Description The acute hepatic porphyrias (AHP) are rare, inborn errors of heme-metabolism
and include acute intermittent porphyria, hereditary coproporphyria, variegate porphyria …

Acute intermittent porphyria (AIP) is an autosomal dominant inherited disease with low
clinical penetrance, caused by mutations in the hydroxymethylbilane synthase (HMBS) …

Background The acute hepatic porphyrias (AHP) are associated with a risk of primary liver
cancer (PLC), but risk estimates are unclear, and what AHP characteristics that predict PLC …

Recurrent attacks of acute intermittent porphyria (AIP) result in poor quality of life and
significant risks of morbidity and mortality. Liver transplantation (LT) offers a cure, but …

Acute intermittent porphyria (AIP) is a rare hereditary metabolic disease characterized by
acute attacks and accumulation of the porphyrin precursors 5‐aminolevulinic acid (ALA) and …

Conclusion In this US study, 1.5% of patients with AHP had HCC. HCC in AHP occurred in
the absence of cirrhosis, which contrasts with other chronic liver diseases. Patients with …

Background Acute intermittent porphyria (AIP) is a genetic disease characterized by acute
neurovisceral attacks. Long-term clinical conditions, chronic symptoms and impaired health …

Simple Summary Porphyria is a metabolic condition which leads to reduced heme
production. While it involves multiple organs systems, porphyria affecting the liver can lead …

The acute hepatic porphyrias (AHP) are associated with long‐term complications such as
primary liver cancer, hypertension, and chronic kidney disease. Data on other related …

Background Acute hepatic porphyria includes four inherited disorders caused by partial
deficiencies of enzymes related to the heme biosynthesis. Clinical manifestations include …