Diffuse Progressive Interstitial Fibrosis of the Lungs in Childhood (Hamman-Rich Syndrome)

Abstract

Three cases of the Hamman-Rich syndrome in childhood are described. The ages of the children at death were 6 1/2, 7 and 23 months. These cases were manifested clinically by dyspnoea, tachypnoea and cyanosis. The respiratory symptoms became steadily more marked and two of the children died with right-sided cardiac failure. The pathological findings in the lungs were similar in all three cases, the main macroscopic appearances consisting of increase in bulk of the lungs with evi dence of fibrosis, and the main histological feature being broadening of the interalveolar interstitial tissue. No treatment was of any avail apart from oxygen. The Hamman-Rich syndrome should be considered in any child who has persistent cough, dyspnoea, tachypnoea, cyanosis improved by oxygen, and heart failure.