Results 51 to 60 of about 5,659 (182)

Liver transplantation for acute-on-chronic liver failure from erythropoietic protoporphyria [PDF]

Clinical and Molecular Hepatology, 2012
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 ...
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   +9 more
doaj   +1 more source

A low‐cost handheld device for monitoring cutaneous photosensitivity

Photochemistry and Photobiology, EarlyView.
The cutaneous photosensitivity detection (CPD) device is a handheld contact‐based measurement device using 405 nm excitation and red fluorescence detection for monitoring skin phototoxicity risk after photodynamic therapy (PDT) procedures involving systemic aminolaevulinic acid (ALA) induced protoporphyrin IX (PpIX) photosensitization.
Md Asaduzzaman Rasel, Ryan Beland, Shakir Khan, Tayyaba Hasan, Jonathan P. Celli   +4 more
wiley   +1 more source

Adjunctive use of Polypodium leucotomos extract in patients with erythropoietic protoporphyria: An exploratory study. [PDF]

Photochem Photobiol
Erythropoietic protoporphyria (EPP) and X-linked protoporphyria (XLP) cause severe photosensitivity, resulting in significant quality of life (QoL) impairment.
Shetty N, Quiñonez RL, Ceresnie MS, Ghosh S, Lim HW, Mohammad TF.   +5 more
europepmc   +2 more sources

Red lines and green lights: Gene therapy for inherited erythroid disorders beyond the haemoglobinopathies

British Journal of Haematology, Volume 209, Issue 4, Page 1469-1481, October 2026.
Gene therapy is revolutionizing treatment paradigms for haemoglobinopathies, establishing a translational framework for disorders that impact red blood cell development. In their paper, Joshi et al. describe the preclinical and early clinical landscape of gene therapies for non‐haemoglobinopathy erythroid disorders and highlight common thematic ...
Gaurav Joshi, Nandakumar Natarajan, Kathryn E. Dickerson, Richard A. Voit   +3 more
wiley   +1 more source

Quality of life correlations in patients with Erythropoietic Protoporphyria and X-Linked Protoporphyria on Afamelanotide: A retrospective cohort study - Supplemental Figure 1

, 2022
Investigating the impact of therapeutic response to afamelanotide on quality of life in patients with erythropoietic protoporphyria and x-linked protoporphyria.THIS DATASET IS ARCHIVED AT DANS/EASY, BUT NOT ACCESSIBLE HERE.
Ceresnie, M (via Mendeley Data), Ceresnie, Marissa   +1 more
core   +1 more source

Tolerability of hormonal treatments in acute hepatic porphyria patients

Internal Medicine Journal, Volume 56, Issue 10, Page 1748-1753, October 2026.
Abstract Background Acute hepatic porphyria (AHP) flares have been associated with female hormones and the use of hormonal treatments. Women with AHP are traditionally advised to avoid exogenous oestrogen and progesterone, limiting options for contraception and management of menorrhagia, dysmenorrhoea and menopausal symptoms.
Andjela Arandjelovic, Charlotte Ross, Gayle Ross   +2 more
wiley   +1 more source

Iron Homeostasis and Liver Diseases

Journal of Cellular and Molecular Medicine, Volume 30, Issue 20, October 2026.
ABSTRACT Iron is an essential micronutrient whose ability to cycle between ferric (Fe3+) and ferrous (Fe2+) states makes it crucial for physiological processes, including oxygen transport, DNA synthesis, and oxidative phosphorylation. However, because both deficiency and excess can cause severe pathology, precise regulation is critical. The liver plays
Peng Yang, Zhitian Shi, Qin Huang, Zhihong Tu, Yue Sun, Lin Wang, Yonglong Wei   +6 more
wiley   +1 more source

Surgical treatment for breast cancer in a patient with erythropoietic protoporphyria and photosensitivity: a case report

Surgical Case Reports, 2021
Background Erythropoietic protoporphyria (EPP) is a rare disorder of heme synthesis. Patients with EPP mainly show symptoms of photosensitivity, but approximately 20% of EPPs are associated with the liver-related complications. We report a case of breast
Akiko Shimazaki, Takuma Hashimoto, Masaya Kai, Tetsuzo Nakayama, Mai Yamada, Karen Zaguirre, Kentaro Tokuda, Makoto Kubo, Ken Yamaura, Masafumi Nakamura   +9 more
doaj   +1 more source

Inherited microcytic anemias due to disorders of iron and heme metabolism: An updated clinical review

HemaSphere, Volume 10, Issue 9, September 2026.
Abstract Microcytic anemia is among the most common hematological abnormalities in clinical practice and is usually attributable to iron deficiency, thalassemia traits, or anemia of inflammation. A small but clinically important subset of patients, however, has inherited disorders of iron metabolism or heme synthesis presenting with persistent ...
Alexandros Makis, Ioanna Saougou, Eleftheria Hatzimichael   +2 more
wiley   +1 more source

Afamelanotide Is Associated with Dose-Dependent Protective Effect from Liver Damage Related to Erythropoietic Protoporphyria

Life, 2023
In animal models, melanocyte-stimulating hormones (MSHs) protect the liver from various injuries. Erythropoietic protoporphyria (EPP), a metabolic disorder, leads to the accumulation of protoporphyrin (PPIX).
Anna-Elisabeth Minder, Xiaoye Schneider-Yin, Henryk Zulewski, Christoph E. Minder, Elisabeth I. Minder   +4 more
doaj   +1 more source