Haem arginate in acute hereditary coproporphyria.
An 11 year old boy presented with severe acute hereditary coproporphyria. Despite supportive measures his condition deteriorated after admission. Haem arginate, started two days after presentation, produced appreciable inhibition of porphyrin precursor overproduction and clinical improvement.
Manning
D J
DJ
Department of Paediatrics, Northern General Hospital, Sheffield.
Gray
T A
TA
D001120:Arginine / Q000627:therapeutic use*; D002648:Child; D003306:Coproporphyrins / Q000652:urine*; D006418:Heme / Q000627:therapeutic use*; D006801:Humans; D008107:Liver Diseases / Q000188:drug therapy* / Q000235:genetics / Q000652:urine; D008297:Male; D011164:Porphyrias / Q000188:drug therapy / Q000652:urine*
D003306:Coproporphyrins; D006418:Heme; D001120:Arginine; C048849:heme arginate