A CASE OF ANÆMIA, CONSEQUENT UPON LUNG DISEASE AND STARVATION, TREATED BY TRANSFUSION OF BLOOD AND SALINE FLUID

Porphyrins in urine, plasma, erythrocyte

Porphyrins in urine, plasma, erythrocytes, bile and faeces in a case of congenital erythropoietic porphyria (Gunther’s disease) treated with blood transfusion and iron chelation: lack of benefit from oral charcoal

✍ Abel Gorchein; Rong Guo; Chang Kee Lim; Ana Raimundo; Humphrey W. H. Pullon; Ala 📂 Article 📅 1998 🏛 John Wiley and Sons 🌐 English ⚖ 155 KB 👁 1 views

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