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. 2007 Dec;83(986):739–748. doi: 10.1136/gut.2006.097576

Table 3 Published clinical reports of liver transplants for erythropoietic protoporphyria (EPP)‐related liver failure.

First author Year Sex Age Complications Outcome
Wells11 1980 M 19 Death at 4 weeks after transplantation Death attributed to disseminated candidiasis
Polson91 1988 M 13 Initially none First liver rejected. Death from complications after second liver transplant
Samuel9 1988 F 24 Transplanted liver functioned poorly initially Alive at 40 months. Good outcome
Bloomer92 1989 F 38 None Patient well 13 months after transplant but with mild hepatic fibrosis in transplant
M 51 Severe skin necrosis and photo‐injury to intestines Death secondary to multiple intestinal perforations
Wagner93 1989 M 49 Sepsis Died 2 months after transplant
Herbert82 1991 F 42 Skin burns and neuropathy Slow recovery. Alive at 36 months
Shehade95 1991 F 40 Skin burns and severe neuropathy Well 3 years after transplant. Mild neuropathy persisted
Steinmüller94 1992 M 51 Successful transplant Well 1 year after transplant
Mion64 1992 M 38 Postoperative seizures Medium term survival (at least)
Ozawa96 1992 M 15 Surgery successful No follow‐up published
Schleiffenbaum97 1992 F 40 Surgery successful Patient well 5 months after transplant
Key75 (Bloomer32) 1992 M 51 Skin burns, small GI bleed, severe haemolytic anaemia Sepsis and death 73 days after surgery
1992 M 13 Severe motor neuropathy, severe haemolytic anaemia Good recovery following surgery
Sarkany13 1993 F 17 None Well 14 months after surgery
Rank98 1993 F 36 Postoperative bleeding Multiorgan failure; death 5 weeks after transplantation
M 51 Severe postoperative neuropathy and skin burns Death within weeks of transplant due to complications including sepsis
M 13 Massive haemolysis during surgery. Severe neuropathy Patient at home and able to care for himself 1 year after transplant
M 27 Severe postoperative neuropathy Gradual recovery of muscle strength
F 18 Minor skin burns Good recovery after transplant
Meerman58,104 1994 F 34 Repeat laparotomy for postoperative bleed Liver fibrosis present 2 years after surgery; remains well 7 years after transplant
F 39 None Portal fibrosis in transplant at 6 and 12 months after transplant. Liver fibrosis progressing at 7 years after transplant
de Torres89 1996 F 41 None Good outcome initially, but protoporphyrin damage to transplanted liver
Lock99 1996 F 58 Severe polyneuropathy Neuropathy persists 1 year after transplant, general condition markedly improved
Harper,83 Thunell100 1998 M 50 Phototoxic abdominal burns, intestinal bleeding after surgery Death from multiorgan failure 1 month after transplant
Gross41 1998 M 39 None Excellent health 5 years after surgery
M 51 Intrahepatic cholestasis
F 59 None Marked improvement 1 year after surgery
F 59 Accumulation of protoporphyrin in transplant Post‐transplant health good
F 17 None Good
F 58 None Good
M 38 None Good
Rüfenacht30 1998 F 49 Cholestasis corrected, liver function normalised Follow‐up 4 years after transplant
F 35 Cholestasis corrected, liver function normalised Follow‐up 2 years after transplant
Nguyen101 1999 M 54 Polyneuropathy Most of neuropathy had recovered 12 months after surgery
Reicheld,65 Do102 1999 2002 M 55 Mild rejection episode at 3 months Use of plasmapheresis and IV haem‐albumin to protect transplanted liver
Jimenez‐Saenz103 1999 M 59 None Good health 30 months after transplant
Leone105 2000 M 35 None Excellent health 4 years after transplant
2000 M 50 Poor health after transplant Died 1 month after transplant
Dellon90 2002 M 54 Neuropathy Transplanted liver affected by protoporphyrin.
Schoenfeld34 2003 M 29 None 3 year postoperative follow‐up