Photosensitivity and Perioperative Polyneuropathy Complicating Orthotopic Liver Transplantation in a Patient with Erythropoietic Protoporphyria

Nguyen, Luat; Blust, Michael H.; Bailin, Michael T.; Melendez, Luis; Raines, Douglas E.

doi:10.1097/00000542-199910000-00038

Cited by 13 publications

(6 citation statements)

References 13 publications

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“…The laboratory data at the time of transplant included an average total bilirubin of 13.2 mg/dl (range, 4.2-35), creatinine of 0.9 mg/dl (range, 0.5-2.6), and INR (measured or estimated from available prothrombin times) of 1.6 (range, 1.0-2.1). 33 The estimated MELD score in the patients prior to transplant was 21 (range, [15][16][17][18][19][20][21][22][23][24][25][26][27][28][29].…”

Section: Resultsmentioning

confidence: 99%

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Liver transplantation for erythropoietic protoporphyria liver disease

et al. 2005

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In erythropoietic protoporphyria (EPP), there is excessive production of protoporphyrin, primarily in the bone marrow, resulting in increased biliary excretion of this heme precursor. Some patients will develop progressive liver disease that may ultimately require liver transplantation. However, excessive production of protoporphyrin by the bone marrow continues after transplantation, which may cause recurrent disease in the allograft. This study was performed to define post-transplant survival, the risk of recurrent disease, and specific management issues in patients transplanted for EPP liver disease. The patients studied consisted of twelve males and eight females, with an average age of 31 (range, 13-56) years at the time of transplantation. The estimated maximum MELD score prior to transplant was 21 (range, 15-29). Unique complications in the perioperative period were light induced tissue damage in four patients and neuropathy in six, requiring prolonged mechanical ventilation in four. Patient and graft survival rates were 85% at 1 year, 69% at 5 years, and 47% at 10 years. Recurrent EPP liver disease occurred in 11 of 17 patients (65%) who survived more than 2 months. Three patients were retransplanted at 1.8, 12.6, and 14.5 years after the initial transplant for recurrent EPP liver disease. In conclusion, the 5-year patient survival rate in patients transplanted for EPP liver disease is good, but the recurrence of EPP liver disease appears to diminish long term graft and patient survival. (Liver Transpl 2005;11:1590-1596.)

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Section: Resultsmentioning

confidence: 99%

“…11 Patients who progress to cirrhosis and develop liver failure have been successfully salvaged with liver transplantation. [12][13][14][15][16][17][18][19][20][21][22] However, liver transplantation does not alter the excessive production of protoporphyrin by the bone marrow. 23 Thus, recipients are at risk for recurrence of disease in the graft.…”

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confidence: 99%

Liver transplantation for erythropoietic protoporphyria liver disease

et al. 2005

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“…CLS‐200‐X is a clear film that has been used and described in 13 previous cases 3, 9. TA‐81 has been used in 1 previous case11 and, with respect to transmission, resembles a thick, inflexible filter described in 2 cases12 as well as a “welding curtain” used in 1 case 13. 61011 is a yellow film that has previously never been described.…”

Section: Discussionmentioning

confidence: 99%

Protection from phototoxic injury during surgery and endoscopy in erythropoietic protoporphyria

et al. 2008

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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 experimental model is used to evaluate the relative protection of different filters, and the results are compared with theoretical calculations regarding the risk for phototoxic injury from light sources in health-care procedures. Whether protective measures are warranted in other illuminated procedures besides liver transplantation has been discussed often but never studied. This study elucidates the risk for phototoxic injury in endoscopy, laparoscopy, and non-liver transplant surgery. A theoretical model and epidemiological data are considered. Our findings indicate that endoscopy, laparoscopy, and surgical procedures other than liver transplantation are safe in the noncholestatic protoporphyria patient and that general recommendations for using filters in these situations are not warranted. Among the tested filters, a flexible yellow filter omitting wavelengths below 470 nm is recommended for liver transplant surgery. This filter has been readily accepted by surgeons and offers a good balance between protection and altered visual color perception. The experimental model, using hemolysis of protoporphyrin-loaded erythrocytes as a measure of phototoxicity, has substantiated theoretical findings on relative filter protection.

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“…This is frequently associated with hypertension, tachycardia, abdominal pain, nausea, vomiting, and constipation . Electromyogram shows axonal denervation without demyelination . Among LTs in EPP, 14 patients (6 in the U.S. series) had neuropathy, which necessitated prolonged mechanical ventilation in 5 …”

Section: Liver Transplantation In Porphyriamentioning

confidence: 99%

Liver transplantation in the management of porphyria

Singal

Parker

Bowden³

et al. 2014

Hepatology

116

122

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Porphyrias are a group of eight metabolic disorders, each resulting from a mutation that affects an enzyme of the heme biosynthetic pathway. Porphyrias are classified as hepatic or erythropoietic, depending upon the site where the gene defect is predominantly expressed. Clinical phenotypes are classified as follows: (1) acute porphyrias with neurovisceral symptoms: acute intermittent porphyria; delta amino-levulinic acid hydratase deficiency porphyria; hereditary coproporphyria; and variegate porphyria and (2) cutaneous porphyrias with skin blistering and photosensitivity: porphyria cutanea tarda; congenital erythropoietic porphyria; hepatoerythropoietic porphyria and both erythropoietic protoporphyrias: autosomal dominant and X-linked. Liver transplantation (LT) may be needed for recurrent and/or life-threatening acute attack in acute intermittent porphyria or acute liver failure or end-stage chronic liver disease in erythropoietic protoporphyria. LT in acute intermittent porphyria is curative. Erythropoietic protoporphyria patients needing LT should be considered for bone marrow transplantation to achieve cure. Conclusion This article provides an overview of porphyria with diagnostic approaches and management strategies for specific porphyrias and recommendations for LT with indications, pretransplant evaluation, and posttransplant management.

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Photosensitivity and Perioperative Polyneuropathy Complicating Orthotopic Liver Transplantation in a Patient with Erythropoietic Protoporphyria

Cited by 13 publications

References 13 publications

Liver transplantation for erythropoietic protoporphyria liver disease

Liver transplantation for erythropoietic protoporphyria liver disease

Protection from phototoxic injury during surgery and endoscopy in erythropoietic protoporphyria

Liver transplantation in the management of porphyria

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