Recovery From Severe Systemic Peripheral Neuropathy Secondary to Erythropoietic Protoporphyria by Liver Transplant: A Case Report

Shizuku, Masato; Kurata, Nori; Jobara, Kanta; Tanaka, Taku; Fukuta, Akimasa; Iwata, Mai Hatanaka; Hara, Kazuhiro; Katsuno, Masahisa; Nakano, Hajime; Ogura, Yasuhiro

doi:10.6002/ect.2022.0157

Cited by 1 publication

(1 citation statement)

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“…Sixty-six cases of liver transplantation for EPP have been reported (Endo et al 2022, Hanaki et al 2020, McGuire et al 2005, Shizuku et al 2022, Tanaka et al 1994, Wahlin et al 2011, Yamashita et al 2020, and almost all of them were reports of decreased-…”

Section: Accepted Manuscript 12mentioning

confidence: 99%

Living-Donor Liver Transplantation for Erythropoietic Protoporphyria: A Case Report and Literature Review

Ogasawara

Tokodai

Nakanishi

et al. 2023

Tohoku J. Exp. Med.

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Erythropoietic protoporphyria (EPP) is a very rare disease with an estimated prevalence of 1 in 200,000 individuals. Decreased ferrochelatase activity causes the accumulation of protoporphyrin in the body, and light exposure results in the generation of active oxygen, causing photosensitivity. Liver damage has the greatest influence on the prognosis, and liver transplantation is the only treatment option for patients with decompensated liver cirrhosis. We report a case of living-donor liver transplantation for decompensated liver cirrhosis associated with EPP. The patient was a 52-year-old male who led a normal life except for mild photosensitivity. When the patient was 37-yearold, hepatic dysfunction was noticed. At 48-year-old, high erythrocyte PP levels, skin biopsy, and genetic tests resulted in a diagnosis of EPP. The patient underwent livingdonor liver transplantation because of decompensated liver cirrhosis. In the operating room and intensive care unit, a special light-shielding film was applied to all light sources to block light with harmful wavelengths during treatment. Due to the need for special measures, a lecture on patients with EPP was given before surgery to deepen understanding among all medical professionals involved in the treatment. As a result, no adverse events occurred during the perioperative period, and the patient was discharged

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