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Author(s):  
Mira Merashli ◽  
Tommaso Bucci ◽  
Daniele Pastori ◽  
Pasquale Pignatelli ◽  
Alessia Arcaro ◽  
...  

Abstract Aim To evaluate the relevance of plasma homocysteine (HC) in Behcet's disease (BD) and its clinical manifestations. Methods Systematic review of EMBASE and PubMed databases according to PRISMA guidelines from inception to July 2021; random-effects meta-analyses for continuous outcomes. Results The search strategy retrieved 48 case–control (2,669 BD and 2,245 control participants) and 5 cohort studies (708 BD participants). Plasma HC was higher in BD than in controls (p < 0.0001) with wide heterogeneity (I2  = 89.7%) that remained unchanged after sensitivity analysis according to year of article publication, age of BD participants, study size, study quality, method of HC determination, and male/female ratio >1.5; some pooled ethnicities explained a small part of the heterogeneity (I2  = 16.3%). Active BD participants had higher HC than inactive ones (p < 0.0001), with moderate heterogeneity (I2  = 49.2%) that disappeared after removal of an outlier study with very high disease activity. BD participants with any vascular involvement had higher HC than those without (p < 0.0001) with wide heterogeneity (I2  = 89.7%); subgroup analysis on venous thrombosis only changed neither effect size (p < 0.0001) nor heterogeneity (I2  = 72.7%). BD participants with ocular involvement had higher HC than those without (p < 0.0001) with moderate heterogeneity (I2  = 40.3%). Conclusion Although causality cannot be inferred, the consistency of the elevation of plasma HC in BD, particularly in patients with active disease, with vascular and ocular involvement suggests an intrinsic involvement of HC in these clinical manifestations.


Blood ◽  
2022 ◽  
Author(s):  
Robert Flaumenhaft ◽  
Keiichi Enjyoji ◽  
Alec A Schmaier

COVID-19 is a primary respiratory illness that is frequently complicated by systemic involvement of the vasculature. Vascular involvement leads to an array of complications ranging from thrombosis to pulmonary edema secondary to loss of barrier function. This review will address the vasculopathy of COVID-19 with a focus on the role of the endothelium in orchestrating the systemic response to SARS-CoV-2 infection. The endothelial receptor systems and molecular pathways activated in the setting of COVID-19 and the consequences of these inflammatory and prothrombotic changes on endothelial cell function will be discussed. The sequelae of COVID-19 vascular involvement at the level of organ systems will also be addressed, with an emphasis on the pulmonary vasculature, but with consideration of effects on other vascular beds. The dramatic changes in endothelial phenotypes associated with COVID-19 has enabled the identification of biomarkers that could help guide therapy and predict outcomes. Knowledge of vascular pathogenesis in COVID-19 has also informed therapeutic approaches that may control its systemic sequelae. Since our understanding of vascular response in COVID-19 continues to evolve, we will consider areas of controversy, such as the extent to which SARS-CoV-2 directly infects endothelium and the degree to which vascular responses to SARS-CoV-2 are unique or common to those of other viruses capable of causing severe respiratory disease. This conceptual framework describing how SARS-CoV-2 infection affects endothelial inflammation, prothrombotic transformation, and barrier dysfunction will provide a context for interpreting new information as it arises addressing the vascular complications of COVID-19.


Hand ◽  
2021 ◽  
pp. 155894472110643
Author(s):  
Paul A. Asadourian ◽  
Aaron Z. Chen ◽  
Bryan Aristega Almeida ◽  
Jessica K. Gordon ◽  
Alissa J. Burge ◽  
...  

Background: When patients with systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) develop digital ischemia, conventional angiography (CA) is traditionally used to assess hand vasculature. Recently, Chang et al described an angiographic classification system for patients with SSc. Conventional angiography uses intravascular contrast agents that are nephrotoxic and vasoconstrictive. Owing to these limitations, this study assesses the use of contrast-enhanced magnetic resonance angiography (MRA) as an alternative to CA to evaluate hand vasculature in patients with digital ischemia. Methods: This retrospective case series reports on 38 contrast-enhanced MRAs of hand vasculature from 30 symptomatic patients with SSc (N = 21) or SLE (N = 9). The radial and ulnar arteries (RA, UA) and the superficial and deep palmar arches were evaluated at standard reference points both quantitatively and qualitatively for their diameter, patency, and Chang classification. Results: In SSc MRAs (n = 26), the UA was significantly smaller than the RA and was occluded in 46%. In SLE MRAs (n = 12), the UA and RA had no difference in diameter and the UA was occluded in 25%. In SSc, the most common Chang classification was type 2 (UA involvement) in 44%. In SLE, the most common Chang classification was type 4 (UA and RA involvement) in 45%, with 18% classified as type 2. Conclusions: Contrast-enhanced MRA used to assess hand vasculature in SSc patients with digital ischemia shows similar patterns of vascular involvement as previously demonstrated by CA. While vascular involvement in SSc predominantly involves the UA, the RA is also frequently involved in SLE.


2021 ◽  
Vol 14 (3) ◽  
pp. 387-389
Author(s):  
Vanessa Zarate ◽  
Alejandra Cahuata ◽  
Roxana Díaz ◽  
Giancarlo Alvarado-Gamarra ◽  
Kenny Chonlon ◽  
...  

Background: We describe an 8-month-old boy with leukemia and SARS-CoV-2 infection who developed Kasabach-Merritt phenomenon. He had a positive SARS-COV-2 RT-PCR sample. Hematologic tests showed coagulopathy and intestinal involvement. She was managed in emergency receiving transfusion support and in hospitalization with social isolation measures, she started propanolol and corticotherapy as initial treatment of infantile angiomas. She presented with symptoms of intestinal obstruction and underwent surgery and evidence of hemorrhagic infarction with foci of intestinal ischemic necrosis, ending in ileostomy. We tried to understand a pathophysiological explanation of the dermatologic and gastrointestinal tract involvement by the virus and the atypical form of COVID-19. Given the emerging evidence of endothelial and vascular involvement in COVID-19, the development of tests to detect vascular lesions may be critical to guide the use of new therapeutic strategies.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Asako Yanagisawa ◽  
Akira Naito ◽  
Takayuki Jujo-Sanada ◽  
Nobuhiro Tanabe ◽  
Keiichi Ishida ◽  
...  

Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension caused by persistent thromboembolism of the pulmonary arteries. In clinical practice, CTEPH patients often show obstructive ventilatory impairment, even in the absence of a smoking history. Recent reports imply a tendency for CTEPH patients to have a lower FEV1.0; however, the mechanism underlying obstructive impairment remains unknown. Methods We retrospectively analyzed CTEPH patients who underwent a pulmonary function test and respiratory impedance test to evaluate their exertional dyspnea during admission for right heart catheterization from January 2000 to December 2019. We excluded patients with a smoking history to rule out the effect of smoking on obstructive impairment. Results A total of 135 CTEPH patients were analyzed. The median FEV1.0/FVC was 76.0%, %FEV 1.0 had a negative correlation with the mean pulmonary artery pressure and pulmonary vascular resistance and the CT Angiogram (CTA) obstruction score. A multivariate regression analysis revealed that the CTA obstruction score was an independent factor of a lower %FEV1.0. In the 54 patients who underwent pulmonary endarterectomy, %FEV1.0 was improved in some cases and was not in some. Mean PAP largely decreased after PEA in the better %FEV1.0 improved cases, suggesting that vascular involvement in CTEPH could be associated with spirometry obstructive impairment. Conclusion %FEV1.0 had a significant correlation with the CTA obstruction score. Obstructive impairment might have an etiological relationship with vascular involvement. Further investigations could shed new light on the etiology of CTEPH.


2021 ◽  
Vol 108 (Supplement_9) ◽  
Author(s):  
John Moir ◽  
Nigel Jamieson ◽  
Raneem Albazaz ◽  
Abdullah Al-Adhami ◽  
John Scott ◽  
...  

Abstract Background In line with the NCRI framework, appropriate staging and classification of pancreatic cancer, with particular relevance to vascular involvement, is essential to ensure patients are offered all potential treatment options both at diagnosis and post-neoadjuvant therapy. This pan-specialty national collaborative consensus project, supported by PCUK,AUGIS,PSGBI,RCSEng,NCRI,RCR and BSGAR, aimed to develop a succinct radiological reporting template to allow a more consistent and standardized means of detailing all clinically-relevant aspects of pancreatic cancer, which, in addition to the aforementioned benefits, will allow more efficient MDT review, improved ability to audit national practice, and optimized clinical trial design. Methods In stage one, a core group of stakeholders from surgery, radiology and oncology was formed to establish current practice and determine the optimal data-set for a template. This included a blinded radiological validation study of established templates in use (Beth-Israel, PROTRACT and Glasgow proformas), and national survey of consultant surgeons on the PSGBI mailing list. Thereafter, consensus meetings led to the development of a provisional template. In stage two, representatives from surgery, radiology and oncology at all UK HPB units attended a consensus meeting to discuss and finalise the first official template draft, with subsequent trial utilisation in clinical practice. Results In stage one, the radiological assessment highlighted a significant variation in reporting of vascular involvement, with 100% concordance in only 30% of cases. Within the surgeon survey, amongst various tumour-related factors, most notably a significant majority preferred a specific range of degrees of vascular involvement, and specific information regarding tributary involvement and associated narrowing/occlusion/thrombosis. Both processes, and subsequent national consensus meetings in stage two, helped establish the ideal factors required in a template with respect to usability, clinical relevance, applicability and IT-factors, resulting in the generation of the PACT-UK proforma that will be presented at the congress. Conclusions This pan-specialty collaborative consensus project has successfully produced the first nationally-developed pancreatic cancer radiological reporting template. In stage three, the PACT-UK group aim to continue trialing the proforma nationally, with upcoming workshops facilitated by RCR/BSGAR to ensure buy-in from radiologists at all HPB units. Further representation from all units is welcomed, with the philosophy that template data-points can and will continue to evolve on the basis of ongoing feedback from consensus meetings, and following the development of more aggressive surgical techniques and novel neoadjuvant therapies. Plans for the use of PACT-UK within national audit and clinical trials is under-way.


PLoS ONE ◽  
2021 ◽  
Vol 16 (11) ◽  
pp. e0259503
Author(s):  
Fan Li ◽  
Weiling Zhang ◽  
Huimin Hu ◽  
Xia Zhu ◽  
Yi Zhang ◽  
...  

Objective After a complete remission to treatment for hepatoblastoma, some children still have recurrence. We identified and explored the factors that influence recurrence after complete remission in a retrospective study. Methods Of 197 children with hepatoblastoma, 140 (71.1%) achieved initial complete remission and were enrolled in factor analysis. Variables consisted of age, sex, PRE-Treatment EXTent of tumor (PRETEXT) stage, pathologic type, metastatic disease, serum alpha-fetoprotein level, vascular involvement, and surgical margin status. We employed univariate and multivariate analyses to assess the relationship between each factor and tumor recurrence. Results Of 140 children who achieved initial complete remission, 42 (30%) had recurrent hepatoblastoma. The 5-year overall survival rates for the non-recurrence and recurrence group were 99.0% and 78.6%, respectively. The overall 1-year, 3-year, and 5-year recurrence-free survival (RFS) rates were 77.8%, 69.8%, and 69.8%, respectively. All recurrences occurred within 2 years from complete remission. The RFS rate was significantly higher in children younger than 3 years and in those with mixed pathological type, PRETEXT II and III, without metastatic disease, without vascular involvement, and microscopic negative margin than in that of children older than 3 years, with epithelial pathological type, PRETEXT IV, metastatic disease, vascular involvement, and macroscopic positive margin (P < 0.001, = 0.020, < 0.001, = 0.004, = 0.002, and < 0.001, respectively). The independent risk factors for recurrence after complete remission were age ≥3 years, PRETEXT IV, and metastatic disease (P < 0.05). Conclusion Age, PRETEXT stage, metastatic disease, vascular involvement, pathologic type, and surgical margin status might be associated with recurrent hepatoblastoma after complete remission; meanwhile, age ≥3 years, PRETEXT IV, and metastatic disease are independent risk factors of recurrence. Further research is needed on the causes of tumor recurrence, which may improve the long-term outcomes of children with hepatoblastoma.


2021 ◽  
Vol 162 (48) ◽  
pp. 1940-1945

Összefoglaló. A varicella zoster vírus (VZV-) fertőzés típusos első megjelenése a bárányhimlő, később a reaktiváció során a herpes zoster. Szemészeti tünet az V/I-es agyideget érintő zoster esetén gyakori. A legrettegettebb szemészeti manifesztáció az akut retinanekrózis, mely fulmináns lefolyású, és súlyos szöveti destrukciót, valamint jelentős funkcionális károsodást, gyakran vakságot hagy maga után. Központi idegrendszeri vascularis érintettség előfordulhat bárányhimlőhöz társulóan vagy a későbbi reaktivációk során is, súlyos következményekhez vezetve. A Semmelweis Egyetem Szemészeti Klinikáján akut retinanekrózis tünetével érkező 65 éves férfi esetét ismertetjük. Az Amerikai Szemorvostársaság (AAO) diagnosztikus kritériumainak mindenben megfelelő klinikai kép alapján azonnal indított adekvát dózisú antivirális kezelés mellett 3 nap múlva, contralateralis hemiparesis hátterében, a képalkotó vizsgálat ipsilateralis ischaemiás stroke-ot igazolt. Intraocularis mintából PCR-vizsgálat bizonyította a vírus jelenlétét. Liquormintában enyhe anti-VZV-IgA-pozitivitás mutatkozott. Az aktuális szemészeti betegség és a stroke társulásának hátterében az észlelt paraméterek, valamint a releváns irodalmi adatok alapján a varicella zoster vírus okozta vasculopathiát valószínűsítettük. Gyermekkorban ez az ischaemiás stroke leggyakoribb oka, felnőttkorban pedig az V/I-es agyideg herpeses érintettsége esetén négy és félszeres a kockázat stroke kialakulására. A VZV-reaktiváció okozta akut retinanekrózis és a stroke társulásának lehetősége, bár ismert a nemzetközi irodalomban, magyar szakirodalom tudomásunk szerint eddig nem tárgyalta, ez kiemeli esetünk közlésének jelentőségét. Orv Hetil. 2021; 162(48): 1940–1945. Summary. The typical first onset of varicella zoster virus (VZV) infection is chickenpox, later herpes zoster during reactivation. Ophthalmic symptoms are common in herpes zoster affecting the V/I cranial nerve. The most dreaded ophthalmic manifestation is acute retinal necrosis, which has a fulminant course and leaves severe tissue damage as well as significant functional impairment, often blindness. Vascular involvement in the central nervous system may occur in association with chickenpox or during subsequent reactivations leading to severe consequences. We report the case of a 65-year-old male patient with symptoms of acute retinal necrosis at the Department of Ophthalmology, Semmelweis University. The clinical picture fulfilled the diagnostic criteria of the American Academy of Ophthalmology (AAO) and after 3 days of the immediately initiated adequate therapy, contralateral hemiparesis appeared, that was confirmed as an ipsilateral stroke by imaging study. The PCR analysis of an intraocular sample confirmed the presence of VZV. Mild anti-VZV IgA positivity was observed in the cerebrospinal fluid sample. Based on the current ophthalmic disease, the associated stroke alongside with the relevant literature data, varicella zoster vasculopathy was probable. VZV vasculopathy is the most common cause of ischemic stroke in childhood and in adulthood herpetic involvement of the V/I cranial nerve elevates 4.5 times the risk of stroke formation. Though the possible association of acute retinal necrosis and stroke caused by VZV reactivation is known in the international literature, to the best of our knowledge it has not been discussed in Hungary so far, which highlights the importance of reporting our case. Orv Hetil. 2021; 162(48): 1940–1945.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Sriram Rao ◽  
Thanh-Lan Bui ◽  
Ali Sasani ◽  
Ben Sadeghi ◽  
Anvesh Macherla ◽  
...  

Abstract Background Patients diagnosed with locally advanced pancreatic cancer are usually not eligible for surgical resection because of significant vascular involvement. Stereotactic body radiation therapy and chemotherapy are the treatments recommended by the National Comprehensive Cancer Network criteria. For patients who do not respond to or tolerate stereotactic body radiation therapy and/or chemotherapy, a new option is irreversible electroporation. Irreversible electroporation is a nonthermal minimally invasive ablation technique that uses electrical pulses to induce apoptosis of tumor cells without damage to the extracellular matrix, thus preserving ducts and vessels. Irreversible electroporation requires very precise needle placement, which has limited its ubiquitous use. Intraprocedural cone-beam computed tomography with navigation can be fused with previous imaging to provide real-time tumor navigation capabilities during the procedure to allow for more accurate needle placement and treatment. Here, we present a patient who underwent percutaneous irreversible electroporation with intraprocedural cone-beam computed tomography fusion guidance to treat his pancreatic cancer. Case presentation The patient, an 88-year-old White male, initially presented with abdominal pain, and was ultimately diagnosed with locally advanced pancreatic cancer. He has an excellent performance status and no other comorbidities. He was started on chemotherapy and radiation therapy, with good response. However, continued vascular involvement of the tumors precluded him from safe surgical resection. The patient underwent irreversible electroporation with intraprocedural cone-beam computed tomography fusion navigation. The primary lesion demonstrates no residual tumor, and the soft tissue involvement of the adjacent vasculature has stabilized. Conclusions Although not curative on its own, irreversible electroporation holds promise as a treatment option for patients with locally advanced pancreatic cancer to increase downsizing to curative surgery or increase quality of life. Cone-beam computed tomography navigation can improve irreversible electroporation by providing guidance during needle guidance. Image fusion with previous advanced imaging can improve lesion visualization and targeting, thereby improving the effectiveness of irreversible electroporation.


Author(s):  
Panupong Pota ◽  
Pannipa Suwannasom ◽  
Surin Woragidpoonpol ◽  
Tanop Srisuwan

Abstract Background IgG4-related disease (IgG4-RD) is a chronic fibroinflammatory condition with multiple-organ involvement. Rupture of coronary artery aneurysms (CAAs) in IgG4-RD is rare. Case summary A 65-year-old man with IgG4-RD has suffered from recurrent episodes of arterial aneurysms since 2003. He presented with chest pain and hypotension caused by localized cardiac tamponade at right ventricle free wall due to the rupture of coronary artery aneurysm(CAA) of left anterior descending artery (LAD). An urgent LAD aneurysm repaired with bovine pericardium and obliterated aneurysmal sac with cryo-acrylate glue was done together with coronary artery bypass grafting (CABG) using saphenous vein graft(SVG) to LAD and SVG to posterior descending artery. Three-month after surgery, the follow-up coronary computed tomography angiography (CCTA) revealed a growing in size of LAD and the second obtuse marginal(OM) branch aneurysm. Heart team discussion agreed to schedule the patient for double coil embolization to LAD and second OM aneurysm under intravascular ultrasound guidance. Both aneurysms were successfully obliterated with vascular coils. Two-week follow-up coronary angiogram showed complete occlusion of LAD aneurysm and near occlusion of the second OM branch aneurysm. Discussion CAA rupture is a life-threatening condition that required prompt detection and treatments. In IgG4-RD patients, acute cardiac tamponade suggesting the rupture of CAA. Coil embolization is an alternative treatment in patients who suffered from recurrent CAA after surgical repair. Serial CCTA is important for early detection of aneurysm in IgG4-RD patients who had vascular involvement.


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