Acute hepatic porphyria

Acute hepatic porphyria encompasses a group of rare, inherited metabolic disorders—primarily acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, and ALAD deficiency porphyria—caused by enzymatic deficiencies in the hepatic heme biosynthetic pathway

Acute hepatic porphyria encompasses a group of rare, inherited metabolic disorders—primarily acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, and ALAD deficiency porphyria—caused by enzymatic deficiencies in the hepatic heme biosynthetic pathway The condition predominantly affects adults, with symptomatic episodes occurring more frequently in females during their reproductive years Pathophysiologically, hepatic heme deficiency leads to the upregulation of delta-amino...

The condition predominantly affects adults, with symptomatic episodes occurring more frequently in females during their reproductive years

This overproduction precipitates acute, potentially life-threatening neurovisceral attacks characterized by severe abdominal pain, autonomic dysfunction, peripheral neuropathy, and neuropsychiatric disturbances, with select subtypes also presenting with cutaneous photosensitivity

This overproduction precipitates acute, potentially life-threatening neurovisceral attacks characterized by severe abdominal pain, autonomic dysfunction, peripheral neuropathy, and neuropsychiatric disturbances, with select subtypes also presenting with cutaneous photosensitivity