Liver transplantation for acute-on-chronic liver failure from erythropoietic protoporphyria.

Pyoung Jae Park, Shin Hwang, Young Il Choi, Young-Dong Yu, Gil Chun Park, Sung Won Jung, Sam Youl Yoon, Gi Won Song, Tae Yong Ha, Sung Gyu Lee

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Erythropoietic protoporphyria (EPP) is an inherited disorder of the heme metabolic pathway that is characterized by accumulation of protoporphyrin in the blood, erythrocytes, and tissues, and cutaneous manifestations of photosensitivity, all resulting from abnormalities in ferrochelatase (FECH) activity due to mutations in the FECH gene. Protoporphyrin is excreted by the liver, and excess protoporphyrin leads to cholelithiasis with obstructive episodes and chronic liver disease, finally progressing to liver cirrhosis. Patients with end-stage EPP-associated liver disease require liver transplantation. We describe here a 31-year-old male patient with EPP who experienced acute-on-chronic liver failure and underwent deceased-donor liver transplantation. Surgical and postoperative care included specific shielding from exposure to ultraviolet radiation to prevent photosensitivity-associated adverse effects. The patient recovered uneventfully and was doing well 24 months after transplantation. Future prevention and treatment of liver disease are discussed in detail.

Original languageEnglish
Pages (from-to)411-415
Number of pages5
JournalClinical and molecular hepatology
Volume18
Issue number4
DOIs
Publication statusPublished - 2012 Dec 1
Externally publishedYes

Fingerprint

Erythropoietic Protoporphyria
Ferrochelatase
Liver Transplantation
Liver Diseases
Skin Manifestations
Cholelithiasis
Postoperative Care
Metabolic Networks and Pathways
Heme
Liver Cirrhosis
Chronic Disease
Transplantation
Erythrocytes
Tissue Donors
Radiation
Mutation
Liver
Genes
Acute-On-Chronic Liver Failure
protoporphyrin IX

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Liver transplantation for acute-on-chronic liver failure from erythropoietic protoporphyria. / Park, Pyoung Jae; Hwang, Shin; Choi, Young Il; Yu, Young-Dong; Park, Gil Chun; Jung, Sung Won; Yoon, Sam Youl; Song, Gi Won; Ha, Tae Yong; Lee, Sung Gyu.

In: Clinical and molecular hepatology, Vol. 18, No. 4, 01.12.2012, p. 411-415.

Research output: Contribution to journalArticle

Park, PJ, Hwang, S, Choi, YI, Yu, Y-D, Park, GC, Jung, SW, Yoon, SY, Song, GW, Ha, TY & Lee, SG 2012, 'Liver transplantation for acute-on-chronic liver failure from erythropoietic protoporphyria.', Clinical and molecular hepatology, vol. 18, no. 4, pp. 411-415. https://doi.org/10.3350/cmh.2012.18.4.411
Park, Pyoung Jae ; Hwang, Shin ; Choi, Young Il ; Yu, Young-Dong ; Park, Gil Chun ; Jung, Sung Won ; Yoon, Sam Youl ; Song, Gi Won ; Ha, Tae Yong ; Lee, Sung Gyu. / Liver transplantation for acute-on-chronic liver failure from erythropoietic protoporphyria. In: Clinical and molecular hepatology. 2012 ; Vol. 18, No. 4. pp. 411-415.
@article{3aad032b3e094343961114cc4cfe5e6b,
title = "Liver transplantation for acute-on-chronic liver failure from erythropoietic protoporphyria.",
abstract = "Erythropoietic protoporphyria (EPP) is an inherited disorder of the heme metabolic pathway that is characterized by accumulation of protoporphyrin in the blood, erythrocytes, and tissues, and cutaneous manifestations of photosensitivity, all resulting from abnormalities in ferrochelatase (FECH) activity due to mutations in the FECH gene. Protoporphyrin is excreted by the liver, and excess protoporphyrin leads to cholelithiasis with obstructive episodes and chronic liver disease, finally progressing to liver cirrhosis. Patients with end-stage EPP-associated liver disease require liver transplantation. We describe here a 31-year-old male patient with EPP who experienced acute-on-chronic liver failure and underwent deceased-donor liver transplantation. Surgical and postoperative care included specific shielding from exposure to ultraviolet radiation to prevent photosensitivity-associated adverse effects. The patient recovered uneventfully and was doing well 24 months after transplantation. Future prevention and treatment of liver disease are discussed in detail.",
author = "Park, {Pyoung Jae} and Shin Hwang and Choi, {Young Il} and Young-Dong Yu and Park, {Gil Chun} and Jung, {Sung Won} and Yoon, {Sam Youl} and Song, {Gi Won} and Ha, {Tae Yong} and Lee, {Sung Gyu}",
year = "2012",
month = "12",
day = "1",
doi = "10.3350/cmh.2012.18.4.411",
language = "English",
volume = "18",
pages = "411--415",
journal = "Clinical and molecular hepatology",
issn = "2287-2728",
publisher = "Korean Association for the Study of the Liver",
number = "4",

}

TY - JOUR

T1 - Liver transplantation for acute-on-chronic liver failure from erythropoietic protoporphyria.

AU - Park, Pyoung Jae

AU - Hwang, Shin

AU - Choi, Young Il

AU - Yu, Young-Dong

AU - Park, Gil Chun

AU - Jung, Sung Won

AU - Yoon, Sam Youl

AU - Song, Gi Won

AU - Ha, Tae Yong

AU - Lee, Sung Gyu

PY - 2012/12/1

Y1 - 2012/12/1

N2 - Erythropoietic protoporphyria (EPP) is an inherited disorder of the heme metabolic pathway that is characterized by accumulation of protoporphyrin in the blood, erythrocytes, and tissues, and cutaneous manifestations of photosensitivity, all resulting from abnormalities in ferrochelatase (FECH) activity due to mutations in the FECH gene. Protoporphyrin is excreted by the liver, and excess protoporphyrin leads to cholelithiasis with obstructive episodes and chronic liver disease, finally progressing to liver cirrhosis. Patients with end-stage EPP-associated liver disease require liver transplantation. We describe here a 31-year-old male patient with EPP who experienced acute-on-chronic liver failure and underwent deceased-donor liver transplantation. Surgical and postoperative care included specific shielding from exposure to ultraviolet radiation to prevent photosensitivity-associated adverse effects. The patient recovered uneventfully and was doing well 24 months after transplantation. Future prevention and treatment of liver disease are discussed in detail.

AB - Erythropoietic protoporphyria (EPP) is an inherited disorder of the heme metabolic pathway that is characterized by accumulation of protoporphyrin in the blood, erythrocytes, and tissues, and cutaneous manifestations of photosensitivity, all resulting from abnormalities in ferrochelatase (FECH) activity due to mutations in the FECH gene. Protoporphyrin is excreted by the liver, and excess protoporphyrin leads to cholelithiasis with obstructive episodes and chronic liver disease, finally progressing to liver cirrhosis. Patients with end-stage EPP-associated liver disease require liver transplantation. We describe here a 31-year-old male patient with EPP who experienced acute-on-chronic liver failure and underwent deceased-donor liver transplantation. Surgical and postoperative care included specific shielding from exposure to ultraviolet radiation to prevent photosensitivity-associated adverse effects. The patient recovered uneventfully and was doing well 24 months after transplantation. Future prevention and treatment of liver disease are discussed in detail.

UR - http://www.scopus.com/inward/record.url?scp=84879469510&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84879469510&partnerID=8YFLogxK

U2 - 10.3350/cmh.2012.18.4.411

DO - 10.3350/cmh.2012.18.4.411

M3 - Article

VL - 18

SP - 411

EP - 415

JO - Clinical and molecular hepatology

JF - Clinical and molecular hepatology

SN - 2287-2728

IS - 4

ER -