Syndromic congenital diaphragmatic hernia: Current incidence and outcome. Analysis from the congenital diaphragmatic hernia study group registry

Background

The aim of this study was to describe the incidence of Congenital Diaphragmatic Hernia, CDH, associated with known or clinically suspected syndromes, and the postnatal outcomes from a large database for CDH.

Methods

Data from the multicenter, multinational database on infants with CDH (Congenital Diaphragmatic Hernia Study Group Registry) born from 1996 to 2020 were analyzed. Patients with known or suspected syndromes were grouped and outcome data were analyzed and compared to those without syndromic features.

Results

A total of 12,553 patients were entered in the registry during the study period, and 421 had reported known syndromes, representing 3.4% of all CDH cases in the registry. A total of 50 different associated syndromes were reported. In addition to those with clinically suspected genetic conditions, a total rate of genetic syndromes with CDH was 8.2%. The overall survival to discharge for syndromic CDH was 34% and for non-syndromic CDH was 76.7%. The most common were syndromes Fryns syndrome (19.7% of all syndromes, 17% survival), trisomy 18 or Edward syndrome (17.5%, 9% survival), trisomy 21 or Down syndrome (9%, 47% survival), trisomy 13 or Patau syndrome (6.7%, 14% survival), Cornelia de Lange syndrome (6.4% of all syndromes, 22% survival) and Pallister-Killian syndrome (5.5% of all syndromes, 39.1% survival). In addition, 379 cases had reported chromosomal anomalies and 233 cases had clinically suspected syndromes, based on two more dysmorphic features or malformations in addition to CDH, but without molecular diagnosis. The syndromic CDH group had lower birth weight and gestational age at birth and increased incidence of bilateral CDH (2.9%) and rates of non-repair (53%). The length of hospital stay was longer, and larger number of patients needed O₂ at 30 days. Extracorporeal life support was used only in 15% of the cases. Those who underwent surgical repair had survival to discharge rates of 73%.

Conclusion

Syndromic CDH is rare and only 3.4% of the reported cases of CDH have a known syndrome or association, but, if including patients with two dysmorphic features malformations, in addition to CDH, altogether as many as 8.2% have a diagnosed or suspected genetic condition. These children have with lower survival rates. Given higher rates of non-repair and decreased extracorporeal life support use, along with a high early mortality, decision-making regarding goals of care clearly influences outcomes. Survival varies depending on the genetic cause. Early genetic diagnosis is important and may influence the decision-making.     

Does adding more lettuce make a hamburger truly green? A metaphor behind the green movement paradigm in designing cities

In designing cities, the label of the green movement these days is being applied to almost everything—starting from works of environmental non-governmental organizations and international as well as local conservation organizations to grass-roots resistance activism including radical environmentalism. This paper seeks to understand the motivation—the paradigm—behind the green movement initiatives and how the paradigm appears to shift from the ground reality. Conclusions suggest that, on one hand, environmental problems are far from being a matter of common sense and, on the other, the matter of intellectual and technological developments—the outcomes of rigorous academic research reflected in endless literature predominantly comprising the fields of environmental sociology, environmental anthropology, and conservation psychology—are in reality often found to be dealt with by shallow planning and policy practices without even realizing the big-picture that the paradigm is merely being used as an advertizing tool for making business profit. It is expected that, unless a shift from such negative practices towards a conscious and genuine commitment to proactive environmental stewardship is made, the situation will continue to be exacerbated.     

Combustion,performance, and emission characteristics of diesel engine using oxyhydrogen gas as a fuel additive

Environmental Science and Pollution Research - A lot of research is being carried out to reduce the environmental pollution resulting from compression ignition engines. For this, various gaseous...     

Heavy metals controlling cardiovascular diseases risk factors in myocardial infarction patients in critically environmentally heavy metal-polluted steel industrial town Mandi-Gobindgarh (India)

Environmental Geochemistry and Health - Heavy metals (HMs) have a very significant clinical role in the pathogenesis, progression and management of cardiovascular diseases (CVDs). The prevalence of...     

Critical Risk Indicators (CRIs) for the electric power grid: a survey and discussion of interconnected effects

The electric power grid is a critical societal resource connecting multiple infrastructural domains such as agriculture, transportation, and manufacturing. The electrical grid as an infrastructure is shaped by human activity and public policy in terms of demand and supply requirements. Further, the grid is subject to changes and stresses due to diverse factors including solar weather, climate, hydrology, and ecology. The emerging interconnected and complex network dependencies make such interactions increasingly dynamic, posing novel risks, and presenting new challenges to manage the coupled human–natural system. This paper provides a survey of models and methods that seek to explore the significant interconnected impact of the electric power grid and interdependent domains. We also provide relevant critical risk indicators (CRIs) across diverse domains that may be used to assess risks to electric grid reliability, including climate, ecology, hydrology, finance, space weather, and agriculture. We discuss the convergence of indicators from individual domains to explore possible systemic risk, i.e., holistic risk arising from cross-domain interconnections. Further, we propose a compositional approach to risk assessment that incorporates diverse domain expertise and information, data science, and computer science to identify domain-specific CRIs and their union in systemic risk indicators. Our study provides an important first step towards data-driven analysis and predictive modeling of risks in interconnected human–natural systems.

     

印度染料与染料中间产品工业的清洁生产:一个成功的废物最小化和资源保护预防性环境管理战略

化工品生产,是印度最古老的家庭工业之一.本文聚焦于染料和染料中间产品行业,这个行业的许多公司都是中小企业.在过去10年左右的时间里,由于对它们的环境性能要求越来越高,它们已经历了几次金融压力.流程终端废物治理与排放已经有所改进,但却是有限的成功,因为它的成本昂贵(而且改变废物形态和消除它们并不是一回事).已经开展的为数不多的印度清洁生产行动中,迄今主要还是示范项目.通过贯彻清洁生产措施,参与企业已经提高了生产力、削减了成本并且降低了它们的污染负荷.实现的节余潜力,是原投资的许多倍.     

Surface signatures of submerged heated jet

The flow induced at the surface of a water body by a submerged heated horizontal turbulent jet was investigated experimentally with the aim of developing parameterizations for surface mean temperature/velocity fields. The jet nozzle diameter was fixed, the depth of the jet beneath the free surface was varied, and two jet Reynolds numbers (5020, 11300) were considered. The surface temperature was measured using a highly sensitive infrared camera, and the near-surface horizontal velocity field was measured using particle image velocimetry. The experimental results were explained using a model based on similarity solutions with variable turbulent viscosity. While classical Schlichting’s solution with constant turbulent viscosity predicts complete similarity for transverse velocity/temperature distributions only in a plane that coincides with the flow axis, the present solution predicts similarity in an arbitrary plane parallel to the flow axis, which was confirmed using data collected at the surface. Comparisons of present data with available previous results also showed general agreement.     

Studies on the family hemiuridae (Trematoda: Digenea), with a description of a new genus from marine fish of Indian waters

A new digenetic trematode, collected 1969–1970 at Ratnagiri (India) from the marine eel Leptocephalus conger, is described as Indostomachicola kinnei n. g., n. sp. The new genus differs from Stomachicola Yamaguti, 1934 and Allostomachicola Yamaguti, 1958 in many characteristics. The new species is characterized by an unlobed ovary, absence of receptaculum seminis, and presence of post-acetabular seminal vesicle and a ventral pit.