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Pulmonary telangiectasia without hypoxemia.

M J Lincoln1, J W Shigeoka

  • 1Pulmonary Section, Veterans Administration Medical Center, Salt Lake City 84148.

Chest
|May 1, 1988
PubMed
Summary
This summary is machine-generated.

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Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease) can cause recurrent lung "pneumonia" due to endobronchial bleeding. This case highlights unusual bronchial circulation involvement in a rare pulmonary vascular malformation.

Area of Science:

  • Pulmonary Medicine
  • Vascular Medicine
  • Genetics

Background:

  • Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is a genetic disorder affecting blood vessels.
  • Pulmonary involvement in HHT typically manifests as arteriovenous malformations (AVMs), which can lead to significant complications.

Observation:

  • An elderly patient presented with recurrent episodes initially diagnosed as pneumonia.
  • These episodes were found to be caused by massive hemorrhage from endobronchial telangiectases.

Findings:

  • The patient exhibited normoxia and no right-to-left shunting when stable, with mild pulmonary arterial hypertension.
  • Pulmonary telangiectases were potentially isolated to the bronchial circulation, a rare presentation.
  • Hemodynamic data and photographic evidence of endobronchial telangiectases are presented.

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Implications:

  • This case expands the understanding of HHT's diverse pulmonary manifestations.
  • It suggests that endobronchial telangiectases should be considered in the differential diagnosis of recurrent pulmonary hemorrhage.
  • Further research into bronchial circulation involvement in HHT is warranted.