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Related Experiment Video

Updated: Jun 20, 2026

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
12:57

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans

Published on: January 8, 2015

Proteus syndrome: a case report.

Erin Zusan1, J Michael Smith, Thomas Parker

  • 1Department of Surgery, Good Samaritan Hospital, Cincinnati, Ohio 45220, USA.

The American Surgeon
|September 25, 2009
PubMed
Summary
This summary is machine-generated.

Proteus syndrome, a rare disorder, can cause severe lung issues like cystic pulmonary emphysema. This case highlights extensive bullous disease in a patient with Proteus syndrome, leading to respiratory failure.

Related Experiment Videos

Last Updated: Jun 20, 2026

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
12:57

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans

Published on: January 8, 2015

Area of Science:

  • Medical Genetics
  • Pulmonology
  • Dermatology

Background:

  • Proteus syndrome is a rare, sporadic hamartomatous disorder characterized by mosaic overgrowth and multisystemic manifestations.
  • Clinical features include craniofacial abnormalities, limb asymmetry, lipomas, and vascular malformations.
  • Cystic lung disease occurs in approximately 10% of patients, potentially leading to cystic pulmonary emphysema.

Observation:

  • A 27-year-old woman with known Proteus syndrome presented with acute on chronic respiratory failure.
  • A CT scan revealed extensive bullous disease, with the right lung almost entirely replaced by cystic changes.
  • The patient's presentation indicated severe pulmonary complications secondary to Proteus syndrome.

Findings:

  • The case demonstrates a severe manifestation of cystic lung disease in Proteus syndrome.
  • Extensive bullous changes significantly compromised lung function, resulting in respiratory failure.
  • The patient's clinical course and management highlight the challenges in treating pulmonary complications of Proteus syndrome.

Implications:

  • This case underscores the importance of monitoring for and managing pulmonary complications in Proteus syndrome patients.
  • Early recognition and intervention for cystic lung disease may improve patient outcomes.
  • Further research into the pathogenesis of lung involvement in Proteus syndrome is warranted.