EXPLORE B: A prospective, long‐term natural history study of patients with acute hepatic porphyria with chronic symptoms
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Inherited Metabolic Disease"
DOI: 10.1002/jimd.12551
Abstract: One‐year data from EXPLORE Part A showed high disease burden and impaired quality of life (QOL) in patients with acute hepatic porphyria (AHP) with recurrent attacks. We report baseline data of patients who enrolled in… read more here.
Keywords: long term; acute hepatic; disease; chronic symptoms ... See more keywords
IL-27 is elevated in sepsis with acute hepatic injury and promotes hepatic damage and inflammation in the CLP model.
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DOI: 10.1016/j.cyto.2019.154936
Abstract: BACKGROUND Immuno-inflammation plays an important role in the pathophysiological process of sepsis-associated acute hepatic injury (AHI). Interleukin 27 (IL-27) is an important inflammatory regulator; however, its role in this condition is not clear. METHODS The… read more here.
Keywords: acute hepatic; serum; elevated sepsis; injury ... See more keywords
Polyphenols from Morchella angusticepes Peck attenuate D-galactosamine/lipopolysaccharide-induced acute hepatic failture in mice
Sign Up to like & getrecommendations! Published in 2019 at "Journal of Functional Foods"
DOI: 10.1016/j.jff.2019.04.064
Abstract: Abstract We investigated the effect of polyphenols extracted from M. angusticepes Peck (PMP) on D-galactosamine/lipopolysaccharide (D-GalN/LPS) induced acute hepatic failure (AHF) in mice. Results indicated that administration of PMP relieved the leakage of serum alanine… read more here.
Keywords: angusticepes peck; mice; acute hepatic; induced acute ... See more keywords
Acute hepatic porphyrias: Identification of 46 hydroxymethylbilane synthase, 11 coproporphyrinogen oxidase, and 20 protoporphyrinogen oxidase novel mutations.
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DOI: 10.1016/j.ymgme.2018.10.008
Abstract: The acute hepatic porphyrias (AHPs) are inborn errors of heme biosynthesis, which include three autosomal dominant porphyrias, Acute Intermittent Porphyria (AIP), Hereditary Coproporphyria (HCP), and Variegate Porphyria (VP), and the ultra-rare autosomal recessive porphyria, δ-Aminolevulinic… read more here.
Keywords: hydroxymethylbilane synthase; oxidase; acute hepatic; hepatic porphyrias ... See more keywords
Givosiran for the treatment of acute hepatic porphyria
Sign Up to like & getrecommendations! Published in 2022 at "Expert Review of Clinical Pharmacology"
DOI: 10.1080/17512433.2022.2075848
Abstract: ABSTRACT Introduction Acute hepatic porphyrias (AHPs) are a family of rare inherited disorders characterized by enzyme dysfunctions in the hepatic pathway of heme biosynthesis. In AHPs, accumulation of the neurotoxic porphyrin precursors delta-aminolevulinic acid and… read more here.
Keywords: givosiran; acute hepatic; hepatic porphyria; givosiran treatment ... See more keywords
Disodium Guanylate Alleviates Acute Hepatic Injury Induced by Carbon Tetrachloride Via Antioxidative Stress and Antiapoptosis In Vivo and In Vitro.
Sign Up to like & getrecommendations! Published in 2019 at "Journal of food science"
DOI: 10.1111/1750-3841.14677
Abstract: Hepatic injury is one of the most common digestive system diseases worldwide in clinic. Guanylic acid or guanosine monophosphate (GMP) was an important component of nucleotides, which is mainly in the form of sodium salt… read more here.
Keywords: acute hepatic; gmp na2; disodium guanylate; injury ... See more keywords
Transfusion-related acute hepatic injury following postoperative platelets administration in pediatric patients undergoing the Fontan procedure.
Sign Up to like & getrecommendations! Published in 2019 at "Congenital heart disease"
DOI: 10.1111/chd.12825
Abstract: OBJECTIVE The final common pathway of single ventricle patients is the Fontan procedure. Among the immediate postoperative complications is acute hepatic injury presented by marked elevation of liver enzymes (alanine transaminase [ALT] and aspartate transaminase… read more here.
Keywords: acute hepatic; fontan procedure; transfusion; injury ... See more keywords
Acute hepatic porphyria and maternal health: Clinical and biochemical follow-up of 44 pregnancies.
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DOI: 10.1111/joim.13376
Abstract: BACKGROUND Pregnancy in women with acute hepatic porphyria (AHP) has historically been associated with significant morbidity. Clinical outcomes have been the focus of previous reports on porphyria and maternal health, with little data available on… read more here.
Keywords: acute hepatic; pregnancy; porphyria maternal; hepatic porphyria ... See more keywords
Efficacy and safety of givosiran for acute hepatic porphyria: 24‐month interim analysis of the randomized phase 3 ENVISION study
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DOI: 10.1111/liv.15090
Abstract: Upregulation of hepatic delta‐aminolevulinic acid synthase 1 with accumulation of potentially toxic heme precursors delta‐aminolevulinic acid and porphobilinogen is fundamental to the pathogenesis of acute hepatic porphyria. Aims: evaluate long‐term efficacy and safety of givosiran… read more here.
Keywords: acute hepatic; efficacy safety; givosiran acute; hepatic porphyria ... See more keywords
Update on the diagnosis and management of the autosomal dominant acute hepatic porphyrias
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DOI: 10.1136/jclinpath-2021-207647
Abstract: The autosomal dominant acute hepatic porphyrias (AHPs), acute intermittent porphyria, hereditary coproporphyria (HCP) and variegate porphyria (VP), are low penetrance adult onset disorders caused by partial deficiency of enzymes of haem biosynthesis. All are associated… read more here.
Keywords: acute hepatic; diagnosis; hepatic porphyrias; autosomal dominant ... See more keywords
Protective effect of baicalin against arsenic trioxide-induced acute hepatic injury in mice through JAK2/STAT3 signaling pathway
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DOI: 10.1177/20587384211073397
Abstract: Baicalin (BA) is a kind of flavonoid that is isolated from Scutellaria baicalensis Georgi, which has been verified to have hepatoprotective effects in some diseases. However, the role of BA in acute hepatic injury induced… read more here.
Keywords: acute hepatic; stat3 signaling; injury; mice ... See more keywords