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Protoporfirin Fraksiyonu (Eritrosit) (FEP)

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Protoporfirin Fraksiyonu (Eritrosit) (FEP)

Klinik kullanımı

Demirin ferroşelataz kademesindeki defekt nedeniyle protoporfirinler eritrositlerin içinde birikir. Eritropoetik porfiria’ya neden olur. Fotosensitif dermatozis şeklinde ortaya çıkar.

Referans değerler

< 1,4 μmol/L RBC

 

#eritropoetik porfiria

Kaynaklar ve Referanslar

  1. [1]Garrido Montes M, Pertusa Mataix R, Garcia Morillo JS. “Therapeutic approach to acute crises of hepatic porphyrias.”. Revista clinica espanola. 2024. PMID: 39313028. PubMed →
  2. [2]Minder AE, Kluijver LG, Barman-Aksözen J, Minder EI, Langendonk JG. “Erythropoietic protoporphyrias: Pathogenesis, diagnosis and management.”. Liver International. 2024. PMID: 39011756. PubMed →
  3. [3]Santos Vicente J, Lu Cortez L, Enríquez de Salamanca R. “[Porphyria cutanea tarda].”. Anales de medicina interna (Madrid, Spain : 1984). 1991. PMID: 1912166. PubMed →
  4. [4]Noyman Y, Edel Y, Snast I, Sherman S, Kaftory R, Lapidoth M, Mimouni D, Hodak E, Levi A. “Inherited genetic late-onset erythropoietic protoporphyria: A systematic review of the literature.”. Photodermatology, Photoimmunology & Photomedicine. 2021. PMID: 33556208. PubMed →
  5. [5]DE ALZAGA S, CALCARAMI J, LOBO R. “[Porphyria].”. Prensa pediatrica; revista americana de puericultura y pediatria. 1953. PMID: 13120738. PubMed →
  6. [6]Balwani M. “Erythropoietic Protoporphyria and X-Linked Protoporphyria: pathophysiology, genetics, clinical manifestations, and management.”. Molecular Genetics and Metabolism. 2019. PMID: 30704898. PubMed →
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