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Endothelial Dysfunction in Acute Hepatic Porphyrias.

Andrea Ricci1, Gilda Sandri2, Matteo Marcacci1

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Diagnostics (Basel, Switzerland)
|June 24, 2022
PubMed
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Endothelial dysfunction, marked by low nitric oxide and high endothelin-1, is linked to symptomatic acute hepatic porphyrias (AHPs). This vascular complication appears tied to the disease

Keywords:
acute hepatic porphyriaschronic kidney diseaseendothelial dysfunctionendothelinhemehypertensionnitric oxideporphyriarare diseasesδ-aminolevulinic acid

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Area of Science:

  • Biochemistry
  • Vascular Biology
  • Rare Diseases

Background:

  • Acute hepatic porphyrias (AHPs) are rare genetic disorders affecting heme biosynthesis.
  • Vascular complications, including endothelial dysfunction, are recognized long-term risks in AHP patients.
  • Heme precursor accumulation is implicated in AHP's diverse clinical manifestations.

Purpose of the Study:

  • To investigate endothelial dysfunction (ED) in patients with acute hepatic porphyrias (AHPs).
  • To assess serum levels of endothelin-1 (ET-1) and nitric oxide (NO) as markers of ED.
  • To correlate ED markers with clinical phenotypes and biochemical alterations in AHPs.

Main Methods:

  • Serum ET-1 and NO levels were measured in 46 AHP patients.
  • Patients were categorized into symptomatic (AP-SP), asymptomatic with biochemical alterations (AP-BA), and asymptomatic without biochemical alterations (AP-AC).
  • ED markers were analyzed in relation to clinical status and urinary heme precursor levels.

Main Results:

  • Symptomatic AHP patients (AP-SP) exhibited significantly lower NO and higher ET-1 levels compared to other groups.
  • Abnormal ED markers were more frequent in AP-SP patients, irrespective of hemin treatment.
  • Elevated heme precursor levels correlated with ED marker alterations, though not significantly.

Conclusions:

  • Endothelial dysfunction is more strongly associated with the clinical phenotype of AHPs than with biochemical markers alone.
  • Clinical manifestation in AHPs may be influenced by disease modifiers affecting endothelial function.
  • Targeting endothelial dysfunction could be a potential therapeutic strategy for symptomatic AHP patients.