title: Renal transplantation in a case of acute intermittent porphyria.
abstract: Acute intermittent porphyria (AIP) results from a deficiency of the porphobilinogen deaminase enzyme of heme biosynthesis. The disease is exacerbated by a wide variety of drugs. Only steroids and azathioprine (Aza) have so far been considered safe in patients with AIP, and cyclosporine is listed as contraindicated. From the transplantation point of view, it is well known that cyclosporine (CsA) is a superior immunosuppressive agent compared to azathioprine. This case report presents a female patient evaluated for renal transplantation. A test dose of CsA was given, and no symptoms of AIP occurred. Renal transplantation with a cadaveric donor was performed, and the patient was immunosuppressed with CsA, Aza, and prednisolone. Following three acute rejections, Aza was replaced by mycophenolate mofetil. Nevertheless, graft function deteriorated slowly, necessitating return to dialysis 3 years after transplantation. No symptoms of AIP were observed while the patient was on cyclosporine and/or mycophenolate mofetil therapy, and the authors conclude that these drugs can be safely administered to at least some patients with AIP.
mesh_terms: D000622:Aminolevulinic Acid / Q000652:urine; D001379:Azathioprine / Q000008:administration & dosage / Q000493:pharmacokinetics / Q000627:therapeutic use; D003404:Creatinine / Q000097:blood; D016572:Cyclosporine / Q000008:administration & dosage / Q000097:blood / Q000627:therapeutic use; D004347:Drug Interactions; D005260:Female; D006084:Graft Rejection / Q000188:drug therapy / Q000517:prevention & control; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic / Q000150:complications / Q000188:drug therapy*; D016030:Kidney Transplantation; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000493:pharmacokinetics / Q000627:therapeutic use; D011162:Porphobilinogen / Q000652:urine; D017118:Porphyria, Acute Intermittent / Q000150:complications / Q000188:drug therapy*; D011239:Prednisolone / Q000008:administration & dosage / Q000493:pharmacokinetics / Q000627:therapeutic use; D006435:Renal Dialysis; D013548:Sweden; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D011162:Porphobilinogen; D016572:Cyclosporine; D000622:Aminolevulinic Acid; D011239:Prednisolone; D003404:Creatinine; D009173:Mycophenolic Acid; D001379:Azathioprine