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Related Concept Videos

Schemas01:42

Schemas

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A schema is a mental construct consisting of a cluster or collection of related concepts (Bartlett, 1932). There are many different types of schemata, and they all have one thing in common: schemata are a method of organizing information that allows the brain to work more efficiently. When a schema is activated, the brain makes immediate assumptions about the person or object being observed.
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The Participant-Reported Implementation Update and Score PRIUS: A Novel Method for Capturing Implementation-Related Data Over Time
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Porphyria cutanea tarda: Recent update.

Ashwani K Singal1

  • 1Department of Medicine, Division of Gastroenterology and Hepatology, University of Alabama at Birmingham, Birmingham, AL, United States.

Molecular Genetics and Metabolism
|January 27, 2019
PubMed
Summary

Porphyria cutanea tarda (PCT) is a common liver disorder causing skin blistering. Effective treatments include phlebotomy or hydroxychloroquine, but controlling risk factors is key to preventing relapse.

Area of Science:

  • Hepatology
  • Dermatology
  • Genetics

Background:

  • Porphyria cutanea tarda (PCT) is the most common human porphyria, stemming from hepatic uroporphyrinogen decarboxylase (UROD) deficiency.
  • It is acquired, often linked to iron overload and factors like alcohol, smoking, viral infections (HCV, HIV), and estrogen use.
  • Familial PCT (Type II) also necessitates susceptibility factors for disease manifestation.

Purpose of the Study:

  • To review the clinical presentation, diagnosis, and management of Porphyria cutanea tarda.
  • To highlight treatment efficacy and safety, including emerging therapies for co-existing conditions.
  • To discuss the potential for relapse and areas for future research.

Main Methods:

  • Review of existing literature on Porphyria cutanea tarda.

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  • Analysis of clinical manifestations, diagnostic criteria, and treatment outcomes.
  • Discussion of risk factors, disease progression, and relapse patterns.

    • Main Results:

    • PCT presents with skin fragility and blistering, typically in the 5th-6th decade, more common in males.
    • Liver issues range from mild biochemical abnormalities to advanced fibrosis, cirrhosis, and hepatocellular carcinoma (HCC), necessitating HCC screening.
    • Phlebotomy and hydroxychloroquine are equally effective and safe treatments for PCT.

    Conclusions:

    • PCT is effectively managed with phlebotomy or hydroxychloroquine, with improved HCV treatment options available.
    • Controlling susceptibility factors is crucial for preventing PCT relapse after remission.
    • Further research is needed on 4-aminoquinolines, HCV-PCT interactions, and long-term relapse rates.