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metabolic crisis
相关语句
  代谢危象
     Acute Metabolic Crisis Induced by Vaccination in Patients of Inherited Endocrine Metabolic Disorders
     预防接种诱发先天缺陷患儿急性代谢危象病例研究
短句来源
     Objectives To describe the c li nical course of patients with acute metabolic crisis induced by vaccination in t hose with inherited metabolic or endocrine disorders.
     目的 回顾了7例预防接种诱发急性代谢危象的遗传代谢、内分泌疾病患儿的临床经过。
短句来源
  “metabolic crisis”译为未确定词的双语例句
     Two patients had positive famil y history. Results Acute metabolic crisis occurred at 3-12 hours after Japanese encephalitis,diphtheria and tetanus toxoi ds and acellular pertussis(DTaP),hepatiti s B or measles vaccine administration in 7 patients.
     结果 7例患者儿分别于接种乙型脑炎、百白破、乙型肝炎、麻疹疫苗后3~12h出现急性代谢紊乱。
短句来源
  相似匹配句对
     The Crisis of Modernization
     现代化的危机——浅议中国现代化进程中应注意的几个问题
短句来源
     Crisis of Realism
     实在的危机
短句来源
     Metabolic Syndrome
     代谢综合症
短句来源
     Metabolic Engineering
     代谢工程
短句来源
     Acute Metabolic Crisis Induced by Vaccination in Patients of Inherited Endocrine Metabolic Disorders
     预防接种诱发先天缺陷患儿急性代谢危象病例研究
短句来源
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  metabolic crisis
Summary: A 32-year-old man with glutathione synthetase deficiency developing an acute metabolic crisis is described.
      
Acute metabolic crisis with extreme deficiency of glutathione in combination with decreased CSF levels of leukotriene C4 in a pa
      
During the second day of life, he developed clinical signs of an acute metabolic crisis with severe hypoglycaemia and hyperammonaemia.
      
At least 7 of these patients had a metabolic crisis during a catabolic condition.
      
The clinical and biochemical phenotype of isolated GKD may vary from a life-threatening childhood metabolic crisis to asymptomatic adult 'pseudohypertriglyceridaemia', resulting from hyperglycerolaemia.
      
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Objective The accumulation of organic acids metabolized in children with inborn errors of metabolism (IEM) may cause acute metabolic crises and result in long-term neurological dysfunction or death.This study intends to evaluate the effect of continuous blood purifiltration (CBP) in the treatment of IEM with severe organic acidemia.Methods 9 children with IEM in severe acidosis crises were treated by CBP. Their blood gas analysis,plasma biochemistry were examined before,during and after CBP.Results...

Objective The accumulation of organic acids metabolized in children with inborn errors of metabolism (IEM) may cause acute metabolic crises and result in long-term neurological dysfunction or death.This study intends to evaluate the effect of continuous blood purifiltration (CBP) in the treatment of IEM with severe organic acidemia.Methods 9 children with IEM in severe acidosis crises were treated by CBP. Their blood gas analysis,plasma biochemistry were examined before,during and after CBP.Results The children weighting 2.6~13kg were fretful or in coma,manifestite as oliguria or anuria with pH(6.87±0.25),BB(1.6±0.08)mmol/L,SB(4.0±0.58)mmol/L,BE(-26.1±2.80)mmol/L,Lactate(15.2±3.64)mmol/L,ammonia(287.53±132.43)mmol/L in blood and were much better.In phenomena with pH were (7.33±0.18)mmol/L,BB(18.0±2.56)mmol/L,SB(19.1±2.25)mmol/L,BE(-3.4±1.62)mmol/L,lactate(3.1±0.55)mmol/L,ammonia(39.21±22.85)μmol/L in blood after CBP.Conclusion The successful use of CBP in these children suggested that CBP may be the optimal technique for the severe organic acidemia crises associated with IEM.

目的 分析、评价连续血液净化 (continuousbloodpurification ,CBP)对先天性代谢缺陷病 (inbornerrorsofmetabolism ,IEM )重症有机酸血症的治疗效果 ,探讨其治疗机制。方法 用BaxterBM 2 5机对 9例IEM重症有机酸血症患儿行CBP治疗 ,对比治疗前后血气、生化值 ,并比较症状、体征改善情况。结果  9例患儿入院时 pH值 (6 87± 0 2 5 )、BB(1 6± 0 0 8)mmol/L、SB(4 0± 0 5 8)mmol/L、BE(- 2 6 1± 2 80 )mmol/L、乳酸 (15 2± 3 6 4 )mmol/L ,血氨 (2 87 5 3± 132 4 3) μmol/L ,呈重度代谢性酸中毒。经 1~ 2次 ,9~ 32hCBP治疗 ,患儿症状、体征好转 ,酸碱平衡部分纠正 ,pH(7 33± 0 18)mmol/L、BB(18 0± 2 5 6 )mmol/L、SB(19 1± 2 2 5 )mmol/L、BE(- 3 4± 1 6 2 )mmol/L、乳酸 (3 1±0 5 5 )mmol/...

目的 分析、评价连续血液净化 (continuousbloodpurification ,CBP)对先天性代谢缺陷病 (inbornerrorsofmetabolism ,IEM )重症有机酸血症的治疗效果 ,探讨其治疗机制。方法 用BaxterBM 2 5机对 9例IEM重症有机酸血症患儿行CBP治疗 ,对比治疗前后血气、生化值 ,并比较症状、体征改善情况。结果  9例患儿入院时 pH值 (6 87± 0 2 5 )、BB(1 6± 0 0 8)mmol/L、SB(4 0± 0 5 8)mmol/L、BE(- 2 6 1± 2 80 )mmol/L、乳酸 (15 2± 3 6 4 )mmol/L ,血氨 (2 87 5 3± 132 4 3) μmol/L ,呈重度代谢性酸中毒。经 1~ 2次 ,9~ 32hCBP治疗 ,患儿症状、体征好转 ,酸碱平衡部分纠正 ,pH(7 33± 0 18)mmol/L、BB(18 0± 2 5 6 )mmol/L、SB(19 1± 2 2 5 )mmol/L、BE(- 3 4± 1 6 2 )mmol/L、乳酸 (3 1±0 5 5 )mmol/L ,血氨 (39 2 1± 2 2 85 ) μmol/L ,较治疗前显著好转。 结论 临床观察提示 ,CBP是治疗IEM有机酸血症的一种快速有效的方法

Objectives To describe the c li nical course of patients with acute metabolic crisis induced by vaccination in t hose with inherited metabolic or endocrine disorders. Method s The clinical features,laboratory findings,diagnosis,treatm ent and outcome were reviewed in 7 patients(5 boys and 2 girls).Only one boy wi th 21-hydroxylase deficiency(21-OHD)was diagnosed previously when he was 1 month old.Others,whose diagnosis were confirmed after hospitalization through endoc rinological tests,urine...

Objectives To describe the c li nical course of patients with acute metabolic crisis induced by vaccination in t hose with inherited metabolic or endocrine disorders. Method s The clinical features,laboratory findings,diagnosis,treatm ent and outcome were reviewed in 7 patients(5 boys and 2 girls).Only one boy wi th 21-hydroxylase deficiency(21-OHD)was diagnosed previously when he was 1 month old.Others,whose diagnosis were confirmed after hospitalization through endoc rinological tests,urine organic acid analysis,blood acylcarnitine profiles or brain MR imaging.Two patients had positive famil y history. Results Acute metabolic crisis occurred at 3-12 hours after Japanese encephalitis,diphtheria and tetanus toxoi ds and acellular pertussis(DTaP),hepatiti s B or measles vaccine administration in 7 patients.Case one and two had acute a drenal insufficiency.Vomiting,dehydration,hyperkalemia,hyponatremia and mark ed elevation of serum 17-hydroxyprogesterone were found in those cases.21-OHD w as diagnosed in the two patients.After hydrocortisone and fludrocortisone suppl ementations,they felt better than before.But case two of acute adrenal cr isis induced by pneumonia when she was one year old.Case 3-7 received vaccine w hen they were 3-9 months old.Case three had mild movement retardation previously with s eizures,coma,metabolic acidosis and ketosis at 10 hours after vacination.Meth y lmalonic aciduria was confirmed by urine organic acid analysis.The boy died at the age of two years because of severe metabolic acidosis.Case 4-7 had norm al development previ o usly.Three patients suffered from fever,seizures,coma,acidosis and hypoglyce mia after vaccination at the age of 5-9 months.Although they got better after allop a thic treatment,severe psychomotor retardation and general palsy remained.Gluta ri c aciduria type 1 was detected in case 4 at the age of 2 years.Leigh syndrome w a s found in case 5-7 at the age of 1 year.Three babies died in respiratory failu r e when they were one and half years old.Typical pathological findings,such as sy mmetrical foci,cystic cavitations with neuronal loss,vascular proliferation,w ere observed by autopsy. Conclusions Careful etiological investigation would be hel pful both for the patients with malnutrition and psychomotor retardation before vaccination to prevent the accidents and explain the causes of adverse events.

目的 回顾了7例预防接种诱发急性代谢危象的遗传代谢、内分泌疾病患儿的临床经过。方法 对7例(男性5例、女性2例)患儿的临床经过、实验室特点、诊疗与转归进行分析。结果 7例患者儿分别于接种乙型脑炎、百白破、乙型肝炎、麻疹疫苗后3~12h出现急性代谢紊乱。例1、2于5岁、3个月时接种疫苗后出现急性肾上腺危象,血浆17-羟孕酮均明显增高,符合2 1-羟化酶缺乏症失盐型。例3出生后运动发育落后,3个月时接种疫苗,10h后抽搐、昏迷,伴酮症、代谢性酸中毒,经尿有机酸分析证实为甲基丙二酸尿症。例4~7接种前发育正常,于5~9个月接种疫苗后发热、抽搐、昏迷,伴代谢性酸中毒、低血糖,经对症治疗虽有好转,但智力严重倒退,全身瘫痪,于1~2岁来院接受病因分析。例4为戊二酸尿症1型。例5~7为Leigh综合征,分别于1岁半左右死于呼吸衰竭。死亡后尸体解剖发现双侧基底节、脑干多灶性坏死伴海绵样变性,神经细胞严重丢失,毛细血管和星形胶质细胞增生。结论 报告了7例因预防接种诱发急性代谢危象的病例。其中2例为2 1-羟化酶缺乏症,5例为遗传代谢病患儿,6例患儿预防接种前原发病未获诊。虽然疫苗本身不是致病原因,但诱发了急性发作。建议对营养发...

目的 回顾了7例预防接种诱发急性代谢危象的遗传代谢、内分泌疾病患儿的临床经过。方法 对7例(男性5例、女性2例)患儿的临床经过、实验室特点、诊疗与转归进行分析。结果 7例患者儿分别于接种乙型脑炎、百白破、乙型肝炎、麻疹疫苗后3~12h出现急性代谢紊乱。例1、2于5岁、3个月时接种疫苗后出现急性肾上腺危象,血浆17-羟孕酮均明显增高,符合2 1-羟化酶缺乏症失盐型。例3出生后运动发育落后,3个月时接种疫苗,10h后抽搐、昏迷,伴酮症、代谢性酸中毒,经尿有机酸分析证实为甲基丙二酸尿症。例4~7接种前发育正常,于5~9个月接种疫苗后发热、抽搐、昏迷,伴代谢性酸中毒、低血糖,经对症治疗虽有好转,但智力严重倒退,全身瘫痪,于1~2岁来院接受病因分析。例4为戊二酸尿症1型。例5~7为Leigh综合征,分别于1岁半左右死于呼吸衰竭。死亡后尸体解剖发现双侧基底节、脑干多灶性坏死伴海绵样变性,神经细胞严重丢失,毛细血管和星形胶质细胞增生。结论 报告了7例因预防接种诱发急性代谢危象的病例。其中2例为2 1-羟化酶缺乏症,5例为遗传代谢病患儿,6例患儿预防接种前原发病未获诊。虽然疫苗本身不是致病原因,但诱发了急性发作。建议对营养发育异常的患儿慎重接种,及早进行详细病因调查,明确原发病。

 
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