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April 9, 2022American Journal of Medical Genetics Part A

Novel COL5A1 variant linked to prominent vascular and cardiac features resembling vascular EDS.

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Why the study?

To describe a patient presenting with a phenotype resembling vascular EDS caused by a novel pathogenic variant in COL5A1.

Population

1 patient with a novel pathogenic variant in COL5A1

Design

Case report

Key result

A novel pathogenic variant in COL5A1 was identified in a patient exhibiting prominent vascular and cardiac features resembling vascular Ehlers-Danlos Syndrome.

Authors

KLKatta LavanyaKMKarishma MahtaniJAJessica Abbott

Discussion

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Overview

Single-case finding warrants diagnostic caution; leaves open whether COL5A1 variants can produce vEDS-like phenotypes pending replication.

Key Points

  • To describe a patient presenting with prominent vascular and cardiac manifestations caused by a novel pathogenic variant in COL5A1.
  • Clinical and genetic assessment of an individual presenting with vascular and cardiac features characteristic of vascular Ehlers-Danlos syndrome.
  • Identification of a novel pathogenic variant in COL5A1, a gene classically responsible for the classical subtype of Ehlers-Danlos syndrome.
  • Observation of a clinical presentation that mimics vascular Ehlers-Danlos syndrome despite the absence of typical COL3A1 mutations.

Study Design

Type

Case Report (n=1)

Structured PICO

P
Population
A single patient with a novel pathogenic variant in COL5A1 exhibiting prominent vascular and cardiac features resembling vascular Ehlers-Danlos Syndrome.
O
Outcome
Phenotypic description

A novel pathogenic variant in COL5A1 can present with a phenotype resembling vascular Ehlers-Danlos Syndrome, highlighting the potential for prominent vascular and cardiac features in classical EDS subtypes.

Cite This Study

Lavanya et al. (2022) conducted a case report in Ehlers-Danlos Syndrome (n=1). Novel pathogenic variant in COL5A1 was evaluated on Phenotype resembling vascular EDS. A novel pathogenic variant in COL5A1 was identified in a patient exhibiting prominent vascular and cardiac features resembling vascular Ehlers-Danlos Syndrome.

synapsesocial.com/papers/6aa2183ad2b6e29a16d3ce3ahttps://doi.org/10.1002/ajmg.a.62745
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Clinical Variability of Classical Ehlers–Danlos Syndrome: A Family with Rare COL5A1 Variant and Case-Based Review2026
  2. 2A novel variant of Ehlers‐Danlos‐syndrome with <i>COL1A2</i> mutation2026
  3. 3Novel <i>COL5A1</i> variants and associated disease phenotypes in dogs with classical <scp>Ehlers‐Danlos</scp> syndrome2024 · 2 citations
  4. 4Case report: A novel COL3A1 variant in a Colombian patient with isolated cerebrovascular involvement in vascular Ehlers–Danlos syndrome2024 · 3 citations
  5. 5Vascular Ehlers-Danlos Syndrome: A Comprehensive Natural History Study in a Dutch National Cohort of 142 Patients2024 · 32 citations