检索结果-南通市图书馆

A study on α-ketoadipic aciduria by gas chromatographic-mass spectrometry

World Journal of Gastroenterology 2000年第5期6卷 766-769页

作者： Yoshito Inoue Morimasa Ohse Toshihiro Shinka Tomiko Kuhara Division of Human Genetics Medical Research InstituteKanazawa Medical UniversityJapan

INTRODUCTIONα-ketoadipate(α-KA),an intermediate in thecatabolism of L-lysine,hydroxylysine,and L-tryptophan,undergoes oxidative deearboxylation toform glutaryl-CoA and then dehydrogenates to formcrotonyl-CoA,the latter undergoes furtherdegradation and enters in TCA cycle,as shown inFigure 1.α-ketoadipic aciduria (Mckusick 245130)is a rare inborn error in the metabolism of α-KA

关键词： L-lysine hydroxylysine Ltryptophan alpha-aminoadipic alpha-ketoadipic aciduria metabolism inborn error mass fragmentography chromatography gas

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Metabolic acidosis in late pregnancy due to 5-oxoproline (pyroglutamic acid)—A case report

引用

Open Journal of Obstetrics and Gynecology 2012年第2期2卷 170-173页

作者： Sahathevan Sathiyathasan K. Jeyanthan R. Hamid Mayday University Hospital Croydon Surrey UK

Introduction: Accumulation of 5-oxoproline (pyroglutamic acid) is a rare cause of severe, high anion gap metabolic acidosis in adults. Case: A 21 year old lady presented at 39 weeks gestation in her first pregnancy with 2 weeks history of shortness of breath. She suffered from ear ache and had been taking Paracetamol on regular basis for a year. She was admitted to having regular alcohol intake until the pregnancy when she stopped. She was not in acute distress and all her observations were stable. The laboratory analysis of renal function was normal. Arterial blood gas showed metabolic acidosis. The anion gap 34 mEq/l which was consistent with metabolic acidosis. Plasma aminoacid screen revealed no abnormality. Chest X-rays showed patchy consolidation keeping with a chest infection. Emergency caesarean section was carried out for fetal distress in first stage of labour. Urine and serum samples were taken which showed an increase in the serum level five Oxoproline to creatinine ratio indicating pyroglutamic metabolic acidosis. Polyglutamate was found in the urine She was admitted to intensive care unit;cardiovascular stable, had no signs of sepsis. She underwent ventilation and haemofiltration. On second day of admission metabolic acidosis was corrected. During the 48 hours treatment with supportive therapy produced general overall improvement. Discussion: Metabolic acidosis that caused by 5-oxoproline results from disruption of the gamma glutamyl cycle. Glutamile synthetase (GS) deficiency is an autosomol recessive disorder. With GS deficiency, reduced glutathione levels increase gamma glutamile synthetase activity, and the resulting gamma—glutamile cystine levels are particularly converted to 5-oxoproline. Intoxication of organic acids is a differential diagnosis of high anion gap metabolic acidosis with no renal impairment, there was no evidence of ethanol, methanol or ethylene glycol ingestion. Suspicion for 5-oxoproline-associated high anion gap metabolic acido

关键词： Metabolic Acidosis Anion Gap Acetaminophen Pyroglutamic aciduria Pregnancy

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

欢迎您,

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

请选择保存的检索档案：

请选择收藏分类：

通借通还

欢迎您,

建议与咨询 留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

请选择保存的检索档案： 新增检索档案 确定 取消

请选择收藏分类： 新增自定义分类 确定 取消

通借通还

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

请选择保存的检索档案：

请选择收藏分类：