The value of intravenous heme-albumin and plasmapheresis in reducing postoperative complications of orthotopic liver transplantation for erythropoietic protoporphyria.

Erythropoietic protoporphyria (EPP) is marked by a deficiency of ferrochelatase, which occurs in all cells and tissues, preventing effective conversion of proto porphyrin IX to heme and thereby blocking effective feedback inhibition of heme synthesis. The major source of the excess protoporphyrin is the bone marrow. Protoporphyrin IX may accumulate, with resultant toxicity chiefly of the marrow, skin, nervous system, and liver. Orthotopic liver transplantation (OLT) is, at present, the only adequate intervention for severe liver compromise secondary to protoporphyrin deposition, but it has been complicated by severe photosensitivity and polyneuropathy. Intravenous heme and plasmapheresis have been proposed but not previously reported as means to reduce the protoporphyrin burden before liver transplantation. We report a man with EPP who underwent preoperative heme-albumin administration and plasmaphereses that led to marked reductions in plasma and erythrocyte protoporphyrin levels. His OLT was uneventful, and he developed neither polyneuropathy nor exacerbation of photosensitivity.

Selective depletion of cerebral norepinephrine with 6-hydroxydopamine and GBR-12909 in neonatal rat.

Specific and extensive neonatal depletions of norepinephrine were produced in 3-day-old rats by combined administration of GBR-12909 (40 mg/kg, i.p.), a specific inhibitor of dopamine uptake, and the neurotoxin 6-hydroxydopamine (125 micrograms, i.c.). Norepinephrine concentrations in prefrontal cortex and hippocampus were reduced by more than 95%, and hypothalamic concentrations were reduced by 57%. Concentrations of dopamine, serotonin, and their respective metabolites were not affected in these regions or in the striatum.