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Introduction: The AMP-activated protein kinase (AMPK) is a master regulator of energy homeostasis that becomes activated by exercise and binds glycogen, an important energy store required to meet exercise-induced energy demands. Disruption of AMPK-glycogen interactions in mice reduces exercise capacity and impairs whole-body metabolism. However, the mechanisms underlying these phenotypic effects at rest and following exercise are unknown. Furthermore, the plasma metabolite responses to an acute exercise challenge in mice remain largely uncharacterized.Methods: Plasma samples were collected from wild type (WT) and AMPK double knock-in (DKI) mice with disrupted AMPK-glycogen binding at rest and following 30-min submaximal treadmill running. An untargeted metabolomics approach was utilized to determine the breadth of plasma metabolite changes occurring in response to acute exercise and the effects of disrupting AMPK-glycogen binding.Results: Relative to WT mice, DKI mice had reduced maximal running speed (p < 0.0001) concomitant with increased body mass (p < 0.01) and adiposity (p < 0.001). A total of 83 plasma metabolites were identified/annotated, with 17 metabolites significantly different (p < 0.05; FDR

Objective: Glycogen is a major energy reserve in liver and skeletal muscle. The master metabolic regulator AMP-activated protein kinase (AMPK) associates with glycogen via its regulatory β subunit carbohydrate-binding module (CBM). However, the physiological role of AMPK-glycogen binding in energy homeostasis has not been investigated in vivo. This study aimed to determine the physiological consequences of disrupting AMPK-glycogen interactions. Methods: Glycogen binding was disrupted in mice via whole-body knock-in (KI) mutation of either the AMPK β1 (W100A) or β2 (W98A) isoform CBM. Systematic whole-body, tissue and molecular phenotyping was performed in KI and respective wild-type (WT) mice. Results: While β1 W100A KI did not affect whole-body metabolism or exercise capacity, β2 W98A KI mice displayed increased adiposity and impairments in whole-body glucose handling and maximal exercise capacity relative to WT. These KI mutations resulted in reduced total AMPK protein and kinase activity in liver and skeletal muscle of β1 W100A and β2 W98A, respectively, versus WT mice. β1 W100A mice also displayed loss of fasting-induced liver AMPK total and α-specific kinase activation relative to WT. Destabilisation of AMPK was associated with increased fat deposition in β1 W100A liver and β2 W98A skeletal muscle versus WT. Conclusions: These results demonstrate that glycogen binding plays critical roles in stabilising AMPK and maintaining cellular, tissue and whole-body energy homeostasis.

Dynamic changes in circulating and tissue metabolites and lipids occur in response to exercise-induced cellular and whole-body energy demands to maintain metabolic homeostasis. The metabolome and lipidome in a given biological system provides a molecular snapshot of these rapid and complex metabolic perturbations. The application of metabolomics and lipidomics to map the metabolic responses to an acute bout of aerobic/endurance or resistance exercise has dramatically expanded over the past decade thanks to major analytical advancements, with most exercise-related studies to date focused on analyzing human biofluids and tissues. Experimental and analytical considerations, as well as complementary studies using animal model systems, are warranted to help overcome challenges associated with large human interindividual variability and decipher the breadth of molecular mechanisms underlying the metabolic health-promoting effects of exercise. In this review, we provide a guide for exercise researchers regarding analytical techniques and experimental workflows commonly used in metabolomics and lipidomics. Furthermore, we discuss advancements in human and mammalian exercise research utilizing metabolomic and lipidomic approaches in the last decade, as well as highlight key technical considerations and remaining knowledge gaps to continue expanding the molecular landscape of exercise biology.

Background and Aim: Megaloblastic anemia (MA) is a rare pathology in childhood due, in the majority of cases, to a deficiency of folic acid and/or vitamin B12 (cobalamin). This study aims to determine the epidemiological, clinical, and paraclinical profiles of MA in children and to specify its etiologies, therapeutic modalities, and treatment responses., Methods: This is a retrospective descriptive study of MA cases in children carried out in the General Pediatrics Department of the Hedi Chaker University Hospital of Sfax over a period of 42 years, from January 1979 to December 2021. We included all the patients under 16 years old with a myelogram showing megaloblastosis. The selected patients' demographic characteristics, physical signs, laboratory findings, and treatment responses were recorded., Results: Twenty cases of MA were collected, including 11 boys and 9 girls. The incidence of MA in children was 0.014%. The median age at diagnosis was 3.37 years. The clinical presentation was anemic syndrome with pallor and asthenia in all the cases. Neurological manifestations were noted in 2 cases and digestive disorders in 10 cases. Seven infants had psychomotor delays. On admission, all our patients had anemia with an average value of 5.6 g/dl. It was macrocytic in 19 cases. The blood count also revealed leukopenia (7 cases), thrombocytopenia (10 cases), and pancytopenia (5 cases). The myelogram showed megaloblastosis in all the cases and sideroblasts in one. A brain MRI was performed on five patients, and it showed abnormalities in three cases. The etiological investigations revealed a vitamin B12 deficiency secondary to a maternal Biermer's disease (6 cases), malnutrition (3 cases), Imerslund's disease (3 cases), congenital deficiency in transcobalamin II (3 cases), Biermer's disease (1 case), giardiasis (1 case), folic acid deficiency secondary to a poor dietary intake (1 case), mitochondrial cytopathy with vitamin B12-Folic acid deficiency (1 case), and Pearson syndrome (1 case). Our treatment included symptomatic measures, replacement therapy, and etiological treatment. Favorable evolution was noted in 11 cases. Five patients had neurological sequelae, and one patient died., Conclusion: Our study highlights the rarity and heterogeneity of the etiological contexts of MA in children. Early diagnosis and therapeutic support can improve the long-term neurological prognosis., (Copyright © 2025 Society francophone de transfusion sanguine (SFTS). Published by Elsevier Masson SAS. All rights reserved.)

Valve-in-valve (ViV) procedures have emerged from an off-label procedure to a safe and efficient alternative to redo aortic valve replacement in the treatment of symptomatic structural valve deterioration (SVD). During ViV procedures, optimal placement of the transcatheter heart valve (THV) inside the degenerated bioprosthesis is of paramount importance regarding complications such as device embolization, coronary obstruction, periprosthetic regurgitation, residual gradients, and mitral valve injury, but also for the attainment of optimal hemodynamics. In the case of the Mosaic (Medtronic, Minneapolis, MN) valve, the limited radiopaque landmarks represent a challenge to a reproducible, optimal implantation. Such implantation may require multiple contrast injections and transesophageal echocardiogram (TEE) guidance. We herein describe a computer-assisted ViV procedure inside a deteriorated Mosaic valve, achieving reproducible optimal placement using a preacquired library of bioprostheses 3D models. Our approach suggests an evolving paradigm in ViV procedures, from safe and efficient toward optimal therapy for symptomatic SVD., (© 2020 Wiley Periodicals LLC.)

Background:  Understanding near-death experiences (NDE) could provide a new insight into the analysis of human consciousness and the neurocognitive processes happening upon the approach of death. With a temporary interruption of systemic perfusion, aortic surgery under hypothermic circulatory arrest (HCA) may be the only available model of reversible clinical death. We present, herein, the results of an observational study designed to assess the incidence of NDE after aortic surgery., Methods:  We performed a prospective study including consecutive patients who underwent thoracic aortic surgery between July 2018 and September 2019 at our institution. Procedures without HCA were included to constitute a control group. The primary outcome was the incidence of NDE assessed with the Greyson NDE scale during the immediate postoperative course, via a standardized interview of the patients in the surgical ward., Results:  One hundred and one patients were included. Twenty-one patients (20.8%) underwent nonelective interventions for aortic dissection. Ninety-one patients had hemiarch replacement (90.1%). Sixty-seven (66.3%) interventions were performed with HCA, with an average circulatory arrest duration of 26.9 ± 25.5 minutes, and a mean body temperature of 23.7 ± 3.8°C. None of the patients reported any recollection from their period of unconsciousness. There was no NDE experiencer in the study cohort., Conclusion:  Several confounding factors regarding anesthesia, or NDE evaluation, might have impaired the chance of NDE recollections, and might have contributed to this negative result. Whether HCA may trigger NDE remains unknown., Competing Interests: None declared., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/).)

Background: The Shelhigh™ SuperStentless (Shelhigh, INC., Union, NJ, USA) is a stentless aortic valve bioprosthesis and aortic root valved conduit. In 2007, this device was recalled by FDA due to malfunction, and subsequently reintegrated by BioIntegral Surgical™ Few data are available over late durability of this device. We performed a long-term follow-up of Shelhigh™ devices implanted at our center., Methods: Between 2002 and 2007, 44 patients underwent aortic valve replacement with a Shelhigh™ device (40 aortic valve bioprosthesis and 4 valved conduit). We performed a clinical and echocardiographic follow-up (9.2 years±4.3). Standardized definitions of valve-related events were adopted., Results: At discharge, maximum and mean aortic gradients averaged 36.1±11.3 and 21.0±6.8 mmHg, respectively. The 30-days mortality was 2.3%. Over the follow-up period, 29 patients died (65.9%); 2 deaths were valve related. Overall survival at 1, 5 and 10 years was 97.7%, 85.8% and 54% respectively. At last echocardiography, average transvalvular gradients had remained globally stable in the population (33.6±12 and 20.4±10.5 mmHg). Eight (19%) structural valve deterioration (SVD) events were reported. Two (5%) non-structural valve dysfunction (NSVD) events occurred (periprosthetic leak). Two (5%) infectious endocarditis events and two (5%) valve thromboses were also deplored. Three (7%) patients required re-operation (2 due to SVD and 1 due to endocarditis)., Conclusions: The immediate hemodynamic performance of the Shelhigh™ aortic bioprostheses was unexpectedly suboptimal. Despite this, hemodynamic performance remained stable over time. Patients survival at follow-up was satisfactory, however, continued surveillance is necessary.

In the setting of postcoarctation aortic repair, Dacron graft dilatation and late aneurysms are not uncommon. Reintervention usually involves redo open surgery and replacement of the aneurysmal graft or the pseudoaneurysmal suture line. The present case describes the endovascular repair of a Dacron anastomotic false aneurysm in an extra-anatomic ascending-to-descending aortic bypass, 19 years after surgical correction of aortic recoarctation., Competing Interests: The authors declare no conflict of interest related to this article., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/).)

Background: Heart failure (HF) readmission is common post-transcatheter aortic valve replacement (TAVR). Nonetheless, limited data are available regarding its predictors and clinical impact. This study evaluated the incidence, predictors, and impact of HF readmission within 1-year post-TAVR, and assessed the effects of the prescription of HF therapies at discharge on the risk of HF readmission and death., Methods: Patients included in the TAVR registry of a single expert center from 2009 to 2017 were analyzed. Competing-risk and Cox regressions were performed to identify predictors of HF readmission and death., Results: Among 750 patients, 102 (13.6%) were readmitted for HF within 1-year post-TAVR. Overall, 53 patients (7.1%) experienced late readmissions (>30 days post-TAVR), and 17 (2.3%) had multiple readmissions. In ≈30% of readmissions, no trigger could be identified. Predominant causes of readmissions were changes in medication/nonadherence and supraventricular arrhythmia. Independent predictors of HF readmission included diabetes mellitus, chronic lung disease, previous acute HF, grade III or IV aortic regurgitation, and pulmonary hypertension both at discharge from the index hospitalization but not HF therapies. Overall, HF readmission did not significantly impact all-cause mortality (hazard ratio [HR], 1.36 [95% CI, 0.99-1.85]). However, late (HR, 1.90 [95% CI, 1.30-2.78]) and multiple HF readmissions (HR, 2.10 [95% CI,1.17-3.76]) were significantly associated with all-cause mortality. Prescription of renin-angiotensin system inhibitors at discharge was associated with a lower rate of all-cause mortality, especially among patients receiving doses of 25% to <50% (HR, 0.67 [95% CI, 0.48-0.94]) and 75% to 100% (HR, 0.61 [95% CI, 0.37-0.98]) of the optimal daily dose., Conclusions: HF readmission is common within 1-year of TAVR. Late and multiple HF readmissions associate with an increased risk of long-term all-cause mortality. Baseline comorbidities (diabetes, chronic lung disease, previous acute HF) and echocardiographic findings at discharge (grade III or IV aortic regurgitation, pulmonary hypertension) identified patients at high risk of HF readmission.

Left-sided double valve disease in the setting of extensive mitral annular calcifications and porcelain aorta raises multiple surgical challenges when considering an open surgical repair. We herein present the case of a 67-year-old patient with severe symptomatic aortic and mitral stenosis associated with extensive mitral annular calcifications and porcelain aorta, successfully treated by simultaneous transapical transcatheter aortic and mitral valve implantation., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: With the progressive aging of the population, aortic surgeons are caring for an increasing number of elderly patients. The objective of this study was to analyze early and late outcomes of aortic surgery with hypothermic circulatory arrest in patients aged 70 and above at our institution., Methods: We performed a retrospective cohort study including every patient aged 70 years or older who underwent aortic surgery with hypothermic circulatory arrest between January 1995 and June 2016 at our institution. Operative results were compared with the contemporary younger counterparts aged <70 years. In-hospital mortality and postoperative stroke were primary outcomes of interest. The main secondary outcomes included acute renal failure, reoperation for bleeding, and spinal cord injury., Results: In the study population, the in-hospital mortality was 16.8% (21/125). Ten (8.0%) patients presented postoperative stroke, and 6 had temporary neurologic disturbance (4.8%). Spinal cord injury occurred in 1 (0.8%) patient. For elective interventions and type A aortic dissections, the in-hospital mortality and stroke rates were 4.6% (3/65) and 7.7% (5/65), 26.8% (11/41) and 12.2% (5/41), respectively. The proportion of non-elective interventions, including type A aortic dissection, and the type of neuroprotective strategy were similar in septuagenarians and younger patients. Patients aged ≥70 had significant shorter cardiopulmonary bypass, myocardial ischemia, and circulatory arrest durations, compared to their younger counterparts. The in-hospital mortality of septuagenarians and younger patients were similar for elective surgery (4.6% vs. 4.7%, P=0.900) and aortic dissections (26.8% vs. 15.1%, P=0.107). There was no statistically significant difference between the two age groups regarding postoperative stroke, spinal cord injury, renal failure requiring dialysis or reintervention for bleeding. Estimated 1-, 3-, and 5-year survival was 78.0%, 70.6%, and 65.7%, respectively. The 5-year survival for elective surgery was 74.9% and 56.0% for non-elective procedures., Conclusions: Aortic surgery with circulatory arrest in the elderly demonstrated favorable early and late results when compared with younger individuals, with an acceptable operative risk even under emergency conditions, and should not be denied only because of the chronological age of the patients.

Background: Valve-in-valve is established as a safe and efficient alternative to redo surgery in the treatment of structural valve deterioration (SVD). In vitro models rely on the radiopaque landmarks of undeteriorated tissue valves to establish the optimal implantation level of the transcatheter heart valves inside the deteriorated valves. In computed assisted procedures, the radiopaque landmarks of the deteriorated valves may be used to guide valve implantation through image fusion. The purpose of this study is to determine whether SVD alters the radiopaque landmarks of stented tissue valves., Methods: Our approach was based on the computation of relevant anatomical measurements from CT images. Radiopaque landmarks of degenerated bioprostheses and the corresponding undeteriorated valves were extracted to create surface meshes and cloud points using grey-level thresholding. 3D registration using an iterative closest point algorithm was used to align the corresponding cloud points, while the modified Hausdorff Distance was applied to determine the differences between them., Results: The proposed evaluation was performed on 19 degenerated tissue valves. 15 valves were scanned from patients evaluated for valve-in-valve procedures, and 4 bioprostheses were scanned after surgical extraction during redo aortic valve replacement. All the degenerated valves were compared to the corresponding undeteriorated models. Overall, the mean difference between degenerated and undeteriorated valves was 0.33 ± 0.12 mm. The maximum observed registration error was 0.66 mm., Conclusions: Our study demonstrates no significant difference between the radiopaque landmarks of deteriorated and undeteriorated bioprostheses after the occurrence of SVD. Our findings suggest therefore that SVD does not alter radiopaque landmarks of stented tissue valves. These results validate in-vitro studies of optimal transcatheter heart valves implantation inside deteriorated tissue valves based on their radiopaque landmarks, and allow the use of non-deteriorated valves' imaging features in computer assisted valve-in-valve procedures., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Introduction: Asthma is a common respiratory childhood disease that results from an interaction between genetic, environmental and immunologic factors. The implication of nucleotide-binding and oligomerization domain 1 and 2 (NOD1/CARD4, NOD2/CARD15) was highlighted in many inflammatory diseases., Methods: In this case-control study, we analyzed the association of three NOD2 polymorphisms and one NOD1 variant, in 338 Tunisian asthmatic children and 425 healthy Controls, using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method. We also assessed NOD1 and NOD2 mRNA and protein levels by qRT-PCR and ELISA techniques., Results: The homozygous AA genotype of rs2075820 was a risk factor for asthma (OR 2.39). The influence of the E266K variant in the presence of the heterozygous AG genotype was higher in male than female groups. The homozygous AA genotype was a risk factor associated with asthma, for patients aged between 6 and 18 years OR 2.39, IC95% (1.04-5.49) p < 0.01. The mRNA expression of NOD1, but not NOD2, was enhanced in asthma patients compared to Controls. We noted a significant difference between asthmatics and healthy controls in NOD1 protein expression (asthma patients : 31.18 ± 10.9 pg/ml, Controls: 20.10 ± 2.58 pg/ml; p < 0.001)., Conclusions: The NOD1 rs2075820 variant was associated with a higher childhood asthma risk and the NOD1 expression at mRNA and protein levels was significantly increased in asthma patients.

Background:  Aortic valve replacement (AVR) in small aortic roots remains a surgical dilemma with a higher risk of patient-prosthesis mismatch (PPM). The Perimount Magna Ease aortic valve (PMEAV) represents an attractive device in such cases. We examined the early hemodynamic performance, the mid-term outcomes of the PMEAV, and the impact of PPM on outcome and functional class., Methods:  We performed a retrospective analysis of prospectively collected in-hospital data, and a prospective single-center follow-up of 849 patients who received a 19 to 23 mm PMEAV (2008-2014). Concomitant mitral or tricuspid replacement was the exclusion criterion. Early hemodynamic features were prospectively collected; mid-term follow-up was conducted according to current guidelines., Results:  Size of implanted prosthesis was 19 mm in 11.5% of patients, 21 mm in 36.9%, and 23 mm in 51.5%. Operative mortality was 4.5% (3.1% for isolated AVR). The rate of severe and moderate PPMs was significantly higher in the 19 mm group. Follow-up was 99.9% complete (3.7 ± 2 years). Actuarial freedom from structural valve deterioration (SVD) at 5 years was 99.1%. At stratified Kaplan-Meier's analysis, PPM and age <70 years were associated with SVD. PPM was not associated with worse functional status (New York Heart Association class) or mortality at follow-up., Conclusion:  This series shows satisfactory clinical outcomes of the PMEAV implanted in small aortic annuli at mid-term follow-up. Although PPM may occur in smaller sizes, it has limited clinical impact, and it is not associated with mid-term mortality or worse functional class. Few SVD events are evidenced; nonetheless, limited follow-up duration and its methodology need to be considered., Competing Interests: All authors declare no conflict of interest with respect to the material presented in this article., (Georg Thieme Verlag KG Stuttgart · New York.)

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Structural valve deterioration is a well-studied phenomenon in bioprosthetic heart valves. Conversely, structural valve deterioration after transcatheter aortic valve implantation is not as well-elucidated. Therefore, late surgical explantation after transcatheter aortic valve implantation is an infrequent procedure, and thus, surgical findings and management of such cases remain unclear, particularly in patients previously labelled as 'inoperable' or at 'high risk'. Herein, we report the case of a late surgical explantation of a transcatheter heart valve (THV) 7 years after its implantation in a patient with a porcelain aorta and periprosthetic regurgitation., (© The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Transfemoral approach stands as the reference access-route for transcatheter aortic valve implantation (TAVI). Nonetheless, alternatives approaches are still needed in a significant proportion of patients. This study aimed at comparing outcomes between transthoracic-approach (transapical or transaortic) and transarterial-approach (transcarotid or subclavian) TAVI. Data from 191 consecutive patients who underwent surgical-approach TAVI from May 2009 to September 2017 were analyzed. Patients were allocated in 2 groups according to the approach. The primary end point was the 30-day composite of death of any cause, need for open surgery, tamponade, stroke, major or life-threatening bleeding, stage 2 or 3 acute kidney injury, coronary obstruction, or major vascular complications. During the study period, 104 patients underwent transthoracic TAVI (transapical: 60.6%, transaortic: 39.4%) whereas 87 patients underwent transarterial TAVI (subclavian: 83.9%, transcarotid: 16.1%). Logistic EuroSCORE I tended to be higher in transthoracic-TAVI recipients. In-hospital and 30-day composite end point rates were 25.0% and 11.5% (p = 0.025), and 26.0% and 14.9% (p = 0.075) for the transthoracic and transarterial cohorts, respectively. Propensity score-adjusted logistic regression demonstrated no significant detrimental association between the 30-day composite end point and transthoracic access (odds ratio 2.12 95% confidence interval 0.70 to 6.42; p = 0.18). Transarterial TAVI was associated with a shorter length of stay (median: 6 vs 7 days, p <0.001). TAVI approach was not an independent predictor of midterm mortality. In conclusion, nontransfemoral transarterial-approach TAVI is safe, feasible, and associated with comparable rates of major perioperative complications, and midterm mortality compared with transthoracic-approach TAVI., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Benign metastasizing leiomyoma (BML) is a rare entity characterized by proliferation of extra-uterine smooth muscle tumors. BML has both malignant behavior and benign characteristics. Here, we present a case of pulmonary BML occurring in a 70-year-old woman treated by surgery., Competing Interests: Conflict of interestThe authors declare that they have no conflict of interest., (© Indian Association of Cardiovascular-Thoracic Surgeons 2018.)

Sickle cell disease is a genetic disorder characterized by a hypercoagulable state. Several complications in this hemoglobinopathy are increased by thrombosis. Factor V Leiden (FVL), prothrombin (PRT) G20210A, and methylenetetrahydrofolate reductase (MTHFR) C677T and A1298C mutations are major inherited risk factors of thrombotic complications. In this study, our aim was to compare the frequencies of these mutations in sickle cell patients with healthy controls. The study population comprised 35 homozygous Hb S (HBB: c.20A>T) patients, 29 compound heterozygous patients [16 Hb S/β 0 -thalassemia (β 0 -thal), four Hb S/β + -thal, seven Hb S/Hb C (HBB: c.19G>A) and two Hb S/Hb O-Arab (HBB: c.364G>A)] and 100 healthy subjects. All patients and controls were subjected to laboratory investigations as well as mutation genotyping. Our findings showed a severe anemia with the lowest values of protein S (PS), protein C (PC) and antithrombin (AT) in the homozygous Hb S group compared to Hb S/Hb C and Hb S/β-thal subjects. No significant difference in FVL genotype between patients and controls was observed, whereas high frequencies of PRT G20210A, MTHFR C677T and MTHFR A1298C mutations in the Hb S patients and a significant association between the MTHFR C677T mutation and Hb S/β 0 -thal were found.

Objectives: Our goal was to evaluate the operative outcomes of the frozen elephant trunk technique using the E-Vita Open Plus® hybrid prosthesis in chronic aortic arch diseases and report clinical and radiological outcomes at the 1-year follow-up., Methods: As determined from a prospective multicentre registry, 94 patients underwent frozen elephant trunk procedures using the E-Vita Open Plus hybrid device for the treatment of chronic aortic conditions, including 50% chronic aortic dissections, 40% degenerative aneurysms and 10% miscellaneous indications. Fifty percent of the cases were reoperations., Results: The perioperative mortality rate was 11.7%. Spinal cord ischaemia and stroke rates were 4% and 9.6%, respectively. The mean cardiopulmonary bypass time was 252 ± 97 min, cardiac ischaemia time was 152 ± 53 min and cerebral perfusion time was 82 ± 22 min. Concomitant procedures were observed in 15% of patients. Among the 83 surviving patients, the survival rate after the 1-year follow-up was 98%. Eleven percent of patients underwent endovascular completion, whereas 4% of patients required aortic reintervention at 1 year., Conclusions: The E-Vita Open Plus hybrid device confirms the favourable short- and mid-term outcomes offered by its predecessor in frozen elephant trunk procedures in patients with chronic aortic arch disease. Implantation of the E-Vita Open Plus is associated with good 1-year survival rates, good rates of favourable aortic remodelling in both chronic dissection and degenerative aneurysms and a reproducible technique in a multicentre registry. Continued follow-up is required due to the risk of evolution at the downstream aorta., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Interleukin-37 (IL-37) has been identified as a novel anti-inflammatory cytokine. The present study aimed to evaluate the expression of IL-37 in serum and in peritoneal fluid to determine its clinical significance in endometriosis. Enzyme-linked immunosorbent assay (ELISA) was performed to examine serum IL-37 levels in patients with endometriosis and healthy controls. Peritoneal fluid IL-37 mRNA and NFκB expression were quantified by real-time reverse transcription polymerase chain reaction assays. The association of IL-37 levels with clinical factors and prognosis of endometriosis was analysed. We found that IL-37 levels in PF and in serum were significantly higher in patients with endometriosis compared to women without endometriosis (P=0.0005). IL-37 levels were highly expressed in PF [132.38±34.62pg/mL; P<0.0001] than in serum [74.10±13.49pg/mL] in endometriosis patients. IL-37 mRNA expression contrasted with NFκB mRNA expression in PF from patients with endometriosis. A significant inverse correlation was observed between IL-37 mRNA and NFκB mRNA expression. IL-37 expression correlates with endometriosis severity. The affected NFκB mRNA expression in endometriosis contributed the to exhibited increase of IL-37. The increased levels of IL-37 may dampen NFκB activation in endometriosis patients., (Copyright © 2017. Published by Elsevier B.V.)

Objective: Valve-in-valve (ViV) procedures are increasingly being considered as an alternative to redo surgery for the treatment of degenerated bioprosthetic heart valves in patients with excessive reoperative risk. The objective of our study was to evaluate the feasibility of computer guidance in transcatheter heart valve (THV) implantation during ViV procedures., Methods: Preprocedural electrocardiogram-gated computed tomography-scan images were processed using semiautomatic segmentation of the degenerated bioprosthesis' radiopaque landmarks and of the ascending aorta. Virtual three-dimensional (3D) reconstructions were created. A virtual plane was subsequently added to the 3D reconstructions, indicating the optimal landing plane of the THV inside the tissue valve. Within a hybrid operating theater, a 3D/2D registration was used to superimpose the 3D reconstructions, while dynamic tracking was allowed to maintain the superimposition onto the fluoroscopic images. The THV was afterward implanted according to the optimal landing plane. Projection of the ascending aorta and the coronary arteries was used to assess the risk of coronary ostia obstruction., Results: Between January 2014 and October 2014, nine patients underwent aortic ViV procedures in our institution. Among those nine patients, five procedures were retrospectively evaluated as a validation step using the proposed method. The mean (SD) superimposition error was 1.1 (0.75) mm. Subsequently, two live cases were prospectively carried out using our approach, successfully implanting the THV inside the degenerated tissue valve., Conclusions: Our study demonstrates the feasibility of a computer-guided implantation of THV in ViV procedures. Moreover, it suggests that augmented reality may increase the reliability of THV implantation inside degenerated bioprostheses through better reproducibility.

The analysis of some extra- and intragenic markers within or closely linked to the cystic fibrosis transmembrane regulator (CFTR) gene is useful as a molecular method in clinical linkage analysis. Indeed, knowing that the molecular basis of cystic fibrosis (CF) is highly heterogeneous in our population, the study of haplotype association with normal and CF chromosomes could be very helpful in cases where one or both mutations remain unidentified. In this study, we analysed with PCR-RFLP and capillary electrophoresis some extra (pJ3.11, KM19 and XV2C) and intragenic (IVS8CA, IVS17bTA and IVS17bCA) polymorphic markers in 50 normal and 10 Tunisian patients carrying the rare E1104X mutation in order to determine the haplotype associated with this mutation. For the extragenic markers, 8 haplotypes were identified. The most frequent of them are the 221 and 112 accounting for 80% of total haplotypes. For the intragenic markers, five haplotypes were present on the E1104X chromosomes. One of them 16-31-13 accounted for 50%. To our knowledge, this is the first work to be interested to the haplotypes linked to the E1104X mutation. This preliminary study of haplotypes could be a helpful method to determine the molecular lesions responsible of this pathology.

Background and Objectives: Analbuminemia is a very rare autosomal recessive disorder. It is an allelic heterogeneous defect caused by a variety of mutations within the albumin gene. We describe in this report two new cases of analbuminemia in Libyans., Design and Methods: The 14 coding exons of the human serum albumin (HSA) gene and their intron-exon junctions were PCR amplified. The products were screened for mutations by Denaturing High Performance Liquid Chromatography (DHPLC). Samples with altered DHPLC profiles were sequenced., Results: DNA sequencing revealed the presence of a novol homozygous G➔T transition in the first base of intron 11 (c.1428+1G>T), in both children. This mutation destroys the GT consensus donor sequence found at the 5' end of most intervening sequences and would cause the defective pre-mRNA splicing., Conclusion: Molecular diagnosis based on DHPLC and DNA sequencing represents a powerful tool to study molecular defects causing analbuminemia., (Copyright © 2012 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.)

Background: To study the disturbances of biochemical parameters which make it possible to evaluate the nutritional state during the cystic fibrosis., Methods: Prospective study about 13 cases hospitalized over a 7 months period, whose age varies between 2 months and 12 years and addressed for a suspicion of cystic fibrosis. For all the patients we made a clinical collection of the data and a biochemical study., Results: Hypoprotidemia (protidemia < 60 g/l) was noted in 6 cases. Three cases presented a hypocalcaemia (calcemia < 2.20 mmol/l). A hypomagnesaemia (magnesemia < 0.70 mmol/L) was observed in 2 cases. The totality of the patients presented zinc concentrations lower than the normal value. Iron concentrations lower than the normal (11- 24 mmol/l) was noted in 5 cases. Four patients (4/13) presented a concentration of iron between 11.2 and 20 mmol/l, whereas a high concentration (32.3 mmol/l) was noted in only one case (7.7%)., Conclusion: The study of the biochemical parameters allowed to evaluate the variation of some trace elements in cystic fibrosis and their consequence on the nutritional state of the patients, which constitutes an essential element for the assessment of these patients.

Background: The delivery of a large baby may indicate that the mother had abnormal glucose tolerance during pregnancy. Glycosylated hemoglobin (HbA1c) concentration might be expected to identify women who had high blood glucose concentration before delivery., Aim: The aim of this study was to identify retrospectively, gestational diabetes in mothers of large baby and determine the HbAlc cutoff value., Methods: HbA1 was measured in 216 patients within the first three days of postpartum: 100 had large babies: weighing over than 4000 g and 113 had normal- sized babies (control group). We exclude mothers who had preterm, hypotrophy baby, stillborn, and diabetic mothers., Results: The mean concentration of HbA1c was significantly higher in group with large babies than in group control (6.17% + 085 vs. 5.17 + 0.57 t = 9.78 p < 0.001). The value of HbAlc = 5.85%, evaluated by ROC curve, was considered as risk factor of macrosomia and then gestational diabetes. 83.5% of mothers with large babies had HbA1c ? 5.85 vs. 7.8% of those with normal sized babies (p < 0.0001). No other significant differences were found between the two groups in other parameters., Conclusion: HbA1c level may be of value as a postpartum screen for unrecognized diabetes and may help discriminate between a constitutionally large but otherwise normal newborn and a large infant of a diabetic mother. HbA1c measurements should be obtained in women with large babies, and, if upper than cutoff value found by curve ROC: 5.85%, maternal and fetal surveillance is recommended.

Unlabelled: Our objective is to compare clinical and biological presentation of patients with bone metastatic disease., Patients and Methods: We collected prospectively 60 patients (adults and children) with proven bone metastasis. Tumors are mainly breast cancer (25/60) or nasopharyngeal carcinoma (8/60). All 8 children presented all with abdominal neuroblastoma. Bone lesions are lytic in 85% of cases. ALP and LDH seem to be sensitive markers for bone mestatasis with 75% and 80% pathologic rates. The highest rates have been observed in patients with multiple bones lesions (> 8) and painful metastases (more than 7 in the VAS). The median survival was 8 months (3 to 54)., Conclusion: Even conventional, some biochemical markers as ALP and LDH remain useful in the diagnosis and prognosis in patients with proven bone metastasis.