Brief clinical report: Pulmonary hypertension and interstitial fibrosis in von Recklinghausen neurofibromatosis

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Abstract

Neurofibromatosis von Recklinghausen (NFvR) has been reported to be associated with diffuse interstitial pulmonary fibrosis. We describe a patient with NFvR presenting in middle age with dyspnea and cyanosis. Chest radiographs showed right ventricular enlargement, dilated proximal pulmonary arteries, and bilaterally increased interstitial markings. Cardiac catheterization showed moderately severe pulmonary hypertension and no evidence of congenital heart defect. The interstitial fibrosis associated with NFvR can result in symptomatic pulmonary hypertension.

Original languageEnglish (US)
Pages (from-to)531-535
Number of pages5
JournalAmerican journal of medical genetics
Volume25
Issue number3
DOIs
StatePublished - Jan 1 1986

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

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