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Elagolix is porphyrogenic and may induce porphyric attacks in patients with the acute hepatic porphyrias.
Mol Genet Metab Rep
December 2022
Department of Internal Medicine, Section on Gastroenterology and Hepatology, Wake Forest University School of Medicine, Winston-Salem, NC, USA.
Elagolix is an FDA-approved treatment for moderate-to-severe pain associated with endometriosis but has been associated with increased acute porphyric attacks in women with the acute hepatic porphyrias (AHPs). A fluorescence-based screening assay for drug porphyrogenicity in LMH cells indicates that elagolix is porphyrogenic; thus, elagolix should be avoided or used with caution in patients with the AHPs.
View Article and Find Full Text PDFCirculating fluorocytes at the first attack of acute intermittent porphyria: a missing link in the pathogenesis.
Clin Chim Acta
January 2011
Department of Pathology, The University of Hong Kong, Queen Mary Hospital, Hong Kong, China.
Background: Acute intermittent porphyria (AIP) is an autosomal dominant disorder of the haem biosynthesis resulting from a partial deficiency of hydroxymethylbilane synthase (HMBS) with incomplete penetrance. By conventional means, it is able to identify asymptomatic mutation carrier by molecular diagnosis, but one cannot reliably predict an acute porphyric attack. The presence of fluorescent red cells (fluorocytes) in AIP is probably under-recognized since AIP is a hepatic porphyria and not associated with photosensitivity.
View Article and Find Full Text PDFThe dual face of endogenous alpha-aminoketones: pro-oxidizing metabolic weapons.
Comp Biochem Physiol C Toxicol Pharmacol
October 2007
Departamento de Bioquímica, Instituto de Química, Universidade de São Paulo, Av. Prof. Lineu Prestes 748, 05508-900, São Paulo, SP, Brazil.
Amino metabolites with potential prooxidant properties, particularly alpha-aminocarbonyls, are the focus of this review. Among them we emphasize 5-aminolevulinic acid (a heme precursor formed from succinyl-CoA and glycine), aminoacetone (a threonine and glycine metabolite), and hexosamines and hexosimines, formed by Schiff condensation of hexoses with basic amino acid residues of proteins. All these metabolites were shown, in vitro, to undergo enolization and subsequent aerobic oxidation, yielding oxyradicals and highly cyto- and genotoxic alpha-oxoaldehydes.
View Article and Find Full Text PDFErythropoietin treatment in the neuropsychiatric porphyrias.
Clin Chim Acta
December 2003
Department of Medicine, King's College Hospital, London, UK.
Background: In a previous report, 31 patients with neuropsychiatric porphyria were studied and nine of these patients were anaemic in association with inappropriately low serum erythropoietin levels. We were also able to demonstrate that treatment with erythropoietin in non-porphyric patients (mainly diabetic patients with autonomic neuropathy) significantly reduced urinary delta-aminolaevulinic acid levels.
Methods: We treated six porphyric patients, five of whom were anaemic, with recombinant human erythropoietin (1000-2000 IU thrice weekly).
Serum erythropoietin levels may be inappropriately low in the acute neuropsychiatric porphyrias.
Clin Chim Acta
March 2002
Department of Medicine, King's College Hospital, Denmark Hill, SE5 9RS, London, UK.
Background: Patients with the acute porphyrias may develop renal failure and autonomic dysfunction. Renal damage and sympathetic failure may both cause erythropoietin (EPO) deficiency. In this study, we have investigated serum erythropoietin levels and autonomic function in patients with acute porphyria in clinical remission.
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