Recommendations for the diagnosis and treatment of the acute porphyrias.
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An update of clinical management of acute intermittent porphyriaAcute Hepatic PorphyriaErythroid heme biosynthesis and its disordersRenal failure affects the enzymatic activities of the three first steps in hepatic heme biosynthesis in the acute intermittent porphyria mouseNeuroglobin, a Factor Playing for Nerve Cell SurvivalHeme deficiency in Alzheimer's disease: a possible connection to porphyriaRole of delta-aminolevulinic acid in the symptoms of acute porphyriaRNAi-mediated silencing of hepatic Alas1 effectively prevents and treats the induced acute attacks in acute intermittent porphyria miceHepatic heme-regulated inhibitor (HRI) eukaryotic initiation factor 2alpha kinase: a protagonist of heme-mediated translational control of CYP2B enzymes and a modulator of basal endoplasmic reticulum stress toneA 22-year-old woman with abdominal pain.Heme acts through the Bach1b/Nrf2a-MafK pathway to regulate exocrine peptidase precursor genes in porphyric zebrafishFirst-in-human study demonstrating pharmacological activation of heme oxygenase-1 in humansHemin-activated macrophages home to the pancreas and protect from acute pancreatitis via heme oxygenase-1 inductionLow-dose hydroxychloroquine is as effective as phlebotomy in treatment of patients with porphyria cutanea tarda.Systemic hemin therapy attenuates blood-brain barrier disruption after intracerebral hemorrhage.Heme oxygenase-1 is induced in peripheral blood mononuclear cells of patients with acute pancreatitis: a potential therapeutic targetAcute intermittent porphyria caused by novel mutation in HMBS gene, misdiagnosed as cholecystitis.Cytochrome P450 regulation: the interplay between its heme and apoprotein moieties in synthesis, assembly, repair, and disposal.Sustained enzymatic correction by rAAV-mediated liver gene therapy protects against induced motor neuropathy in acute porphyria mice.Clinically important features of porphyrin and heme metabolism and the porphyrias.Acute intermittent porphyria associated with respiratory failure: a multidisciplinary approach.Lon peptidase 1 (LONP1)-dependent breakdown of mitochondrial 5-aminolevulinic acid synthase protein by heme in human liver cells.Acute porphyria in a patient with Arnold Chiari malformation.Cardiac calcification in acute intermittent porphyriaA LC-MS/MS method for the specific, sensitive, and simultaneous quantification of 5-aminolevulinic acid and porphobilinogen.Carbon monoxide reverses diabetic gastroparesis in NOD mice.Schizoaffective disorder with missed diagnosis of acute porphyria: a case report and overview.Liver transplantation in the management of porphyria.A method for determining δ-aminolevulinic acid synthase activity in homogenized cells and tissues.Acute porphyrias in the USA: features of 108 subjects from porphyrias consortium.Acute intermittent porphyria presenting with posterior reversible encephalopathy syndrome and lateralized periodic discharges plus fast activity on EEG.One ring to rule them all: trafficking of heme and heme synthesis intermediates in the metazoansPorphyria Diagnostics-Part 1: A Brief Overview of the Porphyrias.Liver transplantation for acute intermittent porphyria is complicated by a high rate of hepatic artery thrombosis.Guide to drug porphyrogenicity prediction and drug prescription in the acute porphyrias.Dual porphyrias revisited.Loss-of-function ferrochelatase and gain-of-function erythroid-specific 5-aminolevulinate synthase mutations causing erythropoietic protoporphyria and x-linked protoporphyria in North American patients reveal novel mutations and a high prevalence ofLiver transplantation for porphyria: who, when, and how?Preclinical Development of a Subcutaneous ALAS1 RNAi Therapeutic for Treatment of Hepatic Porphyrias Using Circulating RNA Quantification.Circadian rhythms in acute intermittent porphyria--a pilot study.
P2860
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P2860
Recommendations for the diagnosis and treatment of the acute porphyrias.
description
2005 nî lūn-bûn
@nan
2005 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2005 թվականի մարտին հրատարակված գիտական հոդված
@hy
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
name
Recommendations for the diagnosis and treatment of the acute porphyrias.
@ast
Recommendations for the diagnosis and treatment of the acute porphyrias.
@en
type
label
Recommendations for the diagnosis and treatment of the acute porphyrias.
@ast
Recommendations for the diagnosis and treatment of the acute porphyrias.
@en
prefLabel
Recommendations for the diagnosis and treatment of the acute porphyrias.
@ast
Recommendations for the diagnosis and treatment of the acute porphyrias.
@en
P2093
P1476
Recommendations for the diagnosis and treatment of the acute porphyrias.
@en
P2093
Claus A Pierach
Herbert L Bonkovsky
James P Kushner
Joseph R Bloomer
Karl E Anderson
Neville R Pimstone
Robert J Desnick
P304
P356
10.7326/0003-4819-142-6-200503150-00010
P407
P577
2005-03-01T00:00:00Z