PEDIATRICS Vol. 22 No. 2 August 1958, pp. 279-288
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THE HAMMAN-RICH SYNDROME IN CHILDHOOD

Report of a Case with Unilateral Pulmonary Arterial and Venous Stenosis and Atriovenous Occlusion

Israel Diamond M.D.1

1 Departments of Pathology of the Children's Hospital and time University of Louisville School of Medicine

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.

Submitted on December 26, 1957
Accepted on February 28, 1958


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