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Novel treatment options for acute hepatic porphyrias
Current Opinion in Gastroenterology, 2021Purpose of review Acute hepatic porphyrias (AHP) are a group of rare diseases that are characterized by episodic acute neurovisceral pain episodes caused by abnormal accumulation of the neurotoxic porphyrin precursor delta-aminolevulinic acid (ALA).
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Givosiran: A Review in Acute Hepatic Porphyria
Drugs, 2021Givosiran (Givlaari®) is an δ-aminolevulinic acid synthase 1 (ALAS1)-directed small interfering RNA (siRNA) approved for the treatment of acute hepatic porphyria (AHP). In the phase 3 ENVISION trial, givosiran significantly reduced the annualized rate of composite porphyria attacks (i.e.
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1981
The acute hepatic porphyrias are the result of hereditary partial deficiencies of individual enzymes in the pathway of haem biosynthesis. Seven enzymes are known to be involved in the pathway, converting glycine and succinyl CoA first to porphyrin precursors and then to porphyrins and finally to haem. The rate of the process is regulated by the initial
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The acute hepatic porphyrias are the result of hereditary partial deficiencies of individual enzymes in the pathway of haem biosynthesis. Seven enzymes are known to be involved in the pathway, converting glycine and succinyl CoA first to porphyrin precursors and then to porphyrins and finally to haem. The rate of the process is regulated by the initial
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The “glucose effect” in acute hepatic porphyrias and in experimental porphyria
Klinische Wochenschrift, 1981The "glucose effect" was investigated in human acute hepatic porphyrias (acute intermittent porphyria, variegate porphyria, coproporphyria and porphobilinogen synthase defect porphyria) and in avian liver cells. 8 patients (7 women) with acute abdominal-neurological porphyria syndrome and 3 patients (2 women) in the remission phase were treated with ...
M, Doss, F, Verspohl
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Treatment of acute hepatic porphyria with hematin
Journal of Hepatology, 1988The efficacy of hematin has been evaluated in eight patients with acute intermittent porphyria: six with acute attacks and two with chronic subacute symptoms. Hematin suppressed the chemical signs of porphyria in all patients and the symptoms in those with acute attacks.
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[Anesthesia and acute hepatic porphyrias].
Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie : AINS, 1992Acute hepatic porphyrias are inherited disorders of heme biosynthesis. A characteristic feature of acute porphyrias is the occurrence of acute attacks which are often precipitated by drugs. Many of the commonly used anaesthetic agents may trigger such a potentially lethal attack.
H, Böhrer, H, Schmidt, E, Martin
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Acute hepatic porphyrias—A guide for hepatologists
HepatologyThe acute hepatic porphyrias (AHPs) are a group of rare, inherited disorders of the heme biosynthesis pathway, usually manifesting with attacks of acute abdominal pain and other neurovisceral symptoms, with or without cutaneous manifestations. AHPs are characterized by the accumulation of porphyrin precursors, porphobilinogen, and/or aminolevulinic ...
Akshata Moghe +2 more
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[Acute hepatic porphyria and Diprivan].
Annales francaises d'anesthesie et de reanimation, 1995Propofol may be used without any restriction in all cases of asymptomatic hepatic porphyria, as a bolus injection for induction and maintenance of anaesthesia and as a continuous infusion to produce anaesthesia of short and medium duration. If in doubt as to the absolute safety of propofol in case of symptomatic hepatic porphyria, preference has to be ...
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Acute hepatic porphyrias: pathophysiology and pathogenesis of acute attacks
Rinsho ShinkeigakuHeme is an iron-containing molecule essential for virtually all living organisms. However, excessive heme is cytotoxic, necessitating tight regulation of intracellular heme concentration. The acute hepatic porphyrias (AHPs) are a group of rare inborn errors of heme biosynthesis that are characterized by episodic acute neurovisceral attacks that are ...
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