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Effects of hemin and hemodialysis in a patient with acute intermittent porphyria and renal failure.氯高铁血红素与血液透析对一名急性间歇性卟啉病合并肾衰竭患者的影响。
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Plasma porphobilinogen as a sensitive biomarker to monitor the clinical and therapeutic course of acute intermittent porphyria attacks.血浆胆色素原作为监测急性间歇性卟啉病发作的临床及治疗过程的敏感生物标志物。
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[Acute intermittent porphyria].[急性间歇性卟啉病]
Minerva Med. 1983 Nov 10;74(43):2605-9.
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[A case of acute intermittent porphyria].[一例急性间歇性卟啉病]
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本文引用的文献

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Acute Intermittent Porphyria: Predicted Pathogenicity of HMBS Variants Indicates Extremely Low Penetrance of the Autosomal Dominant Disease.急性间歇性卟啉症：HMBS基因变异的预测致病性表明常染色体显性疾病的外显率极低。
Hum Mutat. 2016 Nov;37(11):1215-1222. doi: 10.1002/humu.23067. Epub 2016 Sep 5.
2
An update of clinical management of acute intermittent porphyria.急性间歇性卟啉症临床管理的最新进展。
Appl Clin Genet. 2015 Sep 1;8:201-14. doi: 10.2147/TACG.S48605. eCollection 2015.
3
High prevalence of and potential mechanisms for chronic kidney disease in patients with acute intermittent porphyria.急性间歇性血卟啉症患者慢性肾脏病的高患病率和潜在机制。
Kidney Int. 2015 Aug;88(2):386-95. doi: 10.1038/ki.2015.97. Epub 2015 Apr 1.
4
Acute porphyrias in the USA: features of 108 subjects from porphyrias consortium.美国的急性卟啉病：来自卟啉病联盟的108名受试者的特征
Am J Med. 2014 Dec;127(12):1233-41. doi: 10.1016/j.amjmed.2014.06.036. Epub 2014 Jul 10.
5
RNAi-mediated silencing of hepatic Alas1 effectively prevents and treats the induced acute attacks in acute intermittent porphyria mice.RNA干扰介导的肝脏δ-氨基-γ-酮戊酸合酶1沉默可有效预防和治疗急性间歇性卟啉症小鼠的诱导性急性发作。
Proc Natl Acad Sci U S A. 2014 May 27;111(21):7777-82. doi: 10.1073/pnas.1406228111. Epub 2014 May 12.
6
Porphyrin precursors and porphyrins in three patients with acute intermittent porphyria and end-stage renal disease under different therapy regimes.三名患有急性间歇性卟啉病和终末期肾病的患者在不同治疗方案下的卟啉前体和卟啉
Cell Mol Biol (Noisy-le-grand). 2009 Feb 16;55(1):66-71.
7
Hemodialysis: a therapeutic option for severe attacks of acute intermittent porphyria in developing countries.血液透析：发展中国家急性间歇性卟啉症严重发作的一种治疗选择。
Hemodial Int. 2008 Jan;12(1):34-8. doi: 10.1111/j.1542-4758.2008.00237.x.
8
The syndrome of inappropriate antidiuretic hormone secretion (SIADH) and neurological crisis due to acute intermittent porphyria, successfully treated with haemodialysis.抗利尿激素分泌不当综合征（SIADH）及急性间歇性卟啉症所致的神经危机，经血液透析成功治愈。
J Assoc Physicians India. 2007 Sep;55:667-9.
9
Recommendations for the diagnosis and treatment of the acute porphyrias.急性卟啉病的诊断与治疗建议。
Ann Intern Med. 2005 Mar 15;142(6):439-50. doi: 10.7326/0003-4819-142-6-200503150-00010.
10
Charcoal haemoperfusion and haemodialysis in acute intermittent porphyria.急性间歇性卟啉病的活性炭血液灌流和血液透析
Br Med J (Clin Res Ed). 1983 Dec 10;287(6407):1746-7. doi: 10.1136/bmj.287.6407.1746.

氯高铁血红素与血液透析对一名急性间歇性卟啉病合并肾衰竭患者的影响。

Effects of hemin and hemodialysis in a patient with acute intermittent porphyria and renal failure.

作者信息

Attarian Shirin, Yu Chunli, Anderson Karl E, Friedman Ellen W

机构信息

Division of Hematology/Oncology, Department of Medicine, Albert Einstein College of Medicine, Montefiore Medical Center, Bronx, NY.

Department of Genetics and Genomic Sciences, The Icahn School of Medicine at Mount Sinai, New York, NY; and.

出版信息

Blood Adv. 2017 Jun 5;1(14):915-917. doi: 10.1182/bloodadvances.2017005660. eCollection 2017 Jun 13.

DOI:10.1182/bloodadvances.2017005660
PMID:29296735
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5737596/
Abstract

Hemin and hemodialysis had an additive effect in decreasing ALA and PBG in our patient with acute intermittent porphyria and renal failure.The time course of ALA and PBG reaccumulation after hemodialysis is not known.

摘要

在我们这位患有急性间歇性卟啉病和肾衰竭的患者中，高铁血红素和血液透析在降低δ-氨基-γ-酮戊酸（ALA）和卟胆原（PBG）方面具有相加作用。血液透析后ALA和PBG重新蓄积的时间进程尚不清楚。