Hepatic porphyrias is a form of porphyria in which toxic porphyrin molecules build up in the liver. Hepatic porphyrias can result from a number of different...
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mutation. Hepatic porphyrias are those in which the enzyme deficiency occurs in the liver. Hepatic porphyrias include acute intermittent porphyria (AIP),...
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cause of hepatic porphyria, meaning that excess porphyrins originate from the liver rather than the bone marrow as in erythropoietic porphyrias. The clinical...
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Hereditary coproporphyria (redirect from Congenital hepatic porphyria)
(HCP) is a disorder of heme biosynthesis, classified as an acute hepatic porphyria. HCP is caused by a deficiency of the enzyme coproporphyrinogen oxidase...
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Variegate porphyria, also known by several other names, is an autosomal dominant porphyria that can have acute (severe but usually not long-lasting) symptoms...
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Hepatocellular carcinoma (redirect from Hepatic tumor)
Acute and chronic hepatic porphyrias (acute intermittent porphyria, porphyria cutanea tarda, hereditary coproporphyria, variegate porphyria) and tyrosinemia...
90 KB (10,115 words) - 23:42, 7 July 2024
Givlaari, is a medication used for the treatment of adults with acute hepatic porphyria. Givosiran is a small interfering RNA (siRNA) directed towards delta-aminolevulinate...
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are associated with significant sedation. Clonazepam may aggravate hepatic porphyria. Clonazepam is not recommended for patients with chronic schizophrenia...
78 KB (7,580 words) - 02:30, 11 August 2024
Acute intermittent porphyria (AIP) is a rare metabolic disorder affecting the production of heme resulting from a deficiency of the enzyme porphobilinogen...
25 KB (2,873 words) - 22:35, 31 March 2024
Hepatoerythropoietic porphyria is a very rare form of hepatic porphyria caused by a disorder in both genes which code Uroporphyrinogen III decarboxylase...
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Porphyria cutanea tarda is the most common subtype of porphyria. The disease is named because it is a porphyria that often presents with skin manifestations...
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(creatinine clearance <30 ml/min) Caution in patients with pre-existing hepatic porphyria, as diclofenac may trigger attacks Caution in patients with severe...
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Erythropoietic porphyria is a type of porphyria associated with erythropoietic cells. In erythropoietic porphyrias, the enzyme deficiency occurs in the...
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and EMA approved givosiran for the treatment of adults with acute hepatic porphyria (AHP). The FDA also granted givosiran a breakthrough therapy designation...
141 KB (15,474 words) - 20:41, 30 July 2024
the second RNAi therapy, Givlaari (givosiran) used to treat acute hepatic porphyria (AHP). The disease is caused due to the accumulation of toxic porphobilinogen...
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deficiencies; glucose-6-phosphate dehydrogenase deficiency; acute hepatic porphyria; alcoholism; gastrointestinal ulcers or bleeding; pregnancy and lactation;...
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and apolipoproteins A and B. It was first reported to exacerbate hepatic porphyria in 1975. In 1981, phenobarbital, one of primidone's metabolites, was...
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limbs defects Congenital hemolytic anemia Congenital hepatic fibrosis Congenital hepatic porphyria Congenital herpes simplex Congenital hypomyelination...
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Harderoporphyria (category Porphyrias)
Mutations in CPOX usually cause hereditary coproporphyria, an acute hepatic porphyria, however the K404E mutation in a homozygous or compound heterozygous...
5 KB (446 words) - 05:09, 3 December 2023
Novartis sickle cell disease Givosiran Alnylam Pharmaceuticals acute hepatic porphyria Acalabrutinib AstraZeneca relapsed or refractory chronic lymphocytic...
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Erythropoietic protoporphyria (category Porphyrias)
Erythropoietic protoporphyria (or commonly called EPP) is a form of porphyria, which varies in severity and can be very painful. It arises from a deficiency...
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gene can cause increased sensitivity to lead poisoning and acute hepatic porphyria. Alternatively spliced transcript variants encoding different isoforms...
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sclerosing cholangitis. Haemochromatosis Cholesteryl ester storage disease Porphyria Wilson's disease Niemann–Pick disease Non-alcoholic fatty liver disease...
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the preparation Urea cycle disorders Hepatic porphyria Hepatotoxicity Mitochondrial disease Pancreatitis Porphyria Pregnancy (except when no other treatments...
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calcinosis Variegate porphyria (mixed hepatic porphyria, mixed porphyria, South African genetic porphyria, South African porphyria) Verruciform xanthoma...
198 KB (17,958 words) - 01:24, 6 July 2024
porphyrin metabolism in hepatocyte culture: relationship with human acute hepatic porphyrias" (PDF). J Clin Chem Clin Biochem. 23 (9): 505–13. doi:10.1515/cclm...
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Others: changes in weight, reduced carbohydrate tolerance, worsening of porphyria, edema, arthralgias, bronchitis, leg cramps, hemorrhoids, urticaria, angioedema...
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2018). Givosiran (Givlaari) for the treatment of adults with acute hepatic porphyria, administered by administered by intravenous infusion of siRNA-GalNAc...
78 KB (8,788 words) - 19:06, 6 August 2024
inherited hepatic porphyrias with a predisposition to hepatocellular carcinoma. Typical risk factors for HCC need not be present with the acute hepatic porphyrias...
123 KB (13,421 words) - 14:50, 1 August 2024