The Hamman-Rich syndrome in childhood; report of a case with unilateral pulmonary arterial and venous stenosis and atriovenous occlusion

Israel Diamond

Children’s Hospital and University of Louisville School of Medicine
United States

Pediatrics
Pediatrics 1958; 22: 279–288
https://doi.org/10.1542/peds.22.2.279

Abstract
The Hamman-Rich syndrome is described in a 4-year-old Negro male. The clinical picture was that of persistent cough and progressive dyspnea beginning at 4 months of age. Diagnosis was made ante mortem by lung biopsy. The fibrotic process and arteriolosclerosis were more marked in the right lung. The disease may have been initiated by a bout of aspiration. There was accompanying stenosis of the right pulmonary artery and vein and occlusion of the lumen of the right pulmonary vein at its entrance to the left atrium. The hilar vascular findings are believed to be secondary to hilar areolar inflammation.

Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Pulmonary Venous Pathology

Year of Publication: 1958

Age Focus: Pediatric

Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients)

Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No