Long-term Outcome of Hirschsprung Disease: Impact on Quality of Life and Social Condition at Adult Age

BACKGROUND: Hirschsprung disease is a rare congenital disease typically requiring surgical treatment during childhood. Quality of life and social condition at adult age can be impaired by disease-specific sequelae. OBJECTIVE: This study aimed to assess the quality of life and social outcome of adult patients operated on for Hirschsprung disease during childhood. DESIGN: Patients operated on for Hirschsprung disease during childhood were identified and specific questionnaires were sent to them. SETTINGS: Data from 2 referral centers were used. PATIENTS: Patients who completed the questionnaires regarding quality of life and social condition were included. MAIN OUTCOME MEASURES: The Hirschsprung\u27s Disease and Anorectal Malformations Quality of Life disease-specific questionnaire (8 dimensions explored; each scored from 0 to 100 maximum score) and a sociodemographic questionnaire were sent to identified patients. Sociodemographic data were compared with those of the French general population. RESULTS: Thirty-four patients had Hirschsprung disease (men, 76%; mean age, 32 years) were included in the study. Mean total Hirschsprung\u27s Disease and Anorectal Malformations Quality of Life score was 611 of 800 (maximum score 800). The 2 most impaired dimensions were "physical symptoms" and "diarrhea" (62.9/100 and 73.6/100). Fecal continence was only marginally affected (mean score, 89/100). Patients with Hirschsprung disease achieved better educational levels than the French general population. Parental and marital status did not differ between the 2 groups. LIMITATIONS: This study had the limitations inherent to a retrospective study. CONCLUSION: The quality of life of adult patients with Hirschsprung disease sequelae is marginally impaired in this study. Despite the consequences of this congenital abnormality, the condition eventually achieved can be considered as satisfactory. See Video Abstract at http://links.lww.com/DCR/A917

Iatrogenic pneumothorax: experience of a Moroccan Emergency Center

AbstractThe incidence of iatrogenic pneumothorax (IPx) will increase with invasive procedures particularly at training hospitals, which is why we have made a retrospective study of the common diagnostic or therapeutic causes of IPx and its impact on morbidity. From January 2011 to December 2012, 36 patients developed IPx as emergencies, after an invasive procedure .Their mean age was 38 years (range: 19-69 years). Of the patients, 21 (58%) were male and 15 (42%) were female. The purpose was diagnostic in 6 cases and therapeutic in 30 cases. In 8 patients (22%) the procedure was performed due to underlying lung diseases and in 28 patients (78%) for other diseases. The procedure most frequently causing IPnx was central venous catheterization, with 20 patients (55%), other frequent causes were mechanical ventilation, 8 cases (22%) of whom we reported 3 cases of bilateral pneumothorax, 6 cases of thoracentesis (16%) and 2 patients had life-saving percutaneous tracheotomy. The majority of our patients were managed by a small chest tube placement (unilateral n=30, bilateral n=3).The average duration of drainage was 3 days (range: 1-15 days), sadly one of our patients died of ischemic brain damage 15 days after tracheotomy.At training hospitals the incidence of IPnx will increase with the increase in invasive procedures, which should only be performed by experienced personnel or under their supervision

A Review on Multi-Terminal High Voltage Direct Current Networks for Wind Power Integration

With the growing pressure to substitute fossil fuel-based generation, Renewable Energy Sources (RES) have become one of the main solutions from the power sector in the fight against climate change. Offshore wind farms, for example, are an interesting alternative to increase renewable power production, but they represent a challenge when being interconnected to the grid, since new installations are being pushed further off the coast due to noise and visual pollution restrictions. In this context, Multi-Terminal High Voltage Direct Current (MT-HVDC) networks are the most preferred technology for this purpose and for onshore grid reinforcements. They also enable the delivery of power from the shore to offshore Oil and Gas (O&G) production platforms, which can help lower the emissions in the transition away from fossil fuels. In this work, we review relevant aspects of the operation and control of MT-HVDC networks for wind power integration. The review approaches topics such as the main characteristics of MT-HVDC projects under discussion/commissioned around the world, rising challenges in the control and the operation of MT-HVDC networks and the modeling and the control of the Modular Multilevel Converter (MMC) stations. To illustrate the challenges on designing the control system of a MT-HVDC network and to corroborate the technical discussions, a simulation of a three-terminal MT-HVDC network integrating wind power generation and offshore O&G production units to the onshore grid is performed in Matlab's Simscape Electrical toolbox. The results highlight the main differences between two alternatives to design the control system for an MT-HVDC network

Toric Calabi-Yau supermanifolds and mirror symmetry

We study mirror symmetry of supermanifolds constructed as fermionic extensions of compact toric varieties. We mainly discuss the case where the linear sigma A-model contains as many fermionic fields as there are U(1) factors in the gauge group. In the mirror super-Landau-Ginzburg B-model, focus is on the bosonic structure obtained after integrating out all the fermions. Our key observation is that there is a relation between the super-Calabi-Yau conditions of the A-model and quasi-homogeneity of the B-model, and that the degree of the associated superpotential in the B-model is given in terms of the determinant of the fermion charge matrix of the A-model.Comment: 20 pages, v2: references adde

On self-sustaining processes in Rayleigh-stable rotating plane Couette flows and subcritical transition to turbulence in accretion disks

Subcritical transition to turbulence in Keplerian accretion disks is still a controversial issue and some theoretical progress is required in order to determine whether or not this scenario provides a plausible explanation for the origin of angular momentum transport in non-magnetized accretion disks. Motivated by the recent discoveries of exact nonlinear steady self-sustaining solutions in linearly stable non-rotating shear flows, we attempt to compute similar solutions in Rayleigh-stable rotating plane Couette flows and to identify transition mechanisms in such flows by combining nonlinear continuation methods and asymptotic theory. We obtain exact nonlinear solutions for Rayleigh-stable cyclonic regimes but show that it is not possible to compute solutions for Rayleigh-stable anticyclonic regimes, including Keplerian flow, using similar techniques. We also present asymptotic descriptions of these various problems at large Reynolds numbers that provide some insight into the differences between the non-rotating and Rayleigh-stable anticyclonic regimes and derive some necessary conditions for mechanisms analogous to the non-rotating self-sustaining process to be present in flows on the Rayleigh line. Our results demonstrate that subcritical transition mechanisms cannot be identified in wall-bounded Rayleigh-stable anticyclonic shear flows by transposing directly the phenomenology of subcritical transition in cyclonic and non-rotating wall-bounded shear flows. Asymptotic developments, however, leave open the possibility that nonlinear self-sustaining solutions may exist in unbounded or periodic flows on the Rayleigh line. These could serve as a starting point to discover solutions in Rayleigh-stable flows, but the nonlinear stability of Keplerian accretion disks remains to be determined.Comment: 16 pages, 12 figures. Accepted for publication in A&

MESOTHELIUM PERICARDIQUE PRIMITIF SIMULANT UN KYSTE HYDATIQUE PERICARDIQUE

The mesothelioma of pericardium is a rare tumor. Its clinical presentation is polymorphous and its diagnosis is especially histological. We report a case of 63 years old women who presented, one month before, dyspnea on exertion which evolved to orthopnea, associated to atypical thoracic pain, admitted in intensive care unit for symptoms of compressive effusion. Transthoracic echocardiography was favourable of hydatid cyst of pericardium. A thoracotomy, following cardio-circulatory arrest, showed cystic tumor of pericardium invading the left auricle, mediastinal pleura and the left pulmonary hilum. Histopathologic examination confirmed the diagnosis of mesothelioma of pericardium.Le mésothélium péricardique primitif malin est une tumeur rare. Sa présentation clinique est polymorphe et son diagnostic est surtout histologique. Nous rapportons le cas d’une patiente âgée de 63 ans ayant présenté, un mois auparavant, une dyspnée d’effort ayant évolué vers l’orthopnée et associée à des douleurs thoraciques atypiques, a été admise au service des urgences dans un tableau de pré-tamponnade. L’échocardiographie transthoracique était en faveur d’un kyste hydatique péricardique avec compression des cavités cardiaques droites. Suite à un arrêt cardio-circulatoire, une thoracotomie de sauvetage a montré une masse kystique péricardique envahissant l’oreillette gauche, la plèvre mediastinale et le hile pulmonaire gauches. L’examen anatomopathologique à l’autopsie a objectivé un mésothélium péricardique primitif malin

On Local Calabi-Yau Supermanifolds and Their Mirrors

We use local mirror symmetry to study a class of local Calabi-Yau super-manifolds with bosonic sub-variety V_b having a vanishing first Chern class. Solving the usual super- CY condition, requiring the equality of the total U(1) gauge charges of bosons \Phi_{b} and the ghost like fields \Psi_{f} one \sum_{b}q_{b}=\sum_{f}Q_{f}, as \sum_{b}q_{b}=0 and \sum_{f}Q_{f}=0, several examples are studied and explicit results are given for local A_{r} super-geometries. A comment on purely fermionic super-CY manifolds corresponding to the special case where q_{b}=0, \forall b and \sum_{f}Q_{f}=0 is also made.\bigskipComment: 17 page

Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): a collaborative report from the International and European Society for Pediatric Oncology DIPG registries

Purpose Diffuse intrinsic pontine glioma (DIPG) is a brainstem malignancy with a median survival of < 1 year. The International and European Society for Pediatric Oncology DIPG Registries collaborated to compare clinical, radiologic, and histomolecular characteristics between short-term survivors (STSs) and long-term survivors (LTSs). Materials and Methods Data abstracted from registry databases included patients from North America, Australia, Germany, Austria, Switzerland, the Netherlands, Italy, France, the United Kingdom, and Croatia. Results Among 1,130 pediatric and young adults with radiographically confirmed DIPG, 122 (11%) were excluded. Of the 1,008 remaining patients, 101 (10%) were LTSs (survival ≥ 2 years). Median survival time was 11 months (interquartile range, 7.5 to 16 months), and 1-, 2-, 3-, 4-, and 5-year survival rates were 42.3% (95% CI, 38.1% to 44.1%), 9.6% (95% CI, 7.8% to 11.3%), 4.3% (95% CI, 3.2% to 5.8%), 3.2% (95% CI, 2.4% to 4.6%), and 2.2% (95% CI, 1.4% to 3.4%), respectively. LTSs, compared with STSs, more commonly presented at age < 3 or > 10 years (11% v 3% and 33% v 23%, respectively; P < .001) and with longer symptom duration ( P < .001). STSs, compared with LTSs, more commonly presented with cranial nerve palsy (83% v 73%, respectively; P = .008), ring enhancement (38% v 23%, respectively; P = .007), necrosis (42% v 26%, respectively; P = .009), and extrapontine extension (92% v 86%, respectively; P = .04). LTSs more commonly received systemic therapy at diagnosis (88% v 75% for STSs; P = .005). Biopsies and autopsies were performed in 299 patients (30%) and 77 patients (10%), respectively; 181 tumors (48%) were molecularly characterized. LTSs were more likely to harbor a HIST1H3B mutation (odds ratio, 1.28; 95% CI, 1.1 to 1.5; P = .002). Conclusion We report clinical, radiologic, and molecular factors that correlate with survival in children and young adults with DIPG, which are important for risk stratification in future clinical trials

American Society for Bone and Mineral Research-Orthopaedic Research Society Joint Task Force Report on Cell-Based Therapies - Secondary Publication.

Cell-based therapies, defined here as the delivery of cells in vivo to treat disease, have recently gained increasing public attention as a potentially promising approach to restore structure and function to musculoskeletal tissues. Although cell-based therapy has the potential to improve the treatment of disorders of the musculoskeletal system, there is also the possibility of misuse and misrepresentation of the efficacy of such treatments. The medical literature contains anecdotal reports and research studies, along with web-based marketing and patient testimonials supporting cell-based therapy. Both the American Society for Bone and Mineral Research (ASBMR) and the Orthopaedic Research Society (ORS) are committed to ensuring that the potential of cell-based therapies is realized through rigorous, reproducible, and clinically meaningful scientific discovery. The two organizations convened a multidisciplinary and international Task Force composed of physicians, surgeons, and scientists who are recognized experts in the development and use of cell-based therapies. The Task Force was charged with defining the state-of-the art in cell-based therapies and identifying the gaps in knowledge and methodologies that should guide the research agenda. The efforts of this Task Force are designed to provide researchers and clinicians with a better understanding of the current state of the science and research needed to advance the study and use of cell-based therapies for skeletal tissues. The design and implementation of rigorous, thorough protocols will be critical to leveraging these innovative treatments and optimizing clinical and functional patient outcomes. In addition to providing specific recommendations and ethical considerations for preclinical and clinical investigations, this report concludes with an outline to address knowledge gaps in how to determine the cell autonomous and nonautonomous effects of a donor population used for bone regeneration. © 2020 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 38:485-502, 2020

American Society for Bone and Mineral Research-Orthopaedic Research Society Joint Task Force Report on Cell-Based Therapies.

Cell-based therapies, defined here as the delivery of cells in vivo to treat disease, have recently gained increasing public attention as a potentially promising approach to restore structure and function to musculoskeletal tissues. Although cell-based therapy has the potential to improve the treatment of disorders of the musculoskeletal system, there is also the possibility of misuse and misrepresentation of the efficacy of such treatments. The medical literature contains anecdotal reports and research studies, along with web-based marketing and patient testimonials supporting cell-based therapy. Both the American Society for Bone and Mineral Research (ASBMR) and the Orthopaedic Research Society (ORS) are committed to ensuring that the potential of cell-based therapies is realized through rigorous, reproducible, and clinically meaningful scientific discovery. The two organizations convened a multidisciplinary and international Task Force composed of physicians, surgeons, and scientists who are recognized experts in the development and use of cell-based therapies. The Task Force was charged with defining the state-of-the art in cell-based therapies and identifying the gaps in knowledge and methodologies that should guide the research agenda. The efforts of this Task Force are designed to provide researchers and clinicians with a better understanding of the current state of the science and research needed to advance the study and use of cell-based therapies for skeletal tissues. The design and implementation of rigorous, thorough protocols will be critical to leveraging these innovative treatments and optimizing clinical and functional patient outcomes. In addition to providing specific recommendations and ethical considerations for preclinical and clinical investigations, this report concludes with an outline to address knowledge gaps in how to determine the cell autonomous and nonautonomous effects of a donor population used for bone regeneration. © 2019 American Society for Bone and Mineral Research