Erythropoietic protoporphyria and pretransplantation treatment with nonbiological liver assist devices.

Erythropoietic protoporphyria (EPP) is a disease of the heme metabolism due to a deficiency of ferrochelatase, leading to accumulation of protoporphyrin (PPIX) in the erythrocyte (red blood cell [RBC]). The major clinical manifestation in EPP is photosensitivity; however, in a small number of patients liver failure is a significant complication and liver transplantation is the only treatment option. Damage to both abdominal skin and organs occurs when exposed to operating light; however, this problem can be ameliorated by the use of filters that block the transmission of light with wavelength below 470 nm. A more unusual but very serious complication postoperatively is severe motor neuropathy, with few or no known acute available precautions. An effective treatment option is needed to manage EPP crises and to prevent complications after liver transplantation. We successfully treated a patient with EPP-induced liver failure with the molecular adsorbents recirculating system (MARS) and Prometheus in independent sessions. Following treatment with MARS we found a 9.1% reduction of the RBC-PPIX concentration and a 5.9% reduction after treatment with the Prometheus system. Plasmapheresis made a reduction in RBC-PPIX concentration of 0.8%. Following treatment sessions with MARS and Prometheus, the clinical condition was markedly improved and orthotopic liver transplantation was performed without further complications. In conclusion, extracorporeal therapy with MARS or Prometheus seems to be efficient in reducing RBC-PPIX concentration in comparison to plasma exchange.     

A randomized controlled trial of electromagnetic therapy in the primary care management of venous leg ulceration

OBJECTIVE: The aim was to establish the potential efficacy, tolerabilityand side-effect profile of electromagnetic therapy as an adjunctto conventional dressings in the treatment of venous leg ulcers. METHOD: A prospective, randomized, double blind controlled clinicaltrial was carried out in a dedicated leg ulcer clinic basedin one urban general practice. Nineteen patients with leg ulcersof confirmed venous aetiology were assessed. The main outcomemeasures were rate and scale of venous leg ulcer healing, changesin patient-reported pain levels, quality of life, degree ofmobility, side effect profile and acceptability to patientsand staff. RESULTS: Sixty-eight per cent of patients attending this dedicated clinicachieved improvements in the size of their ulcer (4, 21%, healedfully) and in reduced pain levels (P < 0.05) during the trial,despite the chronicity of ulcer histories. Patients treatedwith electromagnetic therapy at 800 Hz were found at day 50to have significantly greater healing (P < 0.05) and paincontrol (P < 0.05) than placebo therapy or treatment with600 Hz. All patients reported improved mobility at the end ofthe study. The electromagnetic therapy was well tolerated bypatients, with no differences between groups in reporting adverseevents, and proved acceptable to staff. CONCLUSION: Despite the small numbers in this pilot study, electromagnetictherapy provided significant gains in the healing of venousleg ulcers and reduction in pain. Keywords. Electromagnetic therapy, RCT, leg ulcers, primary care.