2008
DOI: 10.1002/lt.21527
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Protection from phototoxic injury during surgery and endoscopy in erythropoietic protoporphyria

Abstract: Erythropoietic protoporphyria is an inherited condition characterized by pronounced solar photosensitivity and in a minority of patients severe liver disease that necessitates liver transplantation for survival. Phototoxic injury to abdominal organs and skin has been reported in several cases of liver transplantation surgery, including a few transplants in which protective light filters were used. This study discusses the optimal characteristics of light filters used during liver transplantation surgery. An ex… Show more

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Cited by 61 publications
(69 citation statements)
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“…Patients may develop a mild hypochromic microcytic or normocytic anaemia, which can be associated with decreased serum iron levels and increased serum ironbinding capacity (Deleo et al 1976 ). With the exception of patients with EPP liver failure, operating theatre lights do not cause any problems during or after surgery in EPP patients (Wahlin et al 2008 ). However, operating theatre lights can cause a potentially fatal phototoxic reaction in patients undergoing liver transplantation for protoporphyric liver failure.…”
Section: General Principles Of Treatmentmentioning
confidence: 99%
“…Patients may develop a mild hypochromic microcytic or normocytic anaemia, which can be associated with decreased serum iron levels and increased serum ironbinding capacity (Deleo et al 1976 ). With the exception of patients with EPP liver failure, operating theatre lights do not cause any problems during or after surgery in EPP patients (Wahlin et al 2008 ). However, operating theatre lights can cause a potentially fatal phototoxic reaction in patients undergoing liver transplantation for protoporphyric liver failure.…”
Section: General Principles Of Treatmentmentioning
confidence: 99%
“…The easiest and most effective protective measure for liver transplantation surgery in EPP is to use a filter that blocks the most harmful wavelengths and prevents phototoxic burn injuries induced by surgical luminaires (Figure 7). [177][178][179] The risk of phototoxic injury during surgery is related to the tissue protoporphyrin concentration. The circulating levels in EPP liver transplantation depend on the severity of liver failure and on the success of preoperative treatment.…”
Section: Perioperative Managementmentioning
confidence: 99%
“…[192][193][194] Milder acute attacks may settle spontaneously. More severe cases will become protracted and may be complicated by a number of serious problems such as flaccid quadriparesis, 31,182,195,196 seizures, 155,181,[195][196][197] encephalopathy, severe hyponatremia, 139,155,197 and the posterior reversible encephalopathy syndrome. 195,[198][199][200] In our experience, seizures, which are uncommon, are often secondary to factors such as hyponatremia 155,172 and the use of meperidine in high doses.…”
Section: Acute Attackmentioning
confidence: 99%
“…Consequently, many acute attacks occur in patients not known to have porphyria and patients may be diagnosed following admission to an intensive care unit or medical ward with an unexplained neuropathy, 184,195,201 often initially diagnosed as Guillain-Barré syndrome. 182,192,202 There is still uncertainty as to the pathogenesis of the acute attack. Two principal hypotheses have been put forward: intraneuronal heme deficiency and the overproduction of neurotoxic substances by the liver, whether heme precursors or another compound, whose production parallel that of the heme precursors.…”
Section: Acute Attackmentioning
confidence: 99%