i.e., carotid intima media thickness (cIMT), arterial stiffness (AS), flow-mediated dilation (FMD) and atherosclerotic plaques; evaluation of vascular calcifications in FD patients is not presently available. In this review, we examined the current available literature on vascular aspects in FD. Moreover, we presented our global vascular evaluation, based on Radio Frequency Duplex Ultrasound (RF-DU), plaques, and vascular calcifi- cations, to apply to FD patients. INSET: 845. [ABSTRACT FROM AUTHOR]

OBJECTIVE: This is a retrospective study on Pharmacomechanical Catheter- Directed Thrombolysis (PCDT) in the treatment of acute iliofemoral Deep Vein Thrombosis (DVT). PATIENTS AND METHODS: From March 2013 to November 2016, 22 patients (26 limbs), median age 46.7 years with acute (<21 days) extensive iliofemoral DVT underwent Percutaneous Mechanical Thrombectomy (PMT) with Aspirex (Straub Medical, Wangs, Switzerland), followed by Catheter- Directed Thrombolysis (CDT). Subsequent endovascular stenting was performed for underlying obstruction. The follow-ups were conducted up to 1 year, in two Centers by experienced operators. Post-Thrombotic Syndrome (PTS) was evaluated by assessing the Villalta Scale (VS) and measuring orthostatic venous pressure. RESULTS: Post-operative iliofemoral vein patency was restored in almost all cases (95.5%). Standard urokinase dose was 80.000 IU per hour; mean infusion time was 32.5 hours. Stenting was performed in 15 cases (68%). Median follow-up was 19.9 months (6-48 months); 21/22 patients completed the 12 months follow-up. At 30 days follow-up symptoms disappeared in 21/22 cases (95.5%), with one case (4.5%) of DVT recurrence. At 1-year follow- up there were 3 cases (14.2%) of mild PTS; 18 patients (85.8%) were free from PTS. At 1-year follow-up venous pressure measurement showed normal values in 11 cases (52.4%), mild hypertension in 7 patients (33.3%), moderate hypertension (80-100 mmHg) in 2 cases (9.5%) and severe hypertension (110 mmHg) in one case (4.8%). Neither major nor minor complications were observed. CONCLUSIONS: PMT with Aspirex combined with CDT with urokinase seems to be a safe and effective treatment for acute iliofemoral DVT and it shows promising results in reducing the risk of PTS. Thus, we suggest a controlled trial with this treatment strategy. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To describe and evaluate feasibility and efficacy of a saphenous ablation technique performed in patients with varicose veins (VVs), great saphenous vein (GSV) incompetence, and proximal femoral valve incompetence: the Excluded Saphenous Vein Technique (ESVT). PATIENTS AND METHODS: Patients with primary great saphenous and proximal femoral valve incompetence underwent ESVT. This technique is composed of selective crossectomy, GSV ligation next to the thigh incompetent tributary vein, and saphenous vein sclerosing performed from the proximal zone. Demographic, clinical and instrumental data were collected. CEAP classification was used to describe VVs severity. The primary outcome was perioperative complications. Secondary outcomes were 30-days, 6-months and 1-years GSV occlusion rate, and VVs recurrence rate. RESULTS: During a ten months period, 104 patients were analyzed. Among these, 82 patients underwent ESVT (59 female, age 50 ± 21 years), eighty C2 and two C5, according to CEAP classification. The average length of GSV treated was 23 ± 9 cm. No intraoperative complications occurred. A 1-year follow-up analysis revealed no partial or complete saphenous recanalization, deep venous thrombosis, pulmonary embolism. No VVs recurrence was detected during the follow- up period among the entire population. CONCLUSIONS: ESVT seems to be a safe and effective treatment for primary saphenous reflux and proximal femoral valve incompetence. Further studies are needed to assess long-term results. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To quantify non-coronary vascular calcifications (VC) in asymptomatic patients at low-intermediate cardiovascular risk by a new color Doppler ultrasound (DUS)-based score (the carotid, aortic, lower limbs calcium score, CALCs), and to correlate this score with classical parameters associated with cardiovascular risk [carotid intima media thickness (IMT), and arterial stiffness (AS)]. PATIENTS AND METHODS: All consecutive asymptomatic patients who underwent a screening DUS of non-coronary circulation were evaluated and patients at low-intermediate cardiovascular risk were selected according to Framingham risk score (FRS). Among them, we enrolled 70 patients with US evidence of VC and 71 age, sex and FRS matched controls. The presence of VC was correlated with classical markers of cardiovascular risk, such as AS and intima-media thickness (IMT). AS, expressed as pulse wave velocity (PWV) and arterial distensibility, carotid IMT and CALCs were measured for both groups. AS and c-IMT were assessed by a new Radio-Frequency (RF) DUS-based method. CALCs was generated by our previously described B-mode DUSbased method according to number/size of VC in 11 non-coronary segments (range 0-33). RESULTS: Patients with VC presented higher AS and IMT values than controls (PWV 8.34±0.98 m/s vs. 6.74±0.68 m/s, p<0.0001; arterial distensibility 267±12 mm vs. 315±65 mm, p=0.001; IMT 687±132 mm vs. 572±91 mm, p<0.0001). Mean CALCs of patients with VC was 8.41±7.78. CALCs were significantly correlated with c-IMT (p<0.0001; r=0.3), PWV (p<0.0001; r=0.4) and arterial distensibility (p=0.002; r=-0.1). CONCLUSIONS: DUS-based CALCs is highly correlated with other validated markers of subclinical atherosclerosis, such as c-IMT and AS. Our results demonstrated the ability of CALCs to identify individual predictive factors beyond the traditional risk factors by quantifying an interesting and novel step of the atherogenic process. Future studies on larger series and with adequate follow up are necessary to confirm these results and to evaluate the role of this new marker in monitoring calcific atherosclerosis progression. [ABSTRACT FROM AUTHOR]

Circulating Endothelial Progenitor Cells (EPCs) were discovered by Asahara et al in 1997 and defined as bone marrow CD34+/KDR+ cells endowed with angiogenic potentialities in vitro and in vivo. The most likely assumption is that EPCs consist of several cell subpopulations with functions targeted at accomplishing the post-natal neovascularization process in a synergic and complementary fashion. Indeed, the subsequent identification of numerous and differentiated hematic populations, characterized by the capacity to develop an endothelial phenotype, has posed a number of questions as to the real identity of EPCs. This concept does not represent a sterile speculation but rather it suggests important implications for the future practice of stem cell therapy. The aim of this report was to explore through a critical analysis the two main experimental methodologies, in vitro culture and flow cytometry, applied to EPCs, followed by a brief revaluation of the endothelial progenitors employing a globally functional approach. [ABSTRACT FROM AUTHOR]

BACKGROUND: Carotid intimamedia thickness (c-IMT), arterial stiffness (AS) and vascular calcification (VC) are now considered important new markers of atherosclerosis and have been associated with increased prevalence of cardiovascular events. An accurate, reproducible and easy detection of these parameters could increase the prognostic value of the traditional cardiovascular risk factors in many subjects at low and intermediate risk. Today, c-IMT and AS can be measured by ultrasound, while cardiac computed tomography is the gold standard to quantify coronary VC, although concern about the reproducibility of the former and the safety of the latter have been raised. Nevertheless, a safe and reliable method to quantify non-coronary (i.e., peripheral) VC has not been detected yet. AIM: To review the most innovative and accurate ultrasound-based modalities of c-IMT and AS detection and to describe a novel UltraSound-Based Carotid, Aortic and Lower limbs Calci f icat ion Score (USB-CALCs, simply named CALC), allowing to quantify peripheral calcifications. Finally, to propose a system for cardiovascular risk reclassification derived from the global evaluation of "Quality IntimaMedia Thickness", "Quality Arterial Stiffness", and "CALC score" in addition to the Framingham score. [ABSTRACT FROM AUTHOR]