scholarly journals Follow-up Findings in a Turkish Girl with Pseudohypoparathyroidism Type Ia Caused by a Novel Heterozygous Mutation in the GNAS Gene

2017 ◽  
Vol 9 (1) ◽  
pp. 74-79
Author(s):  
Sezgin Şahin ◽  
Olaf Hiort ◽  
Susanne Thiele ◽  
Olcay Evliyaoğlu ◽  
Beyhan Tüysüz
Keyword(s):  
Heterozygous Mutation ◽  
Gnas Gene ◽  
Type Ia

scholarly journals New optically identified supernova remnants in the Large Magellanic Cloud

2020 ◽  
Vol 500 (2) ◽  
pp. 2336-2358
Author(s):  
Miranda Yew ◽  
Miroslav D Filipović ◽  
Milorad Stupar ◽  
Sean D Points ◽  
Manami Sasaki ◽  
...  
Keyword(s):  
Supernova Remnants ◽  
Supernova Remnant ◽  
Narrow Band Imaging ◽  
Large Magellanic Cloud ◽  
Magellanic Cloud ◽  
Main Body ◽  
Found Objects ◽  
Type Ia ◽  
Optical Sample

ABSTRACT We present a new optical sample of three Supernova Remnants (SNRs) and 16 Supernova Remnant (SNR) candidates in the Large Magellanic Cloud (LMC). These objects were originally selected using deep H α, [S ii], and [O iii] narrow-band imaging. Most of the newly found objects are located in less dense regions, near or around the edges of the LMC’s main body. Together with previously suggested MCSNR J0541–6659, we confirm the SNR nature for two additional new objects: MCSNR J0522–6740 and MCSNR J0542–7104. Spectroscopic follow-up observations for 12 of the LMC objects confirm high [S ii]/H α emission-line ratios ranging from 0.5 to 1.1. We consider the candidate J0509–6402 to be a special example of the remnant of a possible type Ia Supernova (SN) which is situated some 2° (∼1.75 kpc) north from the main body of the LMC. We also find that the SNR candidates in our sample are significantly larger in size than the currently known LMC SNRs by a factor of ∼2. This could potentially imply that we are discovering a previously unknown but predicted, older class of large LMC SNRs that are only visible optically. Finally, we suggest that most of these LMC SNRs are residing in a very rarefied environment towards the end of their evolutionary span where they become less visible to radio and X-ray telescopes.

Back-Table Modified Stent-Graft for Endovascular Repair of Ascending Aorta

2021 ◽  
pp. 152660282110282
Author(s):  
Juan Shi ◽  
Ligang Liu ◽  
Xiang Wei ◽  
Mingjia Ma
Keyword(s):  
Endovascular Repair ◽  
Survival Rates ◽  
Ascending Aorta ◽  
Cerebellar Infarction ◽  
Type Ia ◽  
Risk Patients ◽  
Aorta Replacement ◽  
Aortic Dissections ◽  
Event Rates

Objectives To investigate the effectiveness of modified stent-grafts (SGs) for the management of ascending aortic pathologies. Materials and Methods From January 2015 to December 2019, 31 individuals were treated by ascending aortic endovascular repair with a back-table modified SG for acute (n=4) or chronic (n=1) type A aortic dissections, penetrating aortic ulcers (n=18), pseudoaneurysms (n=2), anastomotic fistula (n=1), and endoleaks after thoracic endovascular aortic repair (TEVAR) (n=5). The commercially available thoracic aortic SGs were modified with a fenestration or truncation technique on the back-table according to aortography during the operation. Results The 30-day mortality and aorta-related mortality rates were 12.9% and 6.5%, respectively. There were 2 strokes, 3 respiratory insufficiencies, and 6 endoleaks during hospitalization. During a mean follow-up of 28.8±16.6 months, the overall survival rates at 1 year and 3 years were both 80.6%. Free from adverse event rates at 1 year and 3 years were 88.9% and 84.7%, respectively. There were 2 deaths during follow-up: One patient died of cachexia 1 month after discharge, and the other patient died of acute myocardial infarction 3 months after discharge. One patient with a pseudoaneurysm underwent open ascending aorta replacement 3 months after discharge for a type Ia endoleak. Another patient suffered from cerebellar infarction 17 months after discharge. Conclusion The modified SG for endovascular repair of the ascending aorta is a practicable alternative and presents acceptable outcomes in high-risk patients.

Treatment of a type Ia endoleak following EVAR using a custom-made inner branch device.

Vascular ◽  
2021 ◽  
pp. 170853812110627
Author(s):  
Gino Gemayel GG ◽  
Michel Montessuit MM ◽  
Anouche Gemayel GA
Keyword(s):  
Abdominal Aortic Aneurysm ◽  
Open Surgery ◽  
Type I ◽  
Novel Technology ◽  
Abdominal Aortic ◽  
Custom Made ◽  
Type Ia ◽  
Type I Endoleak ◽  
Aortic Cuff

Objectives We represent two cases of late proximal type I endoleak following EVAR with aneurysm expansion that were treated with a custom-made graft with inner branches. Methods Two patients of 87 and 82 years old were operated by EVAR 6 and 8 years ago for abdominal aortic aneurysm. Both had proximal type I endoleak with aneurysm sac expansion. Open surgery had a high risk, and a proximal aortic extension with a simple aortic cuff was not possible neither because previous EVAR grafts were already at the level of the renal arteries. A custom-made endograft with inner branches was planned as a fenestrated graft was not technically possible. Results We successfully treated both patients using a custom-made graft with four inner branches from Jotec (Cryolife, Kennesaw, GA). Three months’ follow-up CT scan did not show any endoleaks. All target vessels were patent with good conformability of the bridging stents. Conclusion The treatment of proximal type I endoleak using inner branches’ endografts is feasible. This novel technology might broaden the indications for complex aortic repair in a group of patients where fenestrated endografts are not possible.

Comparing polymer-filled versus self-expanding endografts in Chinese patients

2018 ◽  
Vol 26 (9) ◽  
pp. 667-676
Author(s):  
Yuk Law ◽  
Yiu Che Chan ◽  
Stephen Wing-Keung Cheng
Keyword(s):  
Endovascular Aneurysm Repair ◽  
Aortic Aneurysms ◽  
The Self ◽  
Chinese Patients ◽  
Type Ii ◽  
Mortality And Morbidity ◽  
Type Ia ◽  
One Year ◽  
Aneurysm Repair

Introduction We performed a single-center nonrandomized study on patients who underwent endovascular aneurysm repair using polymer-filled or other self-expanding endografts. Methods Consecutive patients with asymptomatic infrarenal abdominal aortic aneurysms who underwent endovascular repair were retrospectively reviewed. They were divided into a polymer-filled ( n = 20) or self-expanding group ( n = 42). Baseline characteristics, operative mortality and morbidity, and follow-up data were compared. Results Aneurysm diameter, neck and iliac morphologies did not differ between the two groups. Technical success was 100%. The 30-day mortality was 0% and 2.4% in the polymer-filled and self-expanding group, respectively. At a mean follow-up of 17 months, the changes in sac size were −2.1 mm and −5.1 mm ( p = 0.144) at one year, and −3.5 mm and −7.7 mm ( p = 0.287) at 2 years in the polymer-filled and self-expanding group, respectively. The polymer-filled group had 7 (35%) type II endoleaks, and the self-expanding group had 1 (2.4%) type Ia and 13 (31%) type II endoleaks. Neck diameter remained stable in the polymer-filled stent-grafts whereas there was progressive neck degeneration in the self-expanding group. The rates of reintervention and overall survival were similar in both groups. The presence of an endoleak was the only predictor of non-regression of the aneurysm (odds ratio = 17.00, 95% confidence interval: 4.46–64.88, p < 0.001). Conclusion Polymer-filled endografts had similar safety, effectiveness, and durability to other self-expanding endografts. The major advantage is the small iliofemoral access. They also have the potential long-term benefit of a more stable neck.

Endovascular Aneurysm Sealing (EVAS) and Chimney EVAS in the Treatment of Failed Endovascular Aneurysm Repairs

2016 ◽  
Vol 24 (1) ◽  
pp. 115-120 ◽  
Author(s):  
Marwan Youssef ◽  
Sebastian Zerwes ◽  
Rudolf Jakob ◽  
Oroa Salem ◽  
Fritz Dünschede ◽  
...  
Keyword(s):  
Endovascular Aneurysm Repair ◽  
Multiple Organ ◽  
Aortic Aneurysms ◽  
Technical Success ◽  
Graft Thrombosis ◽  
Type Ia ◽  
Aneurysm Repair ◽  
Chimney Graft ◽  
Bifurcated Graft

Purpose: To assess the technical success and clinical outcome of reinterventions using the Nellix Endovascular Aneurysm Sealing (EVAS) System to treat complications after endovascular aneurysm repair (EVAR). Methods: Fifteen consecutive patients (mean age 79 years; 14 men) with prior EVAR were treated with EVAS between March 2014 and December 2015 at 2 institutions. The failed prior EVARs included 13 bifurcated endografts, 1 bifurcated graft plus fenestrated cuff, and 1 tube endograft. Endoleaks were the predominant indications: type Ia in 10 and type III in 5 (3 type IIIa and 2 type IIIb). All patients presented with progressive aortic aneurysms (median 7.85-cm diameter; range 6.5–11). Eight patients were treated on an urgent or emergency basis (6 symptomatic aneurysms and 2 contained ruptures). All patients underwent Nellix relining of the failed stent-graft; 10 had chimney (Ch) procedures in combination with EVAS (chEVAS) because the proximal landing zones were inadequate. Results: Technical success was 100%. All endoleaks were successfully sealed, and no additional intervention was required. No further endoleak after EVAS or chEVAS was recorded. Endobag protrusion occurred in 1 case without sequelae. One elderly patient with ruptured aneurysm died from multiple organ failure 2 months postoperatively. One renal artery guidewire injury led to nephrectomy because of active bleeding. No reinterventions, aneurysm-related mortalities, graft thrombosis, endoleaks, or chimney graft occlusions were observed during a median follow-up of 8 months (range 3–24). Conclusion: The present preliminary experience demonstrates that the use of EVAS/chEVAS is feasible for treatment of failed EVAR. This technique may be used as bailout or an alternative treatment when other established methods are infeasible or not available.

Early Blue Excess from the Type Ia Supernova 2017cbv

2017 ◽  
Vol 14 (S339) ◽  
pp. 47-49
Author(s):  
G. Hosseinzadeh
Keyword(s):  
Light Curve ◽  
Standard Type ◽  
Circumstellar Material ◽  
Type Ia Supernova ◽  
Unusual Distribution ◽  
Photometric Observations ◽  
Type Ia ◽  
Optical Light Curve ◽  
Ia Supernovae

AbstractThis paper presented very early, high-cadence photometric observations of the nearby Type Ia SN 2017cbv. The light-curve is unique in that during the first five days of observations it has a blue bump in the U, B, and g bands which is clearly resolved by virtue of our photometric cadence of 5.7 hr during that time span. We modelled the light-curve as the combination of an early shock of the supernova ejecta against a non-degenerate companion star plus a standard Type Ia supernova component. Our best-fit model suggested the presence of a subgiant star 56 R⊙ from the exploding white dwarf, although that number is highly model-dependent. While the model matches the optical light-curve well, it over-predicts the flux expected in the ultraviolet bands. That may indicate that the shock is not a blackbody, perhaps because of line blanketing in the UV. Alternatively, it could point to another physical explanation for the optical blue bump, such as interaction with circumstellar material or an unusual distribution of the element Ni. Early optical spectra of SN 2017cbv show strong carbon absorption as far as day –13 with respect to maximum light, suggesting that the progenitor system contained a significant amount of unburnt material. These results for SN 2017cbv illustrate the power of early discovery and intense follow-up of nearby supernovæ for resolving standing questions about the progenitor systems and explosion mechanisms of Type Ia supernovæ.

Determination of Endograft Apposition, Position, and Expansion in the Aortic Neck Predicts Type Ia Endoleak and Migration After Endovascular Aneurysm Repair

2018 ◽  
Vol 25 (3) ◽  
pp. 366-375 ◽  
Author(s):  
Richte C. L. Schuurmann ◽  
Kim van Noort ◽  
Simon P. Overeem ◽  
Ruben van Veen ◽  
Kenneth Ouriel ◽  
...  
Keyword(s):  
Endovascular Aneurysm Repair ◽  
Renal Arteries ◽  
Added Value ◽  
Control Group ◽  
Main Body ◽  
Aortic Neck ◽  
Type Ia ◽  
And Migration ◽  
Aneurysm Repair

Purpose: To describe the added value of determining changes in position and apposition on computed tomography angiography (CTA) after endovascular aneurysm repair (EVAR) to detect early caudal displacement of the device and to prevent type Ia endoleak. Methods: Four groups of elective EVAR patients were selected from a dataset purposely enriched with type Ia endoleak and migration (>10 mm) cases. The groups included cases of late type Ia endoleak (n=36), migration (n=9), a type II endoleak (n=16), and controls without post-EVAR complications (n=37). Apposition of the endograft fabric with the aortic neck, shortest distance between the fabric and the renal arteries, expansion of the main body (or dilatation of the aorta in the infrarenal sealing zone), and tilt of the endograft toward the aortic axis were determined on the first postoperative and the last available CTA scan without type Ia endoleak or migration. Differences in these endograft dimensions were compared between the first vs last scan and among the 4 groups. Results: No significant differences in endograft configurations were observed among the groups on the first postoperative CTA scan. On the last CTA scan before a complication arose, the position of the fabric relative to the renal arteries, expansion of the main body, and apposition of the fabric with the aortic neck were significantly different between the type Ia endoleak (median follow-up 15 months) and migration groups (median follow-up 23 months) compared with the control group (median follow-up 19 months). Most endograft dimensions had changed significantly compared with the first postoperative CTA scan for all groups. Apposition had increased in the control group but had decreased significantly in the type Ia endoleak and migration groups. Conclusion: Progressive changes in dimensions of the endograft within the infrarenal neck could be detected on regular CTA scans before the complication became urgent in many patients.

scholarly journals The surgical management of type IA endoleak after thoracic endovascular aortic repair

2020 ◽  
Vol 31 (3) ◽  
pp. 346-353
Author(s):  
Yaojun Dun ◽  
Yi Shi ◽  
Hongwei Guo ◽  
Yanxiang Liu ◽  
Xiangyang Qian ◽  
...  
Keyword(s):  
Surgical Management ◽  
Circulatory Arrest ◽  
Artery Bypass ◽  
Thoracic Endovascular Aortic Repair ◽  
Endovascular Aortic Repair ◽  
Elephant Trunk ◽  
Direct Closure ◽  
Aortic Repair ◽  
Type Ia

Abstract OBJECTIVES Our goal was to investigate the surgical strategy for type Ia endoleak after thoracic endovascular aortic repair (TEVAR) by reporting our experiences. METHODS From November 2012 to September 2019, a total of 23 patients received surgical management for type Ia endoleak after TEVAR. RESULTS The operations included total arch replacement with the frozen elephant trunk technique in 15 patients, direct closure of the endoleak in 2 patients, hybrid aortic arch repair in 4 patients, arch debranching with TEVAR in 1 patient and left common carotid artery to left subclavian artery bypass with TEVAR in 1 patient. Among 21 patients with cardiopulmonary bypass (CPB), the mean CPB and aortic cross-clamp times were 146.7 ± 42.2 and 81.0 ± 43.3 min, respectively. The selective cerebral perfusion time was 18.8 ± 8.2 min in 17 patients with hypothermic circulatory arrest. The in-hospital mortality was 8.7% (2/23). Type Ia endoleak was sealed successfully after surgery in 95.5% (21/22) of patients. The follow-up data were available for all 21 survivors. The median follow-up period was 18 months (range 1–84 months). During the follow-up period, a total of 8 patients died or had aortic events, including 5 deaths and 6 aortic events. CONCLUSIONS Different surgical strategies could be selected to treat patients with type Ia endoleak after TEVAR, with acceptable early and late outcomes.

scholarly journals Strongly Lensed Supernovae in Well-Studied Galaxy Clusters with the Vera C. Rubin Observatory

Symmetry ◽  
2020 ◽  
Vol 12 (12) ◽  
pp. 1966
Author(s):  
Tanja Petrushevska
Keyword(s):  
Galaxy Clusters ◽  
High Redshift ◽  
Cosmological Parameters ◽  
Hubble Constant ◽  
Multiple Images ◽  
Strong Lensing ◽  
Type Ia ◽  
Ia Supernovae ◽  
Massive Galaxy

Strong lensing by galaxy clusters can be used to significantly expand the survey reach, thus allowing observation of magnified high-redshift supernovae that otherwise would remain undetected. Strong lensing can also provide multiple images of the galaxies that lie behind the clusters. Detection of strongly lensed Type Ia supernovae (SNe Ia) is especially useful because of their standardizable brightness, as they can be used to improve either cluster lensing models or independent measurements of cosmological parameters. The cosmological parameter, the Hubble constant, is of particular interest given the discrepancy regarding its value from measurements with different approaches. Here, we explore the feasibility of the Vera C. Rubin Observatory Legacy Survey of Space and Time (LSST) of detecting strongly lensed SNe in the field of five galaxy clusters (Abell 1689 and Hubble Frontier Fields clusters) that have well-studied lensing models. Considering the 88 systems composed of 268 individual multiple images in the five cluster fields, we find that the LSST will be sensitive to SNe Ia (SNe IIP) exploding in 41 (23) galaxy images. The range of redshift of these galaxies is between 1.01<z<3.05. During its 10 years of operation, LSST is expected to detect 0.2±0.1 SN Ia and 0.9±0.3 core collapse SNe. However, as LSST will observe many more massive galaxy clusters, it is likely that the expectations are higher. We stress the importance of having an additional observing program for photometric and spectroscopic follow-up of the strongly lensed SNe detected by LSST.

Outcomes of endosutured aneurysm repair with the Heli-FX EndoAnchor implants

Vascular ◽  
2020 ◽  
Vol 28 (5) ◽  
pp. 568-576
Author(s):  
Georgios Karaolanis ◽  
Constantine N Antonopoulos ◽  
Stylianos Koutsias ◽  
George A Antoniou ◽  
Efthymios Beropoulis ◽  
...  
Keyword(s):  
Therapeutic Option ◽  
Meta Analysis ◽  
Endovascular Aneurysm Repair ◽  
Pooled Analysis ◽  
Aortic Aneurysms ◽  
Technical Success ◽  
Hostile Neck ◽  
Type Ia ◽  
Aneurysm Repair

Objective Endovascular aneurysm repair has gained field over open surgery for the treatment of abdominal aortic aneurysm. However, type Ia endoleak represents a common complication especially in hostile neck anatomy that is recently faced using endoanchors. We conducted a systematic review and meta-analysis to collect and analyse all the available comparative evidence on the outcomes of the endosuture aneurysm repair in patients with or without hostile neck in standard endovascular aneurysm repair. Methods The current meta-analysis was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. All the prospective and retrospective studies reporting primary use of the Heli-FX EndoAnchor implants were considered eligible for inclusion in this study. The main study outcomes (technical success of endoanchor implantation, incidence of type Ia endoleak, aortic stent graft migration and the percentage of patients who presented regression or expansion of aneurysm sac throughout the follow-up) were subsequently expressed as proportions and 95% confidence intervals. Results Eight studies with a total of 968 patients were included in a pooled analysis. The technical success of the primary endoanchor fixation was 97.12% (95%CI: 92.98–99.67). During a mean six months follow-up period, a pooled rate of 6.23% (95%CI: 0.83–15.25) of the patients developed a persistent type Ia endoleak despite the primary implantation. Migration of the main graft was reported in five studies, in which a 0.26% (95%CI = 0.00–1.54) of the patients required an additional proximal aortic cuff. Regression of the aneurysm sac was observed at 68.82% (95%CI: 51.02–84.21). An expansion of the aneurysm sac was found in 1.93% (95%CI: 0.91–3.24) of the participants. The overall survival rate was 93.43% (95%CI: 89.97–96.29) at a mean six months follow-up period. Conclusions Endosuture aneurysm repair with the Heli-FX EndoAnchor implants seems to be technically feasible and safe either for prevention or for repair of intraoperative type Ia endoleak. Despite the primary implants of endoanchors, few cases of persistent type Ia endoleak and migration are still conspicuous. Long-term follow up is needed to determinate the role of this therapeutic option in the treatment of aortic aneurysms.

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