Acute intermittent porphyria in children

Acute intermittent porphyria (AIP), also known as hepatic porphyria and pyrrole porphyria, is a more common form of porphyria. First reported by Gunther in 1911, Waldenstrom comprehensively described the disease on the basis of extensive family research in 1937. The disease is autosomal dominant. The main clinical manifestations are abdominal pain, peripheral nerve damage, hypothalamic lesions, mental disorders, and light-sensitive skin damage. , The former is called photo-contact dermatitis, the latter is photo-drug dermatitis) and a large amount of ALA and porphyrin are excreted in urine.