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.     

世界著名公害事件

比利时马斯河谷事件1930年12月,比利时马斯河谷工业区排放的工业有害废气和粉尘对人体造成综合影响,一周内近60人死亡,市民心脏病、肺病患者的死亡率增高,家畜死亡率也大大增高。     

磁流变阻尼器结构控制策略研究进展

磁流变阻尼器(Magnetorheological Damper,简称MR阻尼器)是一种在振动控制领域中表现出巨大应用潜力的智能型驱动装置,它主要是根据输入电压(电流)的变化产生趋近于最优主动控制力的阻尼力。本文详细综述了MR阻尼器控制策略的研究现状,分析了各种控制策略的优缺点。探讨了如何充分发挥不同电压下的MR阻尼器耗能能力,指出神经网络和模糊逻辑等智能技术的应用是解决这一问题的有效途径。     

中国地震灾害与人文要素的相关性分析

中国的地震灾害与人员伤亡为世界最严重的国家之一，这不仅由于地震多、震级大、烈度高，多数地方建筑物抗震性能较低，而且还与人文因素密切相关。本文着重讨论了人品密度与震害伤亡人数的相关性，并分析了作息时间、作业方式、地震预报、心理状态等人文要素与震害伤亡人数的相关性。     

Efficient degradation of lube oil by a mixed bacterial consortium

A laboratory study was performed to assess the biodegradation of lube oil in bio-reactor with 304# stainless steel as a biofilm carrier. Among 164 oil degrading bacterial cultures isolated from oil contaminated soil samples, Commaonas acidovorans Px1, Bacillus sp. Px2, Pseudomonas sp. Px3 were selected to prepare a mixed consortium for the study based on the efficiency of lube oil utilization. The percentage of oil degraded by the mixed bacterial consortium decreased slightly from 99% to 97.2% as the concentration of lube oil was increased from 2000 to 10,000 mg/L. The degradation of TDOC (total dissolved organic carbon) showed a similar tendency compared with lube oil removal, which indicated that the intermediates in degradation process hardly accumulated. Selected mixed bacterial consortium showed their edge compared to activated sludge. Scanning electron microscopy (SEM) photos showed that biofilms on stainless steel were robust and with a dimensional framework constructed by EPS (extracellular polymeric substances), which could promote the biodegradation of hydrocarbons. The increase of biofilm followed first-order kinetics with rate of 0.216 μg glucose/(cm2·day) in logarithm phase. With analysis of Fourier transform infrared spectroscopy (FT-IR) and gas chromatography-mass spectrometry (GC-MS) combined with removal of lube oil and TDOC, mixed bacterial consortium could degrade benzene and its derivatives, aromatic ring organic matters with a percentage over 97%.     

Resolving the influence of nitrogen abundances on sediment organic matter in macrophyte-dominated lakes,using fluorescence spectroscopy

A controlled experiment was designed to resolve the influence of nitrogen abundance on sediment organic matters in macrophyte-dominated lakes using fluorescence analysis.Macrophyte biomass showed coincident growth trends with time, but different variation rates with nitrogen treatment. All plant growth indexes with nitrogen addition(N, NH4Cl100, 200, 400 mg/kg, respectively) were lower than those of the control group. Four humiclike components, two autochthonous tryptophan-like components, and one autochthonous tyrosine-like component were identified using the parallel factor analysis model. The results suggested that the relative component changes of fluorescence in the colonized sediments were in direct relation to the change of root biomass with time. In the experiment, the root formation parameters of the plants studied were significantly affected by adding N in sediments, which may be related to the reason that the root growth was affected by N addition.Adding a low concentration of N to sediments can play a part in supplying nutrients to the plants. However, the intensive uptake of NH4+may result in an increase in the intracellular concentration of ammonia, which is highly toxic to the plant cells. Hence, our experiment results manifested that organic matter cycling in the macrophyte-dominated sediment was influenced by nitrogen enrichment through influencing vegetation and relevant microbial activity.     

水解－好氧法处理含硫废水的研究

采用水解－好氧法处理炼油过程中产生的含硫废水。试验结果表明：经过此工艺处理后的出水COD、氨氮、硫化物、酚分别为118.5mg/L、29.4mg/L、0.0mg/L和0.50mg/L,相应的去除率分别为90.4％、56.6％、100％和99.3％,出水水质达到国家有关排放标准。同时,设计独特的三相好氧床不仅能有较高的硝化能力,而且无剩余污泥排放。因此,可以省略剩余污泥的处理过程     

Ecological and Economic System Health Assessment Based on Fuzzy Set Pair Analysis： A Case Study of Northwest Region, China

Due to the increasingly serious environmental pollution and destruction,especially humans’ unreasonable activities,the ecological and economic system(EES) issues of Northwest region in China have attracted more and more attention of the researchers.Aiming at evaluating its ecological and economic system health,a multi-objective evaluation framework called PressureState-Response(PSR) was established to describe the ecological and economic health situations.Meanwhile,an integrative set pair model combining set pair analysis(SPA) and fuzzy analytic hierarchy process(FAHP) was proposed to assess the ecological and economic system.Then the EES status of five northwest provinces(Shanxi,Gansu,Qinghai,Ningxia and Xinjiang) of Northwest region in China was evaluated during 1985 to 2009.The EES development trends of five provinces are obtained.In general,the health values of five provinces showed a rising trend.The health values of five provinces grew rapidly during 1985 to 2000.After 2000,the health values of five provinces still followed the present growth trend,but the growth is relatively smooth.The results show that the method proposed is effective for assessing the health of ecological and economic system.