Thelansis

 Acute Hepatic Porphyria (AHP) is a rare subgroup of porphyrias characterized by the occurrence of neurovisceral attacks with or without cutaneous manifestations. Acute hepatic porphyrias encompass four diseases: acute intermittent porphyria (the most common), variegate porphyria, hereditary coproporphyria, and hereditary deficit of delta-aminolevulinic acid dehydratase (extremely rare). Each acute hepatic porphyria results from a deficiency of one of the enzymes in the heme biosynthesis pathway. These deficiencies result in an accumulation of the precursors of porphyrins in the liver (delta-aminolevulinic acid, ALA, and porphobilinogen, PBG) and also, in the case of variegate porphyria and hereditary coproporphyria, an accumulation of porphyrins resulting in cutaneous manifestations. ·        In the United States, the prevalence of porphyria is approximately 1 in 25,000 individuals. The worldwide prevalence ranges from one in 500 to one in 50,000 in...

 Acute Hepatic Porphyria (AHP) is a rare subgroup of porphyrias characterized by the occurrence of neurovisceral attacks with or without cutaneous manifestations. Acute hepatic porphyrias encompass four diseases: acute intermittent porphyria (the most common), variegate porphyria, hereditary coproporphyria, and hereditary deficit of delta-aminolevulinic acid dehydratase (extremely rare). Each acute hepatic porphyria results from a deficiency of one of the enzymes in the heme biosynthesis pathway. These deficiencies result in an accumulation of the precursors of porphyrins in the liver (delta-aminolevulinic acid, ALA, and porphobilinogen, PBG) and also, in the case of variegate porphyria and hereditary coproporphyria, an accumulation of porphyrins resulting in cutaneous manifestations. ·        In the United States, the prevalence of porphyria is approximately 1 in 25,000 individuals. The worldwide prevalence ranges from one in 500 to one in 50,000 in...