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Atypical hemolytic uremic syndrome, or the nondiarrheal form of hemolytic uremic syndrome, is a rare disorder typically classified as familial or sporadic. Recent literature has suggested that approximately 50% of patients have mutations in factor H (CFH), factor I (CFI), or membrane cofactor protein (encoded by CD46). Importantly, results of renal transplantation in patients with mutations in either CFH or CFI are dismal, with recurrent disease leading to graft loss in the majority of cases. We describe an adult renal transplant recipient who developed recurrent hemolytic uremic syndrome 1 month after transplantation. Bidirectional sequencing of CFH, CFI, and CD46 confirmed that the patient was heterozygous for a novel missense mutation, a substitution of a serine reside for a tyrosine residue at amino acid 369, in CFI. This report reemphasizes the importance of screening patients with atypical hemolytic uremic syndrome for mutations in these genes before renal transplantation and shows the challenges in the management of these patients. [ABSTRACT FROM AUTHOR]

We showed recently that the adaptive immune events leading to the development of arthritis in Borrelia burgdorferi isolate 297-vaccinated and Borrelia bissettii-challenged mice involve IL-17. Here, we show in Borrelia-vaccinated and -challenged mice that two cytokines known to induce the production of IL-17, IL-6 and transforming growth factor (TGF)-β, are also involved in the development of arthritis. Vaccinated and challenged mice administered either anti-TGF-β or anti-IL-6 antibodies developed histopathologic changes of the hind paws similar to or greater than untreated control mice. By contrast, simultaneous blockage of these cytokines reduced the severity of arthritis in Borrelia-vaccinated and -challenged mice. Moreover, administration of anti-IL-17 antibodies to these dual-antibody-treated mice completely prevented the development of histopathologic changes of the ankle joints, significantly reduced edema of the hind paws, and prevented the production of anti-outer surface protein A borreliacidal antibodies. These findings demonstrate a role for the combined effects of IL-17, IL-6, and TGF-β in the adaptive immune events leading to the development of Borrelia-induced arthritis. [ABSTRACT FROM AUTHOR]

Progress toward the understanding and management of human colon cancer can be significantly advanced if appropriate experimental platforms become available. We have investigated whether a rat model carrying a knockout allele in the gatekeeper gene Adenomatous polyposis coli (Apc) recapitulates familial colon cancer of the human more closely than existing murine models. We have established a mutagen-induced nonsense allele of the rat Apc gene on an inbred F344/NTac (F344) genetic background. Carriers of this mutant allele develop multiple neoplasms with a distribution between the colon and small intestine that closely simulates that found in human familial adenomatous polyposis patients. To distinguish this phenotype from the predominantly small intestinal phenotype found in most Apc-mutant mouse strains, this strain has been designated the polyposis in the rat colon (Pirc) kindred. The Pirc rat kindred provides several unique and favorable features for the study of colon cancer. Tumor-bearing Pirc rats can live at least 17 months, carrying a significant colonic tumor burden. These tumors can be imaged both by micro computed tomography scanning and by classical endoscopy, enabling longitudinal studies of tumor genotype and phenotype as a function of response to chemopreventive and therapeutic regimes. The metacentric character of the rat karyotype, like that of the human and unlike the acrocentric mouse, has enabled us to demonstrate that the loss of the wild-type Apc allele in tumors does not involve chromosome loss. We believe that the Pirc rat kindred can address many of the current gaps in the modeling of human colon cancer. [ABSTRACT FROM AUTHOR]

Early detection of colorectal cancer is critical for the management of this disease. Biomarkers for early detection of several cancers have been developed and applied clinically in recent years. We have sought to discover candidate biomarkers without the restricted choice of markers placed on microarrays, and without the biological complications of genetic and environmental heterogeneity. We have compared by cDNA subtraction two genetically matched sets of mice, one developing multiple intestinal neoplasia (C57BL/6J-Apc[sup Min]) and the other tumor-free (C57BL/6J). One prominent candidate biomarker, clusterin, was then subjected to a series of validation steps. In situ hybridization and immunohistochemistry were used to analyze clusterin expression at a cellular level on a series of murine intestinal and human colonic neoplasms. Elevated clusterin expression was characterized within certain regions of murine and human tumors regardless of tumor stage, location, or mode of initiation. The cells showing high clusterin levels generally lacked differentiation markers and adenomatous polyposis coli antigen. Tumor cells undergoing apoptosis expressed low levels of clusterin. Its specific expression patterns and correlation with cellular events during tumorigenesis make it a useful diagnostic tool in the mouse and a potential contributor to the set of biomarkers for early detection of human colon cancer. [ABSTRACT FROM AUTHOR]

We report a 32-year-old male 14 years post-living-related kidney transplant presenting with new-onset hematuria and BK viremia. He was found to have BK virus-associated urothelial carcinoma originating in the renal allograft with locally advanced disease and metastases to multiple sites. He also developed acute T-cell-mediated rejection in the setting of immunosuppression reduction for BK viremia prior to undergoing transplant nephrectomy. Eight months following transplant nephrectomy and immunosuppression cessation, distant metastases persisted with partial response to chemotherapy and immunotherapy. Here, we discuss this unique presentation and compare it with other BK virus-associated allograft carcinomas reported in the literature, in addition to discussing evidence for the role of BK virus in oncogenesis. [ABSTRACT FROM AUTHOR]

During the hibernation season, livers from 13-lined ground squirrels ( Ictidomys tridecemlineatus) are resistant to damage induced by ex vivo, cold ischemia-warm reperfusion (IR) compared with livers from summer squirrels or rats. Here, we tested the hypothesis that hibernation also reduces damage to ground squirrel livers in an in vivo, warm IR model, which more closely resembles complications associated with traumatic injury or surgical interventions. We also examined whether protection is mediated by two metabolites, inosine and biliverdin, that are elevated in ground squirrel liver during interbout arousals. Active squirrels in spring and hibernators during natural arousals to euthermia (body temperature 37 °C) were subject to liver IR or sham treatments. A subset of hibernating squirrels was pre-treated with compounds that inhibit inosine synthesis/signaling or biliverdin production. This model of liver IR successfully induced hepatocellular damage as indicated by increased plasma liver enzymes (ALT, AST) and hepatocyte apoptosis index compared to sham in both seasons, with greater elevations in spring squirrels. In addition, liver congestion increased after IR to a similar degree in spring and hibernating groups. Microvesicular steatosis was not affected by IR within the same season but was greater in sham squirrels in both seasons. Plasma IL-6 increased ~twofold in hibernators pre-treated with a biliverdin synthesis inhibitor (SnPP) prior to IR, but was not altered by IR in untreated squirrels. The results show that hibernation provides protection to ground squirrel livers subject to warm IR. Further research is needed to clarify mechanisms responsible for endogenous protection of liver tissue under ischemic stress. [ABSTRACT FROM AUTHOR]

Purpose: The objective of this study is to report a case of a 65-year-old woman who presented with pallor and pain of her left arm secondary to a true arterial brachial aneurysm, which was successfully treated with saphenous vein bypass and embolization of the aneurysm sac. A review of the literature is also presented. Case report: A 65-year-old woman presented with an acute onset of pallor and pain of her left forearm, and hand. On physical examination, there was a pulsatile mass at the forearm. A doppler ultrasound showed a fusiform aneurysmal dilatation of the brachial artery of 23 mm of diameter. A dynamic contrast-enhanced MRI angiogram confirmed a fusiform dilation of the distal brachial artery. The patient was scheduled for open repair. A fusiform 20 x 60 mm aneurysm of the distal brachial artery extending to the cubital fossa was found and a brachial artery to radial and ulnar arteries bypass with interposed reverse right saphenous vein was created. Embolization of the aneurysm sac was performed using Gelita-spon ® (Gelita Medical, Eberbach, Germany). A final angiogram showed an adequate perfusion through the bypass to the hand, and no contrast in the aneurysmal sac. Postoperative course was uneventful with discharge on the fourth postoperative day. Conclusion: Revascularization with autologous saphenous vein graft and exclusion of the aneurysm with local embolization is a good treatment alternative in a patient with symptomatic brachial aneurysm with distal embolization. [ABSTRACT FROM AUTHOR]

Purpose: Bronchoalveolar lavage and transbronchial biopsy can be a useful tool in the evaluation of interstitial lung disease (ILD), but patient selection for this procedure remains poorly defined. Determining clinical characteristics that help with patient selection for bronchoscopy may improve confidence of ILD classification while limiting potential adverse outcomes associated with surgical lung biopsy. The purpose of this study is to identify factors that were associated with change in multidisciplinary ILD diagnosis (MDD) before and after incorporation of BAL and TBBx data. Methods: We conducted a retrospective cohort study of ILD patients at a single center who underwent bronchoscopy in the diagnostic workup of ILD. We performed sequential MDD both pre- and post-bronchoscopy to calculate the frequency of change in diagnosis after incorporating information from BAL and TBBx and identify features associated with change in diagnosis. Results: 245 patients were included in the study. Bronchoscopy led to a change in diagnosis in 58 patients (23.7%). The addition of TBBx to BAL increased diagnostic yield from 21.8 to 34.1% (p = 0.027). Identification of antigen, HRCT scan inconsistent with UIP, and absence of a pre-bronchoscopy diagnosis of CTD-ILD or IPAF were associated with a change in diagnosis after bronchoscopy. Conclusion: Our study suggests clinical features that may assist with patient selection for bronchoscopy. We suggest bronchoscopy in patients with identified antigen or an HRCT that is consistent with a non-IPF diagnosis. Appropriate patient selection for bronchoscopy may improve ILD diagnostic confidence and avoid potential complications from more invasive and higher risk procedures. [ABSTRACT FROM AUTHOR]

Background: C3 glomerulopathy (C3G) is defined by dominant glomerular deposition of C3 and minimal or no immunoglobulin, with two subtypes—dense deposit disease (DDD) and C3 glomerulonephritis (C3GN)—distinguished by features on electron microscopy (EM). Given that this rare disease has generally unfavorable yet highly variable outcomes, we sought out to review the histopathology, complement/genetic studies, and renal outcomes of pediatric patients with C3G at our institution. Methods: All native kidney biopsies performed in a single pediatric hospital over a 10-year period were reviewed for features of C3G. Of 589 biopsy reports, we identified 9 patients fulfilling the diagnostic criteria for C3G and retrospectively reviewed their clinical chart and renal biopsy findings. Results: We identified 4 patients with DDD, 4 with C3GN, and 1 indeterminate case, with features of both C3GN and DDD. Five patients were positive for one or more nephritic factors (C3NeF, C4NeF, C5NeF) with 1 patient additionally positive for complement factor H (CFH) autoantibody. Genetic testing done in 5 of the 9 patients failed to identify any causative mutations. Three patients showed progressive renal dysfunction over a mean follow-up period of 33 months. Conclusions: Complement and genetic studies are now routinely recommended for patients with a histopathological diagnosis of C3G. Careful interpretation of these studies and their prognostic and therapeutic implications in conjunction with biopsy findings is needed to further understand the pathophysiology of this rare disease in children. [ABSTRACT FROM AUTHOR]

Introduction: Making the diagnosis of HP is challenging due to a lack of consensus criteria and variability of both pathologic and radiographic findings. The purpose of this retrospective study was to determine the diagnostic utility of the combination of BAL lymphocyte count and TBBX in patients with HP.Methods: We conducted a retrospective cohort study of all patients with a MDD diagnosis of HP at a single center.Results: 155 patients were included in the study. 49% of patients who underwent BAL had a lymphocyte count > 20, 42% had a lymphocyte count > 30, and 34% had lymphocyte count > 40%. The median BAL lymphocyte count was higher in inflammatory HP compared to fibrotic HP. The addition of TBBX to BAL significantly increased the diagnostic yield regardless of the BAL lymphocyte cutoff used. The yield of bronchoscopy with TBBX and BAL when a lymphocyte count > 40% was used as a cutoff was 52%.Conclusions: Our study suggests that the combination of TBBX with BAL significantly increases the likelihood that the procedure will provide adequate additional information to allow a confident MDD diagnosis of HP and may reduce the need for SLB in the diagnostic workup of HP. [ABSTRACT FROM AUTHOR]

Leukocyte-associated Ig-like receptor 1 (LAIR1) is an ITIM-bearing collagen receptor expressed by leukocytes and is implicated in immune suppression. However, using a divalent soluble LAIR1/Fc recombinant protein to block interaction of cell surface LAIR1 with matrix collagen, we found that whereas Th1 responses were enhanced as predicted, Th17 responses were strongly inhibited. Indeed, LAIR1 on both T cells and monocytes was required for optimal Th17 responses to collagen type (Col)V. For pre-existing "natural" Th17 response to ColV, the LAIR1 requirement was absolute, whereas adaptive Th17 and Th1/17 immune responses in both mice and humans were profoundly reduced in the absence of LAIR1. Furthermore, the addition of C1q, a natural LAIR1 ligand, decreased Th1 responses in a dose-dependent manner, but it had no effect on Th17 responses. In IL-17-dependent murine organ transplant models of chronic rejection, LAIR1+/+ but not LAIR1-/- littermates mounted strong fibroproliferative responses. Surface LAIR1 expression was higher on human Th17 cells as compared with Th1 cells, ruling out a receptor deficiency that could account for the differences. We conclude that LAIR1 ligation by its natural ligands favors Th17 cell development, allowing for preferential activity of these cells in collagen-rich environments. The emergence of cryptic self-antigens such as the LAIR1 ligand ColV during ischemia/reperfusion injury and early acute rejection, as well as the tendency of macrophages/monocytes to accumulate in the allograft during chronic rejection, favors Th17 over Th1 development, posing a risk to long-term graft survival. [ABSTRACT FROM AUTHOR]

Purpose: NEO-102 is a novel chimeric IgG1 monoclonal antibody which recognizes a variant form of MUC5AC expressed specifically by human pancreatic and colorectal tumors. Preclinical models have demonstrated encouraging signs of anti-tumor activity through antibody-dependent cell-mediated cytotoxicity.Methods: This is a phase 1, dose-escalation trial of NEO-102 (Ensituximab) for patients with refractory pancreatic and colorectal cancer. The primary objective was to determine safety and tolerability of escalating doses of NEO-102. Secondary objectives were to assess pharmacokinetics, anti-tumor activity and biologic correlates. Patients whose tumors express NPC-1 antigen were eligible. Dose-escalation was performed in a 3 + 3 design at doses of 1.5, 2, 3 and 4 mg/kg.Results: A total of 19 patients (4 pancreatic and 15 colon cancer) were enrolled at participating institutions in the treatment phase. Most common treatment-related adverse events included anemia, fatigue, fevers, chills and flushing. There was no detectable hemolysis. Of twelve patients evaluable for disease response, the response rate at week 8 included 5 patients with stable disease and 8 patients with progressive disease (PD). Treatment-related grade 3/4 hyperbilirubinemia and anemia were observed at 4 mg/m2. Reversible hypoxia at 3 mg/kg was a dose-limiting toxicity. The maximum tolerated dose was established at 3 mg/kg. Of 74 patients who underwent tissue screening, positive NPC-1 expression was 47 % in colon and 59 % in pancreatic cancer.Conclusions: Treatment with the NEO-102, in this first-in-human study, is well tolerated with a manageable safety profile. A maximum tolerated dose of 3 mg/kg has been established. Toxicity profile is typical for this therapeutic class and allows for combination with conventional cytotoxic therapies. [ABSTRACT FROM AUTHOR]

Abstract: We evaluated the immunocompetence of human T cells in humanized NOD-SCID interleukin (IL)–2r-γ-null (hu-NSG) mice bearing a human thymic organoid, after multilineage reconstitution with isogeneic human leukocytes. Delayed type hypersensitivity (DTH) response was assessed by a direct footpad challenge of the immunized hu-NSG host, or by transfer of splenocytes from immunized hu-NSG, along with antigen, into footpads of C.B-17 scid mice (trans vivo [tv] DTH). Both methods revealed cellular immunity to tetanus toxoid (TT) or collagen type V (ColV). Immunohistochemical analysis of the swollen footpads revealed infiltration of human CD45+ cells, including CD3+ T cells, CD68+ macrophages, and murine Ly6G+ neutrophils. We observed a significant correlation between the percentage of circulating human CD4+ cells and the direct DTH swelling response to TT. The tvDTH response to TT was inhibited by anti–interferon-γ, whereas the tvDTH response to collagen V was inhibited by anti–IL-17 antibody, mimicking the cytokine bias of adult human T cells to these antigens. hu-NSG mice were also capable of mounting a B-cell response (primarily IgM) to TT antigen. The activation of either Th1- or Th17-dependent cellular immune response supports the utility of hu-NSG mice as a surrogate model of allograft rejection and autoimmunity. [Copyright &y& Elsevier]

Purpose: To describe a novel technique to repair a juxtarenal abdominal aortic aneurysm (JAAA) after failed endovascular aortic repair (EVAR) with severely kinked anatomy., Technique: We present a patient who underwent an EVAR with a Medtronic Talent device 15 years ago and a proximal cuff extension 3 years earlier for an abdominal aortic aneurysm. Computed tomography (CT) done for a known gastritis showed a 12 cm JAAA, with a migrated endograft and a type Ia endoleak (EL). Endovascular repair was performed, accessing and navigating the aneurysmal sac outside the previous graft. The type I EL was reached and the suprarenal aorta catheterized. A 4-vessel inner-branched EVAR device was deployed in the distal thoracic aorta and their target vessels bridged through femoral access. A distal bifurcated component was deployed and both iliac limbs were extended to the native distal iliac arteries. Completion angiogram as well as early and 12-month CT showed a fully patent straight course branched EVAR with no ELs., Conclusion: Complex aortic reinterventions in the presence of previous EVAR can be performed by choosing a straighter course along and parallel to the previous endograft. Several technical aspects must be considered to successfully perform this type of reinterventions., Clinical Impact: We present a technique of a complex endovascular aortic repair in a failed EVAR with kinked anatomy, navigating through the thrombosed aneurysmal sac, outside the previously placed endograft and thus obtaining a straighter path for a new branched endograft. The novelty lies in a different approach to repair a failed EVAR with a branched graft through an uncommon access on the side of the previous endograft, avoiding repeated displacement or occlusion of the new endograft. We exemplify the feasibility of such a complex procedure and highlight important steps to perform it, whether in the abdominal or even thoracic Aorta., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Tilo Kölbel: Consultant, proctoring, IP, royalties, research, and travel grants with Cook Medical.

Objective: Iliac branch devices (IBDs) have shown good results but there is little evidence for the risk of internal iliac artery (IIA) endoleak, so there are no clear recommendations on the maximum diameter it should be. Based on limited evidence, it was hypothesised that an IIA of ≥ 11 mm in diameter presents an increased risk of type Ic endoleak., Methods: This was a single centre, retrospective case control study. Patients undergoing an IBD with the main trunk of the IIA as the target vessel, between 2015 and 2021, were identified. Two groups were created: those with a main trunk diameter of < 11 mm; and those with a diameter of ≥ 11 mm. Technical success, freedom from type Ic endoleak, and re-intervention rates were compared. A receiver operating characteristic (ROC) curve was performed to show a cutoff IIA diameter value for risk of type Ic endoleak. Multivariate analysis was performed to assess the risk of type Ic endoleak and the presence of calcification, stenosis, and landing zone length in the IIA., Results: There were 182 IBDs identified. The dilated IIA group (54 IBDs) had significantly lower technical success (91% vs. 98.4%; p = .002), lower freedom from type Ic endoleak (77% vs. 97.1% at 24 months; p = .001), and lower freedom from re-interventions (70% vs. 92.4% at 24 months; p = .002). The ROC curve showed that 10.5 mm was the cutoff diameter for type Ic endoleak. Moderate or severe calcification as well as landing zone length < 5 mm also correlated with type Ic endoleak., Conclusion: IBDs have a statistically significantly higher rate of technical failure, lower freedom from type Ic endoleak, and lower freedom from re-intervention when the IIA is ≥ 11 mm in diameter., (Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.)

Introduction: The management of the false lumen (FL) when dealing with aortic dissection is a crucial aspect since inducing its thrombosis is necessary in order to achieve aortic remodeling. One of the pitfalls of endovascular treatment of aortic dissection (AD) is retrograde distal FL perfusion and pressurization, which prevents FL thrombosis and thus aortic remodeling, while being associated with aneurysmal degeneration of the FL and poor long-term outcomes., Areas Covered: Currently, there is no CE/FDA approved device for FL closure, however different techniques and devices have been proposed to overcome this challenge, the most known of which is the Candy Plug (CP). This review aims to describe the CP device, its implantation technique, and the available data in the literature (PubMed, Cochrane, and EMBASE databases; last queried, December 31, 2023)., Expert Opinions: While the treatment of AD remains technically challenging, the use of the CP technique to close any distal FL reperfusion proved to be feasible and safe with excellent rates of both technical and clinical success. Furthermore, recent studies have shown a quick learning curve with this technique.

Objectives: Electrosurgery has been long used in endovascular procedures, with only case reports in the aortic field. Our aim is to present a case series with the use of an electrified wire to perform catheter-based electrosurgery by applying external current through an electrocautery pen., Methods: Single-center retrospective case series of all patients undergoing complex aortic surgery from October 2020 to August 2023, in whom the electrified wire technique was used: (1) Perforation of a dissection flap or left subclavian artery (LSA) in situ endograft fenestration-a 0.014" polytetrafluoroethylene (PTFE) insulated guidewire is detached from the insulation with a scalpel at the end and a cautery pen is here attached with a clamp. A curved tip catheter or sheath is positioned against the aortic flap or the endograft (through a left brachial access in this case) and the wire pushed, crossing the flap by activating the electrocautery pen and (2) slicing a dissection flap ("powered cheese-wire technique")-after same preparation as above, the middle section of the 0.014 guidewire is removed from the PTFE and bent into a V-shape. Once in the aorta, the guidewire crosses from the true lumen (TL) to the false lumen (FL) and a through-and-through access is obtained. Sheaths are positioned against the flap from both sides and moved up or down while the electricity is activated, slicing the flap and communicating both lumens. Technical success and technical-related complications were evaluated., Results: Eleven cases concerning aortic dissections and 1 case of aortic atresia were treated. Four patients presented urgently, whereas the rest were planned procedures. Seven cases underwent perforation of a dissection flap, 2 cases underwent the powered cheese-wire technique, in 2 cases for an LSA in situ fenestration, and in 1 case to cross an aortic atresia at the aortic isthmus. The technique was in all cases successfully applied. No complications related to the technique occurred., Conclusions: The "electrified wire" technique is a feasible and ready-available tool that can be safely used in complex aortic interventions, especially to perforate aortic tissue like dissection flaps or to perform in situ fenestrated repairs by perforation of the endograft fabric., Clinical Impact: The electrified wire technique described herein is a straightforward technique that uses readily available tools to perform electrosurgery. We present its use in complex aortic procedures. However, it could be envisioned for any vascular procedure that requires crossing of the vessel or even prosthetic material. As we have described in this series, when used along with an adequate properative planning, it can be a safe tool of great utility, as has already been demonstarted in the field of the interventional cardiology., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: T.K. is consultant, proctoring, IP, royalties, research, and travel grants with Cook Medical.

Objective: This study aimed to present the early and mid-term outcomes of fenestrated/branched thoracic endovascular aortic repair (f/bTEVAR) for aortic arch pathologies., Background: f/bTEVAR represents a less invasive treatment option for aortic arch diseases. Previous published series showed decreased early mortality and morbidity compared to open repair., Methods: A single-center retrospective analysis of consecutive patients, managed with f/bTEVAR (Cook Medical, Bloomington, IN, USA) between 01.09.11and 30.06.22 was conducted. Primary outcomes were technical success, 30-day mortality and stroke. Data during follow-up and factors affecting early mortality and stroke were analyzed., Results: 209 patients were included (38.3% females; mean age 69.8±3.4years; mean aortic diameter 61±4.7 mm); 14.4% were managed urgently. Fenestrations/scallop configuration was used in 39.7%, branched devices in 55.5% and branch/scallop combination in 4.8%. Landing to zone 0 was performed in 65.5% and in zone 1 in 32.1%. Non-native aortas were used for landing in 39.2%. Technical success was 97.1% and 30-day mortality 9.5%. Strokes were identified in 10%; 5.7% major. Non-native proximal aortic landing zone was an independent protective factor for stroke (P=0.002). Post-operative stroke (P<0.001) and pericardial effusion (P<0.001) were independently related to 30-day mortality. The mean follow-up was 21±8 months. The estimated survival and freedom from reintervention rates were 79.5% (standard error; SE3.1%) and 47.1% (SE 4.3%) at 24months, respectively., Conclusions: f/bTEVAR presented high technical success and acceptable 30-day mortality. Non-native proximal landing zone was related to lower stroke rate. Half of patients needed a reintervention within the 24-month follow-up., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Post-infectious glomerulonephritis (PIGN) is an immune complex mediated glomerular injury occurring because of an infection, most commonly with group A beta-hemolytic streptococcus in children. C3 glomerulopathy (C3G) is a distinct clinicopathological entity occurring secondary to dysregulation of alternate complement pathway encompassing both C3 glomerulonephritis (C3GN) and dense deposit disease (DDD). While most patients with PIGN attain complete remission with normalized complement levels by 6-8 weeks after presentation, patients with C3G continue to have hypocomplementemia with high rates of progressive kidney disease. Here, we report a patient diagnosed with dense deposit disease after his initial presentation with PIGN three years prior. While current literature continues to explore the overlapping and distinguishing features of PIGN and C3G, including how underlying defects in the alternate complement pathway may commonly contribute to both diseases, this case further exemplifies the importance of recognizing the clinico-pathogenic features of PIGN and C3G in pediatric patients with glomerulonephritis., (© 2023. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Background: Fenestrated and branched endovascular aortic repair (F/BEVAR) of thoracoabdominal aortic aneurysms (TAAAs) has shown high technical success and low early mortality rates. Aneurysm extent has been reported as a factor affecting outcomes. This study aimed to assess the early and midterm follow-up outcomes of patients managed by F/BEVAR for types I through III TAAAs., Methods: A single-center retrospective analysis was conducted, including data from consecutive, elective and urgent (symptomatic and ruptured cases), patients treated for types I through III TAAAs, between October 1, 2011, and October 1, 2022, using F/BEVAR. Degenerative and postdissection TAAAs were included. Patients received prophylactic cerebrospinal fluid drainage (CSFD), except those under therapeutic anticoagulation, those who were hemodynamically unstable, or those with failed CSFD application. When an initial thoracic endovascular aortic repair was performed, as part of a staged procedure, no CSFD was used. Later stages and nonstaged procedures were performed under CSFD. Thirty-day mortality and major adverse events (MAEs) were analyzed. Kaplan-Meier estimates were used for follow-up outcomes., Results: F/BEVAR for types I through III TAAAs was performed in 209 patients (56.9% males; mean age, 69.6 ± 3.2 years; mean aneurysm diameter, 65.2 ± 6.2 mm); 29.2% type I, 57.9% type II, and 12.9% type III. Urgent repair was performed in 26.7% of patients (56 cases; 23 ruptured and 33 symptomatic cases) and 153 were treated electively. Thirty-two patients (15.3%) were classified as American Society of Anesthesiologists (ASA) class IV. CSFD was used in 91% and staged thoracic endovascular aortic repair was performed in 51.2% of patients. Technical success was 93.8% (96.7% in elective vs 94.6% in urgent cases; P = .92). Thirty-day mortality was 11.0% (4.6% in elective vs 28.5% in urgent cases; P < .001) and MAEs were recorded in 17.2% of cases (7.8% in elective vs 42.8% in urgent cases; P < .001). Spinal cord ischemia rate was 20.5% (17.6% in elective vs 28.7% in urgent cases; P = .08), whereas 2.9% of patients presented paraplegia (1.3% in elective and 7.1% in urgent cases; P = .03). The mean follow-up was 16 ± 5 months. Survival was 75.0% (standard error, 4.0%) and freedom from reintervention was 73.3% (standard error, 4.4%) at 36 months. ASA IV and urgent repair were detected as independent factors related to early mortality and MAE, whereas ruptured aneurysm status was related to spinal cord ischemia evolution., Conclusions: Endovascular repair for types I through III TAAAs provides encouraging early outcomes in terms of mortality, MAE, and paraplegia, especially in an elective setting. Setting of repair and baseline ASA score should be taken into consideration during decision-making., Competing Interests: Disclosures N.T. is a proctor for Cook Medical and has intellectual property with Cook Medical and Bentley receiving institutional fees. T.K. is a consultant and proctor for and has intellectual property with Cook Medical, receiving royalties, speaking fees, and research, travel, and educational grants. The remaining authors report no conflicts., (Copyright © 2023 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Infective native aortic aneurysms (INAAs), formerly called mycotic aneurysms, remain an uncommon disease with significant heterogeneity among cases; hence, there is lack of solid evidence to opt for the best treatment strategy. The present study aims to describe a 20-year experience at a single institution treating this uncommon condition., Methods: Retrospective study of all patients treated for INAA at a single academic hospital in Santiago, Chile, between 2002 and 2022. Clinical characteristics are described, as well as operative outcomes per type of treatment. Nonparametric Mann-Whitney U-test or Kruskal-Wallis tests were performed when appropriate, and results were reported as median and ranges. Survival at given timeframes was determined by a Kaplan-Meier curve, with analysis performed through a Cox regression model., Results: During the study period, 1,798 patients underwent aortic procedures at our center, of which 35 (1.9%) were treated for INAA. Of them, 25 (71.4%) were male. One patient had 2 INAAs. Median age was 69.5 years (range: 34-89 years). Of the 36 INAAs, the most frequent location was the abdominal and thoracic aorta in 20 (55.5%) and 11 (30.5%) cases, respectively, followed by the iliac arteries in 4 (11.1%) cases. One (2.7%) patient presented a thoracoabdominal INAA. Overall, endovascular treatment associated with long-term antibiotics was used in 20 (57.1%) patients: 4 of them underwent hybrid treatment. Fifteen (42.8%) patients underwent direct aortic debridement followed by in situ or extra anatomic revascularization. There was a significant difference in age between both treatment strategies (a median of 76.5 years for endovascular versus a median of 57 years for open, P = 0.011). The median hospital stay was 15 days (range: 2-70 days). The early complications rate (<30 postoperative days) was 20% (n = 7). Early mortality rate (inhospital or before postoperative 30 days) was 14.2% (n = 5). Median follow-up was 33 months (range: 6-216 months). The overall survival rates at 1, 3, and 5 years were 69.9% (standard error [SE] 8.0), 61.7% (SE 9.8), and 50.9% (SE 11.8), respectively. Five-year survival rate of patients undergoing endovascular treatment compared with open approach was 45.9% (SE 15.1) versus 80.0% (SE 17.8), respectively (P = 0.431). There were no significant differences in survival between open and endovascular treatment, hazard ratio 3.58 (confidence interval 95%: 0.185-1.968, SE ± 0.45 P = 0.454)., Conclusions: Patients treated by endovascular approach were older than patients treated by open approach. Even though, the open group had a higher 5-year survival rate than the endovascular group, not statically significance differences were found between treatments., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: To report on the outcomes of patients undergoing an iliac branch device implantation after previous open or endovascular aorto-biliac repair, using exclusively femoral access for catheterization and delivery of the covering stent to the hypogastric artery., Methods: Single-center retrospective study in which all patients in whom an iliac branch device was implanted after previous open or endovascular aorto-biliac repair were identified. Patients in whom the hypogastric artery catheterization and delivery of the bridging cover stent were achieved via exclusive femoral access were included. Different techniques were used based on surgeon preference. Technical success and access-related complications, as well as iliac branch device endoleak or occlusions during follow-up, were evaluated., Results: From 2015 to 2021, 28 patients with a prior open or endovascular aorto-biliac repair underwent 34 iliac branch device implantations. Most (71%) had juxtarenal or thoracoabdominal aortic aneurysms, 82% had common iliac artery aneurysms, and 25% had hypogastric artery aneurysms. Bilateral iliac branch device implantations were performed in 21% of the patients, and in 26% of cases, landing in the superior gluteal artery was obtained. An "up-and-over" technique from the contralateral groin was used in 65% of the cases, and a steerable sheath in 35%. Technical success was 94%, with no complications related to access or technique to catheterize and deliver the stents in the hypogastric artery. The cohort had 20% of major complications, with 3 perioperative deaths. Kaplan-Meier estimated an iliac branch device freedom from occlusion and endoleak was 92% and 83% at 2 years., Conclusions: The implantation of an iliac branch device over previous aortic or open endografts involving the aortic bifurcation is feasible and safe. We suggest using a femoral approach as the primary access of choice., Clinical Impact: In this study we present 28 patients with previous aortoiliac grafts in which iliac branch devices were performed as a subsequent step.We demonstrated the feasibility of the technique despite the difficulty of crossing a neobifurcation, with a steep angle, without complications associated with the technique. Based on our experience, we recommend transfemoral access as the first option for bypassing the hypogastric artery stent, preserving upper extremity access and its possible complications., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: T.K.—consultant, proctoring, IP, royalties, research, and travel grants with Cook Medical.

We describe the feasibility of covered endovascular reconstruction of the aortic bifurcation (CERAB) through a single femoral access and a steerable sheath. We present the technique, which we used for a patient with severe aortoiliac calcification and bilateral involvement of the common femoral artery. The patient underwent endarterectomy of the left common femoral artery plus CERAB with an aortic stent graft and bilateral covered stents for the common iliac artery with kissing dilatation with a steerable sheath using only left femoral access. CERAB can be performed using unilateral access with the aid of a steerable sheath, reducing the potential for access site complications., Competing Interests: T.K. receives consulting, proctoring, and intellectual property fees, royalties, and research and travel grants from 10.13039/100010479Cook Medical Inc. J.T., E.B., F.R., G.P., and S.C. have no conflicts of interest., (© 2023 The Authors.)

Introduction: Endovascular repair using off-the-shelf endografts is a viable solution in patients with ruptured or symptomatic complex aortic aneurysms. This analysis aimed to present the peri-operative and follow-up outcomes in urgent and emergent cases managed with the t-Branch multibranched thoracoabdominal endograft., Methods: Prospectively collected data from all consecutive urgent and emergent cases managed in two aortic centers between January 1st, 2014, to November 30th, 2022, using the t-Branch device (Cook Medical Inc., Bjaeverskov, Denmark) were analyzed. Patients presenting with ruptured aortic complex aneurysms were characterized as emergent and patients with aneurysms >90 mm of diameter, or symptomatic aneurysms were characterized as urgent. Technical success, 30-day mortality, major adverse events (MAE) and spinal cord ischemia (SCI) rates were assessed., Results: 225 patients (36.5% females, 72.5 ± 2.8 years) were included; 73.0% were urgent. The mean aneurysm diameter was 109 ± 3.9 mm and 44.4% were type I-III TAAAs. Females ( p  = .03), para-renal aneurysms ( p  = .02) and ASA score IV ( p  < .001) were more common in emergent cases. Technical success was 97.8%. Thirty-day mortality and MAE rates were 17.8% and 30.6%, respectively. SCI rate was 14.7%, (4.8% paraplegia rate) with 22.2% of patients receiving prophylactic cerebrospinal drainage. Thirty-day mortality (13.3% vs. 26.7%, p  = .04) and MAE (26.0% vs. 43.0%, p  = .02) were more common among emergent cases while technical success (97.6% vs. 98.3%, p  = .9), and SCI (13.3% vs. 18.3%, p  = .4) were similar. Survival at 12-months was 83.5% (SE 5.9%) for the urgent and 77.1% (SE 8.2%) for the emergent group (log rank, p  = 0.96)., Conclusion: T-Branch represents an effective and safe solution for the management of urgent and emergent cases with complex aortic aneurysms, with high technical success, promising early mortality and SCI rates., Competing Interests: TK is a consultant and proctor for and has intellectual property with Cook Medical, receiving royalties, speaking fees, and research, travel, and educational grants. TJ is a consultant and proctor for HammerMed—Polish distributor of Cook Medical, receiving royalties, speaking fees, and travel grants. All authors have completed the ICMJE uniform disclosure form and declare no support from any organization for the submitted work; no financial relationships with any organizations that might have an interest in the submitted work; no other relationships or activities that could appear to have influenced the submitted work. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (© 2023 Nana, Spanos, Jakimowicz, Torrealba, Jama, Panuccio, Rohlffs and Kölbel.)

Objective: This study aims to report two cases of symptomatic extrinsic compression of the inferior vena cava and left iliac vein caused by vertebral osteophytes., Methods: We present two case reports of extrinsic venous compression by vertebral osteophytes. Both cases were endovascularly treated, with a successful outcome. A review of the literature of this unusual condition is also presented., Results: The first patient is an 80-year-old woman who presented to the vascular surgery clinic with bilateral lower extremity edema and pain. A computed-tomography angiography (CTA) revealed extrinsic compression of the inferior vena cava from enlarged osteophytes. Venography and intravascular ultrasound were performed, confirming the diagnosis. A self-expanding venous stent was successfully deployed in the inferior vena cava relieving the extrinsic compression. The edema resolved the following day and was discharged without complications. The second patient is a 61-year-old male that presented to the emergency department with a left iliofemoral deep venous thrombosis. CTA showed left iliac vein compression by a lumbar osteophyte. Percutaneous thrombectomy was successfully achieved and an expanding stent was deployed covering the entire lesion. One month after the procedure the patient died from COVID-19-associated respiratory failure., Conclusion: Osteophytes must be considered when dealing with extrinsic venous compression, especially in elderly people.

Purpose: Sarcopenia has been identified as an independent predictor of mortality in patients with infrarenal abdominal aortic aneurysm and may also affect outcomes in patients with complex aortic pathologies. The aim of this study was to assess sarcopenia, combined with the American Society of Anesthesiologists (ASA) score, as predictors for spinal cord ischemia (SCI) in patients treated with the t-Branch off-the-shelf device., Materials and Methods: A single-center retrospective observational study was conducted including elective and urgent patients managed with the t-Branch device (Cook Medical, Bjaeverskov, Denmark) between January 1, 2018, and September 30, 2020. Data were collected according to the STrengthening the Reporting of OBservational studies in Epidemiology (STROBE) statement. The psoas muscle area (cm 2 ) and attenuation (Hounsfield units, HU) were measured in the arterial phase of the pre-operative computed tomography angiography for each patient. The lean psoas muscle area (LPMA) was used to stratify patients into 3 groups, and further stratification was performed with a combination of the ASA score and LPMA., Results: Eighty patients were included (mean age at 71±9 years; 62.5% males). Thoracoabdominal aneurysms were managed in 72.5% of cases (42.5% for type I-III). Thirty-seven (46%) were treated urgently. Eleven patients died within 30 days (14%). Twelve patients (15%) presented SCI of any severity. Among the LPMA groups, the only statistically significant difference was recorded in age; group 3 was older compared with groups 1 and 2 (67.1 years vs 72.1 years vs 73.5 years, p=0.004). After ASA combined LPMA categorization, 28 patients were considered as low risk, 16 as moderate risk, and 36 as high risk. A statistically significant difference was recorded in terms of SCI (3.5% [1/28] in low risk vs 12.5% [2/16] in moderate risk vs 25% [9/36] in high risk, p=0.049). Multivariate analysis showed that moderate-risk patients were at risk to evolve to SCI (p=0.04)., Conclusions: Low-risk patients, with ASA score I-II or LPMA>350cm 2 HU, are at lower risk for developing SCI after BEVAR using the t-Branch device. Patients' stratification according to the combination of ASA score and psoas muscle area and attenuation may identify a group at higher risk of SCI after branched endovascular aneurysm repair., Clinical Impact: Sarcopenia has been identified as a factor of increased mortality in patients managed for aortic aneurysm repair. However, significant heterogeneity has been recorded in the tools assessing its presence. In this analysis, an already used method, combining the ASA score and psoas muscle area and attenuation, has been used to assess the impact of sarcopenia in patients managed with the t-branch device. This analysis showed that patients at low risk, with an ASA score I-II or LPMA>350cm2HU were at lower risk to evolve spinal cord ischemia. Along this line, sarcopenia may be a valuable marker for the prediction of perioperative adverse events , other than mortality, in patients managed using complex endovascular repair.

Purpose: The purpose of this study was to describe the use of a wire and catheters embedded with optical fiber (Fiber Optic RealShape [FORS]) to catheterize tortuous target vessels avoiding radiation., Technique: A virtual biplane vies was simulated coupling traditional x-ray system, preoperative CT scan, and FORS system to treat an isolated hypogastric aneurysm. Despite the complex anatomy, catheterization of all target vessels was possible in 12 minutes with 19 seconds of fluoroscopy time (Radiation Exposure 3.8 mGy×cm 2 ). A minimal invasive endovascular exclusion of the aneurysm was achieved through selective coil-embolization of the iliolumbar artery and implantation of balloon expandable covered stents, thus preserving the perfusion of the superior gluteal artery., Conclusion: FORS guidance allowed catheterization of a target vessel with challenging anatomy with a low radiation exposure.

Purpose: To describe the use of physician-modified endograft (PMEG) with the exclusive use of inner branches or in combination with fenestrations for the urgent treatment of complex aortic aneurysms., Technique: We present two urgent cases. A patient with a 6.8 cm saccular juxtarenal aneurysm and another patient with a contained rupture of the thoracoabdominal aorta right above the celiac trunk (CT). In both cases, a Cook Zenith TX2 thoracic endograft was back-table modified, in the first case by adding three fenestrations and one inner branch for the left renal artery to improve sealing due to its partial involvement in the aneurysm and, in the second case, with the use of two inner branches for the CT and superior mesenteric artery. Both procedures were successful, with uneventful postoperative courses and complete aneurysm exclusion on postoperative CT angiography., Conclusion: Use of PMEGs with inner branches is feasible for urgent repair in complex aortic anatomy.

Objective: The objective of this study was to evaluate renal function and renal parenchymal length changes secondary to the coverage or preservation of accessory renal arteries (ARAs) in complex aortic repair., Methods: This was a single-center retrospective study identifying all patients undergoing fenestrated or branched endovascular aortic repair (f-b EVAR) who presented with ARAs. Two groups were created, a preserved ARA group, with incorporation of the vessel as a dedicated fenestration or branch in the endograft plan, and a non-preserved ARA group, without incorporation of them. Early >30% decline of glomerular filtration rate (GFR), kidney infarcts, and endoleaks were evaluated. Mid-term results with freedom from kidney shrinkage (defined as length decrease >10%) at follow-up, freedom from GFR decrease >30%, or need for postoperative dialysis at follow-up were also analyzed. Primary assisted patency of incorporated ARAs was calculated., Results: From 2011 through 2020, 145 patients undergoing complex aortic repair presented with an ARA. After excluding ruptured aneurysms, 33 patients had the ARA preserved with their incorporation into the stent graft (preserved ARA group), and 99 did not have preservation of them (not-preserved ARA group). There were no statistical differences in demographics or type of aneurysm. Patients in the ARA-preserved group had more ARAs (median of two per patient vs one in the non-preserved ARA group; P = .01) and bigger ARAs (median 4 vs 3 mm in the non-preserved ARA group; P = .001). Early postoperative worsening >30% of GFR (23% vs 6%; P = .03) as well as postoperative renal infarction (57% vs 6%; P = .001) and ARA-related endoleaks (20% vs 0%; P = .01) were statistically higher for the not-preserved ARA group. Mid-term kidney length showed significant shrinkage in the not-preserved ARA group compared with the ARA preserved group (9.7% vs 0%; P = .001). Freedom from >30% GFR decline at 2 years was significantly higher for the preserved ARA group (83% vs 47%; P = .01).Two-year primary assisted patency of incorporated ARA was 94%., Conclusions: Complex aortic repair incorporation of ARA is feasible, with low complications and good primary assisted patency at 2 years. It leads to less postoperative early renal dysfunction as well as higher freedom for mid-term renal disfunction and kidney shrinkage., (Copyright © 2022 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to evaluate early and mid-term results of non-standard management of the supraaortic target vessels with the use of the inner branch arch endograft in a single high-volume center., Material and Methods: A single-center retrospective study including all patients undergoing implantation of an inner branch arch endograft from December 2012 to March 2021, who presented a non-standard management of the supraaortic target vessels (any bypass other than a left carotid-subclavian or landing in a dissected target vessel). Technical success, mortality, reinterventions, endoleak (EL), and aortic remodeling at follow-up were analyzed., Results: Twenty-four patients were included. In 17 (71%) cases, the non-standard management was related to innominate artery (IA) compromise (12 with IA dissection, 2 with short IA, 2 with short proximal aortic landing zone that required occlusion of IA, 1 with occluded IA after open arch repair). Two (8%) cases were related to an aberrant right subclavian artery (RSA), 1 patient (4%) due to the concomitant presence of a left vertebral artery (LVA) arising from the arch and an occluded left subclavian artery (LSA), and another patient presented with an occluded LSA distal to a dominant vertebral artery. Three (13%) cases were exclusively related to management in patients with genetic aortic syndromes. Twenty (83%) patients had a previous type A aortic dissection. Ten (42%) patients presented a thoracic or thoracoabdominal aortic aneurysm and 8 (33%) patients an arch aneurysm, 6 of them associated to false lumen (FL) perfusion. There were 2 (8%) perioperative minor strokes, and 1 patient with perioperative mortality. Seven patients presented an early type I endoleak, all resolved at follow-up. Seven patients required reinterventions during follow-up (7 reinterventions related to continuous false lumen perfusion, 3 related to Type Ia endoleak, 2 related to surgical bypass). All patients who presented with FL perfusion had complete FL thrombosis at follow-up. No patient presented aneurysm growth at follow-up., Conclusions: The use of the inner branch arch endograft with a non-standard management of the supraaortic target vessels is a possible option. Despite a high reintervention rate, regression or stability of the aneurysmal diameter was achieved in all the patients with follow-up.

Purpose: The purpose of this article is to study 1-year results of Zenith branch iliac endovascular graft (ZBIS) with the off-label use of a 13 mm spiral Z limb to connect to the aortic main body., Materials and Methods: A retrospective review from 2015 to 2019 of all iliac branch devices (IBDs) was performed at 1 institution that were connected to an aortic main body with a 13 mm spiral Z limb and had at least 1-year follow-up with computed tomography (CT). Primary endpoints are freedom from ZBIS separation from the connection limb, endoleak (EL), or reintervention at 1 year. Secondary endpoints are primary and secondary ZBIS patency, presence of any EL, and aortic reinterventions., Results: Of 149 IBDs implanted in this period, 45 ZBIS in 35 patients were connected with a 13 mm limb and had a 1-year CT; 97% of patients had common iliac artery (CIA) aneurysms, 7% of patients had hypogastric artery (HA) aneurysms, and 30% of patients had bilateral ZBIS implantation. Technical success was 98%. In 84% of cases, the Advanta V12 was used as the HA mating stent; 56% of patients had an EL, mostly type II, which resolved spontaneously in 70% at 1 year, and 9% of ZBIS required reinterventions at 1 year (2 for thrombosis, 2 for type Ic EL from HA mating stent). One-year ZBIS primary patency and secondary patency were 96% and 100%, respectively. No EL was noted to be related to the 13 mm connection limb. No migration or separation of the devices occurred., Conclusions: The use of 13 mm spiral Z limb to connect a ZBIS with the main body in our series yields a high technical success rate and good 12-month outcomes without device separation or migration.