卟啉病
- 网络Porphyria;porphyrin;PCT
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目的:研究急性间歇性卟啉病(Acuteintermitentporphyria,AIP)患者卟啉代谢的变动及其相关酶活性。
Objective : To study the abnormalities in porphyrin metabolism in patients with acute intermittent porphyria ( AIP ), AIP carriers and normal healthy subjects , and their erythrocyte and liver enzyme activity .
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肝性血卟啉病合并病毒性肝炎1例
Hepatic porphyria with hepatitis B virus infection : a case report
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目的研究和比较先天性红细胞生成性卟啉病(CEP)患者、迟发性皮肤卟啉病(PCT)患者和正常健康者的红细胞酶活性。
Objective To study and compare the enzyme activities of erythrocyte in Congenital erythropoietic porphyrias ( CEP ), Porphyria cutanea tardas ( PCT ) and healthy normals subjects .
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急性间歇性卟啉病患者卟啉代谢的变动及相关酶活性的研究
Study of porphyrin metabolism and enzymes activity in acute intermittent porphyria
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大剂量维生素疗法预防尿毒症性皮肤卟啉病(德语)
High-dose vitamin therapy as prophylaxis against porphyria cutanea uremica ( Germ )
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尿卟啉谱分析在卟啉病诊断中的应用
Application of urinary porphyrin pattern analysis at diagnosis of porphyria
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迟发性皮肤卟啉病是卟啉症中最常见的一型,发病率1.4~3.5∶100000。
Porphyria cutanea tarda porphyria is the common type .
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急性间歇性卟啉病的临床分析
Acute intermittent porphyria : an analysis of five cases
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急性间歇性卟啉病伴严重低钠血症一例
A case of acute intermittent porphyria with severe hyponatremia
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两种皮肤损害性卟啉病患者酶活性的研究
The study of enzyme activity in two kinds of porphyria patients with skin lesion
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结论国内肝性卟啉病并非十分少见;
Conclusion The incidence of hepatic porphyria is not as rare as supposed before .
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国产β-胡萝卜素治疗红细胞生成性原卟啉病
β - carotene therapy for erythropoietic protoporphyria
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β-内酰胺类抗生素诱导的假卟啉病
β - Lactam antibiotic - induced pseudoporphyria
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肝性卟啉病&附1例报告
A case report on hepatic porphyria
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卟啉病是一种少见病,本文结合5例临床分析,对卟啉病的诊断、治疗及预后进行了探讨。
Porphyria is a rare disease . The diagnosis , treatment and prognosis of porphyria were discussed .
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一个南非大家族中多样性卟啉病的临床和生化表达的系统研究
A systematic study of the clinical and biochemical expression of variegate porphyria in a large South African family
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苯巴比妥过敏、有征候性或潜伏性卟啉病史者禁用苯巴比妥。
Phenobarbital is contraindicated in patients with known phenobarbital sensitivity or a history of manifest or latent porphyria .
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西班牙变异性卟啉病的基因研究:确定突变及家系携带者的检测
Genetic studies in variegate porphyria in Spain . Identification of gene mutations and family study for carrier detection
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急性间歇性卟啉病占94.44%;混合型占5.56%。
Among them , acute intermittent porphyria accounted on 94.44 % , and mixed type porphyria , 5.56 % .
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急性间歇性卟啉病:对严重纯合子显性疾病的研究为急性卟啉病神经损害提供了新视角
Acute intermittent porphyria : Studies of the severe homozygous dominant disease provides insights into the neurologic attacks in acute porphyrias
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目的探讨伴有神经、精神症状的肝性卟啉病流行病学、临床表现。
Objective To summarize the domestic epidemiology and clinical manifestations of the hepatic porphyria accompanied by neural and psychic symptoms .
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目的:建立新的卟啉化合物的快速测定法,确定卟啉病诊断用的尿卟啉谱。
Objective : To establish the method of quantitative analysis of porphyrins , and obtain the urinary porphyrin pattern for the diagnosis of porphyria .
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结果发现血卟啉病神经系统表现为周围感觉运动神经病4例、内脏自主神经病9例、抽搐发作4例及精神症状6例。
The results show that the most common neurologic manifestations of Porphyria are peripheral motor and / or sensory neuropathy , autonomic neuropathy , mental change and seizures .
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糖尿病或糖尿病倾向、高血压、静脉曲张、静脉炎、多发性硬化症、癫痫、卟啉病、手足抽搐及小舞蹈病患者慎用。
Diabetes or diabetes-prone , high blood pressure , varicose veins , phlebitis , multiple sclerosis , epilepsy , porphyria , hands and feet used with caution in patients with convulsions and small chorea .
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采用临床对照试验观察口服β-胡萝卜素治疗红细胞生成性原卟啉病的光敏反应,治疗组33例,显效率为60%,总有效率100%。
In a controlled clinical study , oral β - carotene was used to reduce the severity of photosensitivity reactions in patients with erythropoietic protoporphyria . Out of 33 cases treated with β - carotene , 60 % showed significant response , and total response rate was 100 % .