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Porphyrias, Hepatic

Known as: Hepatic Porphyrias, Hepatic Porphyria, Porphyrias, Hepatic [Disease/Finding] 
A group of metabolic diseases due to deficiency of one of a number of LIVER enzymes in the biosynthetic pathway of HEME. They are characterized by… 
National Institutes of Health

Related topics

Related topics

14 relations

Narrower (6)

Acute intermittent porphyriaCafe-au-lait macules with pulmonary stenosisCoproporphyriaPorphobilinogen synthase deficiency

Broader (2)

Disorders of Porphyrin MetabolismLiver diseases
In BloodMicrobiologicalchemically inducednursing therapy

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2018
Highly Cited
2018
Recurrent attacks of acute hepatic porphyria: major role of the chronic inflammatory response in the liver
Acute intermittent porphyria (AIP) is an inherited disorder of haem metabolism characterized by life‐threatening acute… 
Review
2017
Review
2017
Acute hepatic porphyrias: Recommendations for evaluation and long‐term management
The acute hepatic porphyrias are a group of four inherited disorders, each resulting from a deficiency in the activity of a… 
Review
2015
Review
2015
Acute Hepatic Porphyria
The porphyrias comprise a set of diseases, each representing an individual defect in one of the eight enzymes mediating the… 
Highly Cited
2014
Highly Cited
2014
RNAi-mediated silencing of hepatic Alas1 effectively prevents and treats the induced acute attacks in acute intermittent porphyria mice
Significance There is an unmet need for a more effective, faster-acting, and safer therapy for the life-threatening acute… 
Highly Cited
2013
Highly Cited
2013
The incidence of inherited porphyrias in Europe
Retrospective estimates of the prevalence of porphyrias have been reported but there has been no large scale prospective study of… 
Highly Cited
2009
Highly Cited
2009
Hereditary hepatic porphyrias in Finland.
The occurrence of hepatic porphyrias--acute intermittent porphyria (AIP) and variegate porphyria (VP)--in Finland has been… 
Highly Cited
1987
Highly Cited
1987
Reduction of the C2 and C4 vinyl groups of Sn-protoporphyrin to form Sn-mesoporphyrin markedly enhances the ability of the metalloporphyrin to inhibit in vivo heme catabolism.
Highly Cited
1975
Highly Cited
1975
Effects of hematin in hepatic porphyria. Further studies.
The present study was carried out in five cases of hepatic porphyria, including three of acute intermittent porphyria, one of… 
Highly Cited
1974
Highly Cited
1974
Toxicity of 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) in C57B1/6 mice.
Highly Cited
1970
Highly Cited
1970
Experimental porphyria induced in rats by hexachlorobenzene. A study of the porphyrins excreted by urine.
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