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BACKGROUND: Autoimmune responses have been suggested to involvement in patients with Behcet's syndrome (BS). There has been growing attention towards the roles of cutaneous lymphocyte antigen (CLA)+ regular T cells (Tregs) in autoimmune diseases. The role of CLA+ Tregs in BS is still uncertain. This study aims to clarify the impact of CLA+ Tregs on BS. METHODS: We collected peripheral blood from a total of 107 patients with BS and 114 healthy controls (HCs). The number of CLA+ Tregs, natural killer (NK) cells, B cells, and several subtypes of CD4+ T cells were detected using flow cytometry and compared between patients and HCs. RESULTS: The absolute number and proportion of CLA+ Tregs among CD4+ T lymphocytes and CD4+ Tregs were lower in patients with BS than in HCs. CLA+ Tregs were positively related with NK cells (r = 0.500, P < 0.001) and B cells (r = 0.470, P < 0.001) and negatively related with effector T cells (r=-0.402, P < 0.001) in patients with BS. Patients with BS and arterial aneurysms had CLA+ Treg cell deficiency. A decreased proportion of CLA+ Tregs was associated with arterial aneurysms in patients with BS. The proportion of CLA+ Tregs in patients with BS increased with corticosteroids and immunosuppressants. CONCLUSION: CLA+ Tregs decrease in association with arterial aneurysm in patients with BS. CLA+ Tregs may be a predictor of response to BS treatment.

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Atherosclerosis and systemic hypertension are the most common pathogeneses of solitary acquired arterial aneurysms. The rare occurrence of multiple synchronous or metachronous arterial aneurysms requires considering alternative underlying causes. We present the unusual case of a male patient who sequentially developed multiple co-existing arterial aneurysms between the ages of 51 and 59. The sites of involvement included high-pressure systemic arteries and low-pressure pulmonary arteries. We discuss the broad differential diagnosis that includes heritable and non-inheritable etiologies. A keen clinical awareness of this broader array of arterial aneurysms is essential for accurate early diagnosis and proper management.

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INTRODUCTION: Wound infections represent a serious complication after vascular surgery particularly after vascular reconstructive procedures. We aimed to identify risk factors predisposing patients to these complications. METHODS: This was a retrospective review of open vascular surgical procedures performed between April 2014 and March 2019 in Kuwait. Patient demographics, procedures performed and their indications, and post-operative outcomes were collected and analyzed. Patients with pre-operative active infections were excluded from the analysis. Statistical analysis was performed, and odds ratios (ORs) and relative risks were calculated for the outcomes of interest. Fisher's exact test and two-tailed t test were used where appropriate. RESULTS: 391 patients were identified. The majority (54%) presented with chronic limb threatening ischemia. The mean age was 58 (±10) years, with a male predominance (76%). Wound infection occurred in 53 (14%) patients. The most commonly isolated organism was Staphylococcus aureus (47%). Diabetes (OR 8.03, 95% CI: 1.9142-33.7439, p = 0.0044), hypertension (OR 2.38, 95% CI: 1.2960-4.3684, p = 0.0052), ischemic heart disease (OR 2.30, 95% CI: 1.4349-4.6987, p = 0.0016), hyperlipidemia (OR 2.12, 95% CI: 1.0305-4.3620, p = 0.0412), and chronic renal failure (OR 2.55, 95% CI: 1.0181-6.4115, p = 0.0457) were all found to be significantly associated with the development of post-operative wound infections in vascular surgery patients. CONCLUSION: Diabetes, hypertension, ischemic heart disease, hyperlipidemia, and chronic renal failure were associated with post-operative wound infections. Anticipation of wound complications in patients with these risk factors may aid early diagnosis and treatment.

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Although exostosis or osteochondroma is a common bone tumor, associated vascular complications are rare. Clinical and radiological diagnoses are sometimes challenging, and there is no codification for surgical management. We report two cases of popliteal arterial pseudoaneurysms due to osteochondroma of the distal femur. A review of the current literature about case series and case reports of patients affected by arterial pseudoaneurysm complicating osteochondroma was also performed.

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PURPOSE: To clarify whether a true neuroretinitis is part of  idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. METHODS: Critical literature review. RESULTS: Neuroretinitis has been considered as a prominent feature of IRVAN syndrome. It has been described as late diffuse optic  disc staining on fluorescein angiography. However, patients with IRVAN syndrome usually do not present with visual function alterations consistent with optic neuropathy or neuroretinitis. The typical stellate exudative maculopathy seen in IRVAN syndrome is primarily caused by leakage from retinal arteriolar aneurysms at or near the optic disc, and it could be misinterpreted as a feature of neuroretinitis. CONCLUSION: There is no clinical evidence of true neuroretinitis at any of the disease stages in patients with IRVAN syndrome. The acronym « IRVARE ¼ (Idiopathic Retinal Vasculitis, Aneurysms, and Retinal Exudates) is proposed to better characterize this syndrome.

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Loeys-Dietz syndrome (LDS) is a rare connective tissue disease associated with mutations in transforming growth factor (TGF) signaling leading to an increased risk of arterial calcification, aneurysms, and/or dissections. We report a case in which genetics evaluation revealed a rare variant E244K in the TGFB3 gene. The variant leads to the substitution of glutamic acid for lysine, two amino acids with dissimilar properties. Analysis from evolutionary data shows the glutamic acid is maintained across species. The clinical significance of the E244K variant in association with LDS was never previously reported as pathologic. This case report aims to report that the significance of the E244K variant in the TGFB3 gene is found to be pathologic in our case. A search on the Genome Aggregation Database (gnomAD) did not reveal any previously identified individuals with this variant, despite being a well-covered region. ClinVar has a few entries for E244K, where most of them are listed as unknown significance. Bringing together the genotype evidence with our patient's clinical picture, we consider the variant to be pathogenic for this family.

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BACKGROUND: Multiple arterial aneurysms are often seen in the elderly and are caused mainly by atherosclerosis.

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PURPOSE/AIM: This study aims to compare efficacy and safety of Low-profile Visualized Intraluminal Support (LVIS) stent and Pipeline Embolization Device (PED)-assisted endovascular interventions in patients with middle cerebral arterial aneurysms. MATERIALS AND METHODS: This retrospective study included a total of 144 MCA patients, who went to our hospital from January 2013 to December 2017. These patients were divided into two groups: LVIS group, 81 cases; PED group, 63 cases. Then, the basic clinical characteristics were collected. The Raymond grade was used to evaluate the embolism immediately after the surgery and at six months after surgery. The Glasgow Outcome Scale (GOS) and modified Rankin scale were used at six months after surgery, and the recurrence rate was also recorded. RESULTS: The mean aneurysm diameter was 7.7 ± 5.1, which range from 2-25 mm. There were 25 cases with ruptured aneurysms. The results revealed that 57 (70.4%) cases in the LVIS group and 41 (65.1%) cases in the PED group presented with complete embolisms immediately after the surgery. The mean GOS score after six months was 4.6 ± 0.9 in the LVIS group and 4.6 ± 0.9 in the PED group. Furthermore, a total of 86.4% cases had a good prognosis in the LVIS group, while 87.3% cases had a good prognosis in the PED group. Moreover, recurrence was found in six (7.4%) cases in the LVIS group and in five (7.9%) cases in the PED group. CONCLUSION: Both these two methods are effective and safe, with a high good prognosis rate and low recurrence. However, there was no significant difference between the PED and LVIS stent methods.

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Normally, smooth muscle cells (SMCs) are localized in the tunica media of the vasculature, where they take responsibility for vascular contraction and extracellular matrix (ECM) generation. SMCs also play a significant role in obedience and elastic rebound of the artery in response to the haemodynamic condition. However, under pathological or stressed conditions, phenotype switching from contractile to synthetic state or other cell types will occur in SMCs to positively or negatively contribute to disease progression. Various studies demonstrated that functional changes of SMCs are implicated in several cardiovascular diseases. In this review, we present the function of vascular SMCs (VSMCs) and the involved molecular mechanisms about phenotype switching, and summarize the roles of SMCs in atherosclerosis, hypertension, arterial aneurysms and myocardial infarction, hoping to obtain potential therapeutic targets against cardiovascular disease in the clinical practices.

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BACKGROUND: Matrix metalloproteinases (MMPs) are involved in vascular wall degradation, and drugs able to modulate MMP activity can be used to prevent or treat aneurysmal disease. In this study, we evaluated the effects of statins on MMP-2, MMP-9, and neutrophil gelatinase-associated lipocalin (NGAL) in both plasma and tissue in patients with aneurysmal disease. METHODS: We performed a prospective, single-blind, multicenter, control group clinical drug trial on 184 patients of both sexes >18 years old with a diagnosis of arterial aneurysmal disease. Enrolled patients were divided into two groups: Group I under statin treatment and Group II not taking statins. In addition, 122 patients without aneurysmal disease and under statin treatment were enrolled as a control group (Group III). The expression of MMPs and NGAL in plasma was evaluated using ELISA, while their expression in endothelial tissues was evaluated using Western blot. RESULTS: The ELISA test revealed greater plasma levels (p < 0.01) of MMPs and NGAL in Groups I and II vs. Group III. Western blot analysis showed higher expression (p < 0.01) of MMPs and NGAL in Group II vs. Group I, and this increase was significantly higher (p < 0.01) in patients treated with low potency statins compared to high potency ones. CONCLUSIONS: MMPs and NGAL seem to play a major role in the development of aneurysms, and their modulation by statins suggests that these drugs could be used to prevent arterial aneurysmal disease.

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Loeys-Dietz syndrome (LDS) is a connective tissue disorder first described in 2005 featuring aortic/arterial aneurysms, dissections, and tortuosity associated with craniofacial, osteoarticular, musculoskeletal, and cutaneous manifestations. Heterozygous mutations in 6 genes (TGFBR1/2, TGFB2/3, SMAD2/3), encoding components of the TGF-ß pathway, cause LDS. Such genetic heterogeneity mirrors broad phenotypic variability with significant differences, especially in terms of the age of onset, penetrance, and severity of life-threatening vascular manifestations and multiorgan involvement, indicating the need to obtain genotype-to-phenotype correlations for personalized management and counseling. Herein, we report on a cohort of 34 LDS patients from 24 families all receiving a molecular diagnosis. Fifteen variants were novel, affecting the TGFBR1 (6), TGFBR2 (6), SMAD3 (2), and TGFB2 (1) genes. Clinical features were scored for each distinct gene and matched with literature data to strengthen genotype-phenotype correlations such as more severe vascular manifestations in TGFBR1/2-related LDS. Additional features included spontaneous pneumothorax in SMAD3-related LDS and cervical spine instability in TGFB2-related LDS. Our study broadens the clinical and molecular spectrum of LDS and indicates that a phenotypic continuum emerges as more patients are described, although genotype-phenotype correlations may still contribute to clinical management.

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BACKGROUND: Few long-time follow-up studies describe all complications, treatment outcome of complications, and mortality in relation to endovascular aneurysm repair (EVAR). The purpose of this study was to evaluate the incidence and treatment outcome including mortality of radiological visible complications related to the EVAR procedure at a single center with up to 10 years' surveillance. MATERIALS AND METHODS: Patients treated with EVAR from March 2006 to March 2016 at a Danish university hospital, 421 in total, were included. Patient and aneurysm characteristics, follow-up, and secondary intervention data were collected from a national database and medical records. Follow-up computed tomography angiography and plain abdominal X-ray reports were reviewed for complications. Scans and X-rays with suspected complications were evaluated by an interventional radiologist. RESULTS: A total of 172 complications in 147 patients, mainly in the beginning of the follow-up period, were found; 35% had a least one complication. The main part of complications (62%) was type II endoleaks, followed by stent graft stenosis (11%), type I endoleaks (9%), and stent graft occlusion (7%). A total of 66 (38%) complications, observed in 55 patients, were treated with reintervention, of which 77% were treated with endovascular procedures and 23% with surgical treatment, that is, 13% of all studied patients had a complication that required a reintervention. The remaining 2 of the 3 complications were treated conservatively. We found no increased all-cause mortality in connection with having a complication including those requiring reintervention. CONCLUSION: We presented a 10-year single-center study of EVAR. Many patients treated with EVAR had a radiological visible complication, mainly in the beginning of the follow-up period. Only a smaller fraction required reintervention and having a reintervention-requiring complication was not connected to increased mortality.

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A 73-year-old man had multiple coronary aneurysms that resulted in acute myocardial infarction on the day before surgery for cerebral aneurysms. Emergent coronary angiography revealed that the lesion that caused the myocardial infarction was a distal left circumflex artery, and two huge coronary aneurysms were also found in the left circumflex artery. A two-stage treatment strategy was planned, including coronary aneurysm surgery, followed by cerebral aneurysm surgery. He underwent coronary artery aneurysmorrhaphy with closure of the ostia of the afferent and efferent arteries, and coronary artery bypass grafting with a saphenous vein graft applied to the left circumflex artery. The pathological findings suggested chronic thromboangiitis, as the inflammatory cells were observed to have infiltrated the coronary artery wall. The tissue remodeling of the aneurysmal wall indicated a positive response to tenascin C. We report a case of multiple coronary aneurysms, focusing on the pathological findings. .

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PURPOSE: To describe two young Saudi brothers with bilateral progressive retinal arterial aneurysms and a subtotal exudative retinal detachment with Coats-like presentation in the older sibling as the initial presentation of Familial Retinal Arterial Macroaneurysms (FRAM). OBSERVATIONS: Two young Saudi brothers with a family history of consanguinity presented with the classic clinical presentation and genetic identification of FRAM. In this report, we describe the presence of prominent peripheral retinal capillary changes mimicking Coats' disease. CONCLUSIONS AND IMPORTANCE: FRAM can present similar to bilateral Coats' disease and should be considered in the differential diagnosis of Coats-like retinopathy. The diagnosis of FRAM may have a significant implication because of the associated cardiac abnormality, such as supravalvular pulmonary stenosis, which should be evaluated by echocardiography and managed accordingly.

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OBJECTIVE: To evaluate the parameters related to the high risk of preoperative seizures in infants (1 year or younger) with ruptured intracranial aneurysms. METHODS: Infants with ruptured intracranial aneurysms treated at our institution from January 2012 to January 2018 were retrospectively analyzed. Seventeen similar cases of infant patients with seizures reported in published studies were reviewed. RESULTS: The mean age of the 7 infant patients treated at our institution was 4.1 ± 3.3 months (range, 28 days to 11 months), with 2 male and 5 female subjects. One patient (14.3%) had an internal carotid artery aneurysm with subarachnoid hemorrhage, and the remaining 6 patients (85.7%) had middle cerebral artery (MCA) aneurysms in the distal arterial region with lobe hemorrhage. Five of the 7 infants (71.4%) had seizures, 4 of whom (4/5, 80.0%) had MCA distal arterial aneurysms with lobe hemorrhage. Management was successful for all patients with aneurysm clipping or resection surgery, and one patient experienced postoperative seizures. Of the 17 reviewed cases of infant patients with seizures, 10 patients (58.8%) exhibited the typical distal arterial aneurysm with lobe hemorrhaging, and 6 (60%) of them had aneurysms in MCA. CONCLUSIONS: Infant patients with ruptured intracranial aneurysms have a high risk of preoperative seizures. The typical radiologic finding of distal arterial aneurysm with lobe hemorrhage was frequently observed in the MCA, and it might be related to the high risk of preoperative seizures in this population. Microsurgical techniques effectively control postoperative seizures in infants with ruptured intracranial aneurysms.

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BACKGROUND: Although research on pediatric intracranial aneurysms is well documented, studies of pediatric intracranial distal arterial aneurysms (PIDAAs) remain scarce. METHODS: All pediatric patients (≤ 18 years) with intracranial aneurysms located distally to the M2 segment of the middle cerebral artery (MCA), A2 segment of the anterior cerebral artery, P2 segment of the posterior cerebral artery, and other cerebral arteries distal to the main branch who were treated at our center between January 2012 and April 2017 were retrospectively reviewed. RESULTS: Thirty-five PIDAAs were observed in 35 patients with a mean age of 9.2 ± 5.9 years and a male ratio of 71.4% (n = 25). Sudden onset of severe headache was the most common symptom (n = 22, 62.9%), followed by seizures (n = 21, 60%). Twenty-seven patients (77.1%) had ruptured aneurysms, and 18 of them (66.7%) exhibited combined lobe intracerebral hematoma. MCA was the most common site (n = 23, 65.7%). Large or giant aneurysms were observed in 16 patients (45.7%). Eight patients (22.9%) had pseudoaneurysms, six of whom (75%) reported a head trauma history. Thirty patients (85.7%) had favorable outcomes at the end of the 6-month follow-up. Kaplan-Meier 6-month seizure curves revealed a significantly higher decrease in seizure rates in the surgical group than in the endovascular group (P = 0.036). CONCLUSIONS: PIDAAs constitute a high proportion of pediatric intracranial aneurysms. PIDAAs are associated with an increased risk of seizures, a predilection for the MCA, and a high incidence of pseudoaneurysms with head trauma history. Surgical intervention is of greater benefit than endovascular treatment in controlling the risk of seizures.

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PURPOSE: To develop a better understanding of pediatric intracranial aneurysms (PIAs). METHODS: All PIAs treated in our center from January 2012 to April 2017 were retrospectively included. Clinical data, treatment summaries, and follow-up outcomes were retrieved and analyzed. RESULTS: A total of 66 PIAs were found in 64 patients with a mean age of 11.4 ± 5.7 years, 68.8% of whom were male. The most common symptoms were seizure (n = 7, 63.6%) for the 0-5 age group and headache (n = 38, 71.7%) for the 6-18 age group. Fifty-one PIAs (77.3%) were located in the anterior circulation, with the middle cerebral artery (MCA) being the most common site (n = 28, 42.4%). Fifteen patients (23.4%) had PIAs that were pseudoaneurysms, and nine of them (60%) had a combined history of head trauma. Thirty-five patients (54.7%) had distal arterial aneurysms, and 21 of them (60%) presented with seizure. During a mean follow-up time of 1.6 ± 1.2 years, 79.7% of patients (n = 51) had favorable outcomes, 5 patients (7.8%) died, and the remaining 8 patients (12.5%) had unfavorable outcomes with severe neurological deficits. CONCLUSION: Apart from characteristics consistent with previous studies, several new findings regarding PIAs were reported, including a difference in the most common symptoms in different age groups, the MCA as the predominant location of PIAs, the high ratio of pseudoaneurysms and their head trauma etiology, and the incidence of distal arterial aneurysms and their relationship with the risk of seizures.

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Multipotent stem cells - such as mesenchymal stem/stromal cells and stem cells derived from different sources like vascular wall are intensely studied to try to rapidly translate their discovered features from bench to bedside. Vascular wall resident stem cells recruitment, differentiation, survival, proliferation, growth factor production, and signaling pathways transduced were analyzed. We studied biological properties of vascular resident stem cells and explored the relationship from several factors as Matrix Metalloproteinases (MMPs) and regulations of biological, translational and clinical features of these cells. In this review we described a translational and clinical approach to Adult Vascular Wall Resident Multipotent Vascular Stem Cells (VW-SCs) and reported their involvement in alternative clinical approach as cells based therapy in vascular disease like arterial aneurysms or peripheral arterial obstructive disease.

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