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Annals of Clinical and Translational... Jun 2024Fabry disease is caused by enzymatic defects in alpha-galactosidase A that leads to the accumulation of glycosphingolipids throughout the body, resulting in a...
OBJECTIVE
Fabry disease is caused by enzymatic defects in alpha-galactosidase A that leads to the accumulation of glycosphingolipids throughout the body, resulting in a multisystemic disorder. The most common neurological manifestations are neuropathic pain, autonomic nervous system dysfunction and strokes, but some rarer neurological manifestations exist. Among these, aseptic meningitis is a possible complication. Our objectives were to measure the prevalence of this complication in a cohort of patients with Fabry disease, and to describe its clinical features.
METHODS
We conducted a retrospective review of Fabry disease patients followed at our tertiary referral center between 1995 and September 2023 with at least one episode of meningitis, and performed a systematic review to identify similar published cases.
RESULTS
Four patients out of 107 (3.7%) had at least one episode of aseptic meningitis. Our systematic review identified 25 other observations. The median age of these 29 patients was 29.0 years, the median cerebrospinal fluid leukocyte count was 24 cells/mm3 with a predominance of lymphocytes in 64.7% of cases. In 82.8% of the patients, the diagnosis of Fabry disease was unknown before the meningitis. Large artery stenosis was present in 17.2% of patients and 57.1% of patients had a recent stroke concomitant with the meningitis. Several differential diagnoses were evoked, such as multiple sclerosis or central nervous system vasculitis.
INTERPRETATION
Our study suggests that Fabry disease should be considered as a cause of aseptic meningitis. The pathophysiological mechanisms underlying meningeal inflammation remain largely unknown but may reflect the dysregulation of pro-inflammatory signaling pathways.
Topics: Humans; Fabry Disease; Meningitis, Aseptic; Adult; Male; Female; Retrospective Studies; Middle Aged; Young Adult; Adolescent; Aged; Child
PubMed: 38717582
DOI: 10.1002/acn3.52043 -
European Journal of Vascular and... Dec 2022Since the first description of the median arcuate ligament syndrome (MALS), the existence for the syndrome and the efficacy of treatment for it have been questioned. (Review)
Review
OBJECTIVE
Since the first description of the median arcuate ligament syndrome (MALS), the existence for the syndrome and the efficacy of treatment for it have been questioned.
METHODS
A systematic review conforming to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement was conducted, with a broader view on treatment for MALS including any kind of coeliac artery release, coeliac plexus resection, and coeliac plexus blockage, irrespective of age. Online databases were used to identify papers published between 1963 and July 2021. The inclusion criteria were abdominal symptoms, proof of MALS on imaging, and articles reporting at least three patients. Primary outcomes were symptom relief and quality of life (QoL).
RESULTS
Thirty-eight studies describing 880 adult patients and six studies describing 195 paediatric patients were included. The majority of the adult studies reported symptom relief of more than 70% from three to 228 months after treatment. Two adult studies showed an improved QoL after treatment. Half of the paediatric studies reported symptom relief of more than 70% from six to 62 months after laparoscopic coeliac artery release, and four studies reported an improved QoL. Thirty-five (92%) adult studies and five (83%) paediatric studies scored a high or unclear risk of bias for the majority of the Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) items. The meaning of coeliac plexus resection or blockage could not be substantiated.
CONCLUSION
This systematic review suggests a sustainable symptom relief of more than 70% after treatment for MALS in the majority of adult and paediatric studies; however, owing to the heterogeneity of the inclusion criteria and outcome parameters, the risk of bias was high and a formal meta-analysis could not be performed. To improve care for patients with MALS the next steps would be to deal with reporting standards, outcome definitions, and consensus descriptions of the intervention(s), after which an appropriate randomised controlled trial should be performed.
Topics: Adult; Humans; Child; Median Arcuate Ligament Syndrome; Quality of Life; Constriction, Pathologic; Celiac Artery; Decompression, Surgical
PubMed: 36075541
DOI: 10.1016/j.ejvs.2022.08.033 -
Journal of Personalized Medicine Apr 2022Patients with severe aortic valve stenosis and concomitant pulmonary hypertension show a significantly reduced survival prognosis. Right heart catheterization as a... (Review)
Review
Severe Aortic Valve Stenosis and Pulmonary Hypertension: A Systematic Review of Non-Invasive Ways of Risk Stratification, Especially in Patients Undergoing Transcatheter Aortic Valve Replacement.
Patients with severe aortic valve stenosis and concomitant pulmonary hypertension show a significantly reduced survival prognosis. Right heart catheterization as a preoperative diagnostic tool to determine pulmonary hypertension has been largely abandoned in recent years in favor of echocardiographic criteria. Clinically, determination of echocardiographically estimated systolic pulmonary artery pressure falls far short of invasive right heart catheterization data in terms of accuracy. The aim of the present systematic review was to highlight noninvasive possibilities for the detection of pulmonary hypertension in patients with severe aortic valve stenosis, with a special focus on cardiovascular biomarkers. A total of 525 publications regarding echocardiography, cardiovascular imaging and biomarkers related to severe aortic valve stenosis and pulmonary hypertension were analyzed in a systematic database analysis using PubMed Central. Finally, 39 publications were included in the following review. It was shown that the current scientific data situation, especially regarding cardiovascular biomarkers as non-invasive diagnostic tools for the determination of pulmonary hypertension in severe aortic valve stenosis patients, is poor. Thus, there is a great scientific potential to combine different biomarkers (biomarker scores) in a non-invasive way to determine the presence or absence of PH.
PubMed: 35455719
DOI: 10.3390/jpm12040603 -
Cureus Feb 2023Carotid endarterectomy (CEA) is a surgical procedure that treats the narrowed carotid arteries, which may be narrowed by atherosclerosis. Stenting is the insertion of a... (Review)
Review
Carotid endarterectomy (CEA) is a surgical procedure that treats the narrowed carotid arteries, which may be narrowed by atherosclerosis. Stenting is the insertion of a wire mesh scaffold into the narrowed portion of the carotid artery to keep it open by preventing blood from clotting. Using the study done over 10 years back as a point of reference, this study will seek an update on an assessment comparing CEA and stenting in studies carried out between 2015 and to date. The PICOS (population, intervention, control, outcome, and study designs) criteria were used to construct a set of inclusion and exclusion guidelines. This meta-analysis and systematic review used two forms of investigative analysis; both quantitative and qualitative assessments. From the studies, stroke (95% CI: 0.51-0.71, P < 0.001), myocardial infarction (95% CI: 1.49-3.42, P = 0.001), and stroke or death analysis (95% CI: 0.53-0.77, P < 0.001) were noted to be significant. From the analysis, CEA was observed as having better treatment results in terms of stroke events and stroke or death incidences when compared to stenting. Carotid stenting was observed as having lower cases of myocardial infarctions when compared to endarterectomy.
PubMed: 36942176
DOI: 10.7759/cureus.35070 -
European Journal of Vascular and... Mar 2022Intravascular lithotripsy (IVL) is a novel technique for plaque modification during endovascular revascularisation for peripheral artery disease (PAD) with severe... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Intravascular lithotripsy (IVL) is a novel technique for plaque modification during endovascular revascularisation for peripheral artery disease (PAD) with severe calcification. The aim of this paper was to perform a systematic review and meta-analysis of contemporary data to elucidate the efficacy and safety of IVL in lower extremity PAD.
DATA SOURCES
A systematic literature search with pre-defined search terms was performed using PubMed, Web of Sciences, OvidSP, and EMBASE.
REVIEW METHODS
A meta-analysis was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. Patient characteristics, lesion calcification, pre-IVL and post-IVL diameter stenosis, complications, and stent rates were evaluated.
RESULTS
Nine studies were included, encompassing a total of 681 patients (769 lesions) with IVL performed for PAD, of which 75.53% (95% confidence interval [CI] 66.08% - 83.03%) of the lesions were reported to have severe calcification. Comparison between pre-IVL and post-IVL diameter stenosis demonstrated a diameter stenosis reduction of 59.3% (95% CI 53.30% - 65.31%). Vascular complications were rare, with flow limiting or type D/E/F dissection occurring in only 1.25% (95% CI 0.60% - 2.61%) of cases. The overall pooled event rate for stent placement was 15.89% (95% CI 5.22% - 39.34%).
CONCLUSION
This meta-analysis supports IVL as an effective and safe approach for calcified plaque modification in lower extremity PAD, achieving a diameter stenosis reduction of 59.3% (95% CI 53.30% - 65.31%) with minimal vascular complications. Routine use of this device is not recommended; further high quality evidence is required to elucidate the efficacy of IVL with respect to different clinical characteristics such as lesion location and length, and in comparison with other treatment modalities such as atherectomy.
Topics: Humans; Lithotripsy; Lower Extremity; Peripheral Arterial Disease; Treatment Outcome; Vascular Calcification
PubMed: 34887206
DOI: 10.1016/j.ejvs.2021.10.035 -
Frontiers in Neurology 2022We performed a meta-analysis to indirectly compare the treatment effectiveness of balloon angioplasty and stenting for patients with intracranial arterial stenosis.
AIMS
We performed a meta-analysis to indirectly compare the treatment effectiveness of balloon angioplasty and stenting for patients with intracranial arterial stenosis.
METHODS
Literature searches were performed in well-known databases to identify eligible studies published before January 04, 2021. The incidence of restenosis, transient ischemic attack (TIA), stroke, death, and dissection after balloon angioplasty or stenting were pooled. An indirect comparison of balloon angioplasty vs. stenting was performed, and the ratios of incidence (RIs) with 95% confidence intervals (CIs) were calculated using the random-effects model.
RESULTS
120 studies that recruited 10,107 patients with intracranial arterial stenosis were included. The pooled incidence of restenosis after balloon angioplasty and stenting were 13% (95%CI: 8-17%) and 11% (95%CI: 9-13%), respectively, with no significant difference between them (RI: 1.18; 95%CI: 0.78-1.80; = 0.435). Moreover, the pooled incidence of TIA after balloon angioplasty and stenting was 3% (95%CI: 0-6%) and 4% (95%CI: 3%-5%), and no significant difference was observed (RI: 0.75; 95%CI: 0.01-58.53; = 0.897). The pooled incidence of stroke after balloon angioplasty and stenting was 7% (95%CI: 5-9%) and 8% (95%CI: 7-9%), respectively, and the difference between groups was found to be statistically insignificant (RI: 0.88; 95%CI: 0.64-1.20; = 0.413). Additionally, the pooled incidence of death after balloon angioplasty and stenting was 2% (95%CI: 1-4%) and 2% (95%CI: 1-2%), with no significant difference between groups (RI: 1.00; 95%CI: 0.44-2.27; = 1.000). Finally, the pooled incidence of dissection after balloon angioplasty and stenting was 13% (95%CI: 5-22%) and 3% (95%CI: 2-5%), respectively, and balloon angioplasty was associated with a higher risk of dissection than that with stenting for patients with intracranial arterial stenosis (RI: 4.33; 95%CI: 1.81-10.35; = 0.001).
CONCLUSION
This study found that the treatment effectiveness of balloon angioplasty and stenting were similar for patients with symptomatic intracranial arterial stenosis.
PubMed: 35775041
DOI: 10.3389/fneur.2022.878179 -
Frontiers in Pharmacology 2023Stroke is currently the second-leading cause of death just behind ischaemic heart disease. Drug therapy is currently the standard of care for patients with symptomatic...
Stroke is currently the second-leading cause of death just behind ischaemic heart disease. Drug therapy is currently the standard of care for patients with symptomatic intracranial artery stenosis (sICAS). Stenting is an important treatment for the prevention and treatment of ischemic stroke. It has been suggested that vertebral artery stenting might reduce this risk, but operation-related complications limit the application of stenting in the treatment of ischemic stroke. The differences in the safety and efficacy of stenting combined with drugs and drugs alone in the treatment of sICAS are unclear. The aim of this study was to assess the impact of both treatment modalities on the prognosis of patients with sICAS through a systematic review and meta-analysis. The Chinese databases (CNKI, Wanfang, VIP, CBM, DUXIU) and English databases (Pubmed, Embase, Ovid_medline, Cochrane library, Web of science)were searched to identify all studies describing sICAS. The "Risk of Bias Assessment" tool and the "Jadad Scale" provided by the Cochrane Collaboration were used to evaluate the risk of bias and quality of the collected literature. The risk ratio (RR) and its 95% confidence interval (CI) were determined using Stata statistical software version 14.0. A total of 11 studies were included, comprising a total of 1,915 patients. The combined results of the study showed no significant difference between the incidence of transient cerebral ischemia (TIA)and stroke in patients with sICAS treated with drugs in combination with stents versus drugs alone. The incidence of death or stroke, cerebral haemorrhage, disabling stroke or death was significantly higher in patients receiving stent-combined drug therapy versus drug therapy alone for sICAS. Studies suggest that stenting combined with medication for patients with sICAS may increase the incidence of death or stroke, cerebral haemorrhage, stroke or death, but has no significant effect on the incidence of TIA and stroke. The studies report inadequate and conflicting data and therefore the safety and efficacy of stenting for sICAS should be interpreted with caution. https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42022377090, identifier CRD42022377090.
PubMed: 37361212
DOI: 10.3389/fphar.2023.1122842 -
Diagnostics (Basel, Switzerland) Feb 2023Stenosis grade of the carotid arteries has been the primary indicator for risk stratification and surgical treatment of carotid artery disease. Certain characteristics... (Review)
Review
Stenosis grade of the carotid arteries has been the primary indicator for risk stratification and surgical treatment of carotid artery disease. Certain characteristics of the carotid plaque render it vulnerable and have been associated with increased plaque rupture rates. Computed tomography angiography (CTA) and magnetic resonance angiography (MRA) have been shown to detect these characteristics to a different degree. The aim of the current study was to report on the detection of vulnerable carotid plaque characteristics by CTA and MRA and their possible association. A systematic review of the medical literature was executed, utilizing PubMed, SCOPUS and CENTRAL databases, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) 2020 guidelines. The study protocol has been registered to PROSPERO (CRD42022381801). Comparative studies reporting on both CTA and MRA carotid artery studies were included in the analysis. The QUADAS tools were used for risk of bias diagnostic imaging studies. Outcomes included carotid plaque vulnerability characteristics described in CTA and MRA and their association. Five studies, incorporating 377 patients and 695 carotid plaques, were included. Four studies reported on symptomatic status (326 patients, 92.9%). MRA characteristics included intraplaque hemorrhage, plaque ulceration, type VI AHA plaque hallmarks and intra-plaque high-intensity signal. Intraplaque hemorrhage detected in MRA was the most described characteristic and was associated with increased plaque density, increased lumen stenosis, plaque ulceration and increased soft-plaque and hard-plaque thickness. Certain characteristics of vulnerable carotid plaques can be detected in carotid artery CTA imaging studies. Nevertheless, MRA continues to provide more detailed and thorough imaging. Both imaging modalities can be applied for comprehensive carotid artery work-up, each one complementing the other.
PubMed: 36832133
DOI: 10.3390/diagnostics13040646 -
Genes Jun 2024The ring finger protein 213 gene (RNF213) is involved in several vascular diseases, both intracranial and systemic ones. Some variants are common in the Asian population...
The ring finger protein 213 gene (RNF213) is involved in several vascular diseases, both intracranial and systemic ones. Some variants are common in the Asian population and are reported as a risk factor for moyamoya disease, intracranial stenosis and intracranial aneurysms. Among intracranial vascular diseases, both moyamoya disease and intracranial artery dissection are more prevalent in the Asian population. We performed a systematic review of the literature, aiming to assess the rate of RNF213 variants in patients with spontaneous intracranial dissections. Four papers were identified, providing data on 53 patients with intracranial artery dissection. The rate of RNF213 variants is 10/53 (18.9%) and it increases to 10/29 (34.5%), excluding patients with vertebral artery dissection. All patients had the RNF213 p.Arg4810Lys variant. RNF213 variants seems to be involved in intracranial dissections in Asian cohorts. The small number of patients, the inclusion of only patients of Asian descent and the small but non-negligible coexistence with moyamoya disease familiarity might be limiting factors, requiring further studies to confirm these preliminary findings and the embryological interpretation.
Topics: Humans; Adenosine Triphosphatases; Aortic Dissection; Asian People; Genetic Predisposition to Disease; Intracranial Aneurysm; Moyamoya Disease; Polymorphism, Single Nucleotide; Ubiquitin-Protein Ligases
PubMed: 38927660
DOI: 10.3390/genes15060725 -
Frontiers in Neurology 2022Intracranial angioplasty with a self-expandable stent (SES) is an important endovascular therapy for symptomatic intracranial arterial stenosis. We sought to update the...
BACKGROUND
Intracranial angioplasty with a self-expandable stent (SES) is an important endovascular therapy for symptomatic intracranial arterial stenosis. We sought to update the evaluation of the perioperative safety and long-term outcomes of self-expandable stent for the treatment of symptomatic intracranial arterial stenosis.
METHODS
We comprehensively searched the published literature from each database through Sept 16, 2022, for the PubMed, EMBASE, Web of Science, Cochrane, and Clinical Trials databases. The characteristics of the studies and patients, perioperative complications, and long-term outcomes were extracted. The pooled outcomes and 95% confidence intervals (CIs) were estimated by Stata Statistical Software 14.0.
RESULTS
A total of 4,632 patients from 58 studies were included. The pooled rate of perioperative stroke or death was 6.32% (95% CI 5.04-7.72%); ischemic stroke beyond 30 days through 1 year was 2.72% (95% CI 1.41-4.38%). Perioperative complications differed between the 2014-2022 and 2005-2013 subgroups, as did long-term outcomes between the off-label SES and Wingspan subgroups.
CONCLUSION
The perioperative complications of intracranial angioplasty with SES have been reduced, but the risk of perioperative stroke or death is still higher than that of aggressive medical therapy, and additional studies are needed to determine whether it has better long-term outcomes than aggressive medical therapy. Perioperative complications varied between the 2014-2022 and 2005-2013 subgroups, as did long-term outcomes between the off-label SES and Wingspan subgroups. Given the high level of heterogeneity observed between the included studies, these results should be interpreted with caution and additional studies are needed.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/prospero/, identifier: CRD42022316066.
PubMed: 36698897
DOI: 10.3389/fneur.2022.1074228