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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。

We have salvaged and treated many emergency and critical disease which conclude cardiac arrest, acute myocardial infarction with shock, malignant cardiac arrhythmia, septic shock, massive gastrointestinal hemorrhage, massive emptysis, severe respiratory failure, acute respiratory distress syndrome, diabetic ketoacidosis, hyperosmolar coma, severe pancreatitis and multiple organ failure .

曾多次成功地进行了心肺复苏、急性心肌梗塞合并休克、恶性心律失常、感染中毒性休克、消化道大出血、大咯血、严重呼吸衰竭、急性呼吸窘迫综合征、酮症酸中毒和高渗性昏迷、重症胰腺炎及多器官功能衰竭等急重症的抢救和治疗。

"Patients Suffering From Severe Malnutrition Due to Major Enterectomy, Intestinal Failure, or Other Chronic Disease, Already on a Fully Intravenous Diet for More Than 30 Days, and Still Unable to Obtain Sufficient Nutrition Through an Oral Diet ",Patients Suffering From Severe Malnutrition Due to Major

"因肠道大量切除或失去功能,或其他慢性疾病引起严重营养不良者,给予全静脉营养已超过三十天,口摄饮食仍无法提供足量营养","因肠道大量切除或失去功能,或其他慢性疾病引起严重营养不良者,给予全静脉营养已超过三十天,口摄饮食仍无法提供足量营养","214 "," 90,853,771 ","424,550 "

Beijingensis under three droughttreatments, normal, moderate and severe stresses. The results showed:(1) the canker disease ofboth cultivars was serious gradually with increased drought;(2) the bark tissue cells sufferedplasmolysis, more evident with the severity of drought stress;(3) under the drought andinoculation with B. dothidea, cells of two cultivars damaged at different degree, mailyrepresented in the changes of organelles, such as chloroplast swollen and distorted, number ofmitochondria increased and membrane system indistinct; then organelles suffered furtherdamagement with inoculation time, thinned mitochondrias stroma, decreased cristae, crumpledand partly broken membrane of chloroplasts with stroma exosmosis. At last, the chloroplastspartly disorganized;(4) the hyphae growed mainly intercellular in resistant cultivar and notonly intercellular but also intracellular in susceptible cultivar, which directly caused thenecrosis of cells;(5) under the severe drought, the damage of cells enhanced the infection ofpathogen and drought and pathogen stressed together and promoted the disease development;the damage from pathogen on cells was more serious than that from drought.

结果显示:(1)随着干旱胁迫程度的增加,2种杨树溃疡病害发生渐趋严重;(2)干旱胁迫下,杨树树皮组织细胞发生质壁分离,并随胁迫程度的增加而严重;(3)干旱胁迫下接种病原菌,2种杨树细胞发生不同程度的损伤,主要表现为细胞器发生较大变化,如出现叶绿体肿胀变形、线粒体数量增多,质膜模糊不清等现象;随接种时间的延长,细胞器受到进一步损伤,叶绿体被膜折皱,严重时局部破裂,基质外渗,并部分最终解体;(4)毛白杨中的菌丝主要在细胞间隙中穿行,而北京杨的菌丝除在细胞间隙中生长之外,侵入细胞内部也较多,直接导致细胞的解体;(5)干旱胁迫下细胞的损伤促进了病原菌的侵染,干旱和病原菌的双重胁迫加剧了病害的发生程度,并且病原菌侵染对细胞的破坏程度大于水分胁迫。

Probably influenced by contemporary theories on the usefulness of music as a therapeutic device passed on to her by her personal physician, Giuseppe Cervi, the Queen of Spain, Elisabetta Farnese, had come to believe that Farinelli's voice might be able to cure the severe depression from which her husband, King Philip V, had long suffered, to the severe detriment of affairs of state.

可能是受到当时理论的影响,西班牙皇后Elisabetta Farnese的私人医师Giuseppe Cervi 告诉她音乐治病的有用性。

The silicone rod is an effective material for use in frontalis suspension in treating severe ptosis. Elasticity and ease of adjustment are favorable characteristics of using a silicone rod in frontalis slings procedure to correct severe ptosis with poor Bell's phenomenon.

silicone rod frontalis slings是用来矫正眼瞼下垂病人很有效的方法,而且因为这材质的弹性及方便调整的特性,对於严重眼瞼下垂而且Bell's phenomenon不良的病人,其手术效果及术后减少exposure keratopathy的严重程度,均比传统使用之材质好。

This case are same to light, moderate arid severe erosion type, which turn out to be a curve similar to Gaussian distribution, but soil erosion types at the two sides of the curve are totally different, in the left side of the curve, soil erosion are dominated by moderate and severe erosion type, arid in the right side of the curve, mainly soil erosion type is light erosion.

这种情况对于轻度、中度及强度土壤侵蚀的影响是类似的,都呈类正态分布的曲线形式,但在曲线的两侧土壤侵蚀类型是有本质区别的,在曲线的左侧土壤侵蚀类型的分布是中度和强度为主,而在曲线右侧则以轻度为主。

The operation methods were chosen according to Hailer index, which were subdivided into four degrees: slight, middle, severe and extreme severe ones.

按不同程度来选择Nuss手术或两次多点或双弧形支架或双支撑架固定法手术,并评价手术是否安全可行和有效。

Results A total of 51 cases of patients with 6 cases of pacemaker dysfunction occurred: Pacing electrode catheter dislocation example 1 (1.9%), electrode fracture example 1 (1.9%), electrode insulation layer breakdown of example 1 (1.9%), 4 cases of perceptual dysfunction (7.8%), pacemaker-mediated tachycardia I example (1.9%), adopted to adjust the pacing parameters or replacement of pacemakers and pacing electrode catheter, pacing function returned to normal; 5-year follow-up, 3 cases of capsular hematoma and capsular rupture (5.9%), out by local hematocele, antibiotic rinse, closed cavity, in severe cases, re-incision and drainage to remove blood clots after compression bandaging article, in severe cases of infection, clear sinus, closed cavity, catheter inserted in the contra-lateral re-production of capsular bag implantation of pacemaker, to strengthen the anti-inflammatory treatment, made a very good effect. Two cases with pacemaker syndrome, 13 cases with cardiac dysfunction, 8 cases with cerebral infarction.

结果:51例患者中共有6例发生起搏器功能异常:起搏电极导管脱位、电极断裂、电极绝缘层破裂起搏器介导的心动过速各1例(1.9%),感知功能障碍4例(7.8%),通过程控或更换起搏器和起搏电极导管后,起搏功能恢复正常。5年随访中,囊袋血肿、囊袋破溃者3例(5.9%),经局部抽出积血,抗生素冲洗,严重者重新手术后取得很好疗效;2例患者出现起搏器综合征,13例患者出现心功能不全,8例患者并发脑梗死。

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