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Leber congenital amaurosis is the most severe retinal dystrophy causing blindness or severe visual impairment before the age of 1 year.

Leber先天性黑蒙是最严重的视网膜营养不良性疾病,可导致患者在1岁前发生失明或严重视力损伤。

Artificial joint for wrist is gradually applied in clinic, and provides an alternation for patients with severe wound of wrist joint, anchylosis and severe traumatic arthritis.

人工腕关节逐渐被应用于临床,为因腕关节严重创伤、关节僵硬和严重创伤性关节炎的患者提供了一个可供选择的新方法。

Severe and unusually severe OSA patients could be at higher risk of arrhythmic death than milder OSA.

严重和极严重的阻塞性睡眠呼吸暂停综合症患者比较轻的患者有较高的致死性心律失常的风险。

Results摘要: The incidence rates of renal dysfunction, shock, cardiovascular failure and gastrointestinal hemorrhage, the score of acute physiology and chronic health evaluation Ⅱ and the frequencies of pulse and breath in the severe hypoalbuminemia group were all higher than those in the mild hypoalbuminemia group (P<0.05 or P<0.01). The differences of incidence rate of hepatic failure and the scores of Ranson and Balthazar CT between these two groups had no statistical significance (P>0.05). The incidence rate of infection and the mortality in the severe hypoalbuminemia group were higher than those in the mild hypoalbuminemia group (P<0.01) in the later stage of SAP.

结果摘要:重度低白蛋白血症组肾功能衰竭、休克、心衰及消化道出血的发生率高于轻度低白蛋白血症组(P<0.01,肝功能衰竭发生率两组比较无统计学差异;Ranson评分及Balthazar CT评分两组间比较,差异无统计学意义(P>0.05);重度低白蛋白血症组的急性生理和慢性健康评价指标Ⅱ(acute physiology and chronic health evaluationⅡ, APACHEⅡ)评分、脉搏和呼吸频率均明显高于轻度低白蛋白血症组(P<0.05或P<0.01;重度低白蛋白血症组后期感染发生率及病死率均高于轻度低白蛋白血症组

Results: The incidence rates of renal dysfunction, shock, cardiovascular failure and gastrointestinal hemorrhage, the score of acute physiology and chronic health evaluation Ⅱ and the frequencies of pulse and breath in the severe hypoalbuminemia group were all higher than those in the mild hypoalbuminemia group (P<0.05 or P<0.01). The differences of incidence rate of hepatic failure and the scores of Ranson and Balthazar CT between these two groups had no statistical significance (P>0.05). The incidence rate of infection and the mortality in the severe hypoalbuminemia group were higher than those in the mild hypoalbuminemia group (P<0.01) in the later stage of SAP.

结果:重度低白蛋白血症组肾功能衰竭、休克、心衰及消化道出血的发生率高于轻度低白蛋白血症组(P<0.01,肝功能衰竭发生率两组比较无统计学差异;Ranson评分及Balthazar CT评分两组间比较,差异无统计学意义(P>0.05);重度低白蛋白血症组的急性生理和慢性健康评价指标Ⅱ(acute physiology and chronic health evaluationⅡ, APACHEⅡ)评分、脉搏和呼吸频率均明显高于轻度低白蛋白血症组(P<0.05或P<0.01;重度低白蛋白血症组后期感染发生率及病死率均高于轻度低白蛋白血症组

The complications in the early stage, related parameters, and the incidence rate of infection and mortality in the later stage were evaluated respectively. Results: The incidence rates of renal dysfunction, shock, cardiovascular failure and gastrointestinal hemorrhage, the score of acute physiology and chronic health evaluation Ⅱ and the frequencies of pulse and breath in the severe hypoalbuminemia group were all higher than those in the mild hypoalbuminemia group (P<0.05 or P<0.01). The differences of incidence rate of hepatic failure and the scores of Ranson and Balthazar CT between these two groups had no statistical significance (P>0.05). The incidence rate of infection and the mortality in the severe hypoalbuminemia group were higher than those in the mild hypoalbuminemia group (P<0.01) in the later stage of SAP.

结果:重度低白蛋白血症组肾功能衰竭、休克、心衰及消化道出血的发生率高于轻度低白蛋白血症组(P<0.01,肝功能衰竭发生率两组比较无统计学差异;Ranson评分及Balthazar CT评分两组间比较,差异无统计学意义(P>0.05);重度低白蛋白血症组的急性生理和慢性健康评价指标Ⅱ(acute physiology and chronic health evaluationⅡ, APACHEⅡ)评分、脉搏和呼吸频率均明显高于轻度低白蛋白血症组(P<0.05或P<0.01;重度低白蛋白血症组后期感染发生率及病死率均高于轻度低白蛋白血症组

Spontaneous rupture of esophagus also known as Boerhaave's syndrome, is an extremely severe thoracic disease with high mortality, and is difficult to diagnose because of its rarity and frequent confusion with other severe clinical conditions, such as acute myocardial infarction, perforated peptic ulcer and acute pancreatitis.

自发性食管破裂,又称Boerhaave综合征,是临床上一种严重的胸科急症,因临床少见且易与其他疾病,如急性心肌梗死、消化性溃疡穿孔、急性胰腺炎等混淆,临床诊断较困难,死亡率高。

The organization liable for severe illegal practice will be deprived of the operating qualification by the original certification or approval organizations; the executives and other personnel directly responsible for the severe illegal practices are subject to such disciplinary actions as post-lowering, deposal or dismissal according to applicable regulations; any people liable for the illegal practice constituting a crime will be subject to the criminal liability

情节严重的,由原认证或者批准机关取消其相应的资格;对直接负责的主管人员和其他直接责任人员,依法给予降级、撤职或者开除的处分;构成犯罪的,依法追究刑事责任

Hepatic diseases are common, about 10% of them die of acute liver failure , ALF is a sever clinical pathophysiological syndrome of metabolic, secretic and immune disarrangement caused by severe hepatocyte dysfunction and decompensation, caused by drug, toxin, severe hepatitis and cirrhosis, liver injury or/and hepatectomy.

本课题为国家"863"计划生物项目的系列研究之一肝病是多发病,其中大约10%患者死于急性肝功能衰竭(acuteliver failure,ALF),是由药物、毒物、重症肝炎或肝硬变、肝外伤或/和肝切除术后致肝细胞功能严重受损、失代偿,引起机体代谢、内分泌、免疫等功能紊乱的一种严重临床病理生理综合症。

Gene mutations were detected in 25 patients and a 18-year-old girl among 16 families. Trinucleotide repeats of CAG were 73~79. The fragments of abnormal alleles were 380~402bp, and all patients were heterozygous. The copy numbers of normal alleles were 18~40, fragments from 202~270bp. SCV reduction was much obvious compared to MCV, MCV and SCV in lower limb ?ere much more slow than that in upper's. BAEP, VEP were also delayed in latency. The anticipation in parental sex bias were much more obvioius than that in matental's. Cerebellar ataxia was most severe, the next were dysarthria and bulging eyes. Amytrophy was seen only in bed ridden patients. Cerebellar atrophy was more severe than brain stem, cord atrophy was n't observed in all MJD.

结果检出10个家系25例病人及1例症状前18岁女孩有MJD基因突变,CAG三核苷酸重复73~79次,异常等位基因片段长380~402bp,均为杂合子;正常人CAG三核苷酸重复18~40次,等位片段长200~270bp,电生理发现MJD的SCV减慢比MCV明显,而下肢的MCV、SCV又较上肢明显,BAEP、VEP均有不同程度的潜伏期延长或波的异常;MJD的父亲遗传早于母亲,进展也较块,临床以小脑性共济失调为突出症状,其次为构音障碍、突眼等,肌肉萎缩仅见于晚期病人;MRI示小脑萎缩较明显,脑干萎缩并不严重,未见明显的颈髓萎缩。

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But in the course of internationalization, they meet with misunderstanding and puzzlement.

许多企业已经意识到了这一点,但在国际化的进程中,仍存在一些误区与困惑。

Inorder toaccomplish this goal as quickly as possible, we'll beteamingup with anexperienced group of modelers, skinners, and animatorswhosenames willbe announced in the coming weeks.

为了尽快实现这个目标,我们在未来数周内将公布与一些有经验的模型、皮肤、动画制作小组合作。

They answered and said to him, Are you also from Galilee?

7:52 他们回答他说,难道你也是出于加利利么?