OVERT PCT IN A SYMPTOMATIC AIP PATIENT ON HEMODIALYSIS

The patient is a woman born in 1953 who had her first AIP attack at the age of 24 years. Since the age of 40 years she was continuously affected by recurrent serious acute attacks. On each occasion she was treated during 3-4 days with glucose and electrolyte infusions, analgetics and heme infusion (NormosangR). At the age of 50 years she has received 220 NormosangR cures, corresponding to 110 g hemarginate containing 9.5 g iron. During the last years renal impairment has progressed to end-stage renal disease (ESRD) requiring hemodialysis (HD), with three dialysis occasions per week, and administration of erythropoietin and parenteral iron. After the start of hemodialysis treatment the clinical picture has changed. The acute attacks have diminished in intensity and frequency, but the incipient motoric neuropathy has rapidly progressed and she is now bound to wheelchair. She has developed intensive PCT symptoms with skin blisters on sun exposed areas of the body. The porphyric biochemical pattern has changed as well. Plasma porphyrins are increased (>1000 nmol/L, normal <10 nmol/L) and the porphyrin pattern is dominated by polycarboxylated porphyrins in accordance with PCT. The levels of plasma porphyrins are not, or only slightly, affected by HD. The plasma levels of the porphyrin precursors ALA and PBG are affected by dialysis, especially PBG that is shown to be much lowered by dialysis. Clinical symptoms of AIP, although less common than before, are treated as usual. In contrast, attempts to treat PCT symptoms are impeded by the concurrent metabolic conditions, AIP and ESRD. Different aspects of the management are discussed in the presentation.