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Isolation of an hydroxylated porphyrin from urine and faeces of patients with cutaneous hepatic porphyria

✍ Scribed by Elder, G.H.; Chapman, J.R.

Book ID
123262506
Publisher
Elsevier Science
Year
1970
Tongue
English
Weight
177 KB
Volume
208
Category
Article
ISSN
0304-4165

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Congenital erythropoietic porphyria is a rare genetic disorder in which deficiency of uroporphyrinogen III synthase results in excessive production of Type I porphyrins. The main clinical features are severe photodestruction of the skin and haemolytic anaemia. Treatment consists of shielding from li