Pulmonary interstitial fibrosis in an infant

Abstract

A Chinese male infant 23 wk of age was admitted with cyanosis and dyspnea accompanied by a productive cough. There was marked tachypnea and tachycardia. Death occurred suddenly after feeing on the 4th day after admission. At this stage he weighed only 5,000 g. At autopsy the combined weight of both lungs and the heart was 280 g. The lungs appeared firm and indurated with multiple fissures. The bronchi were filled with vomitus. The heart was top normal in size. Right ventricular hypertrophy was marked with the wall thickness (0.4 cm) equal to that of the left ventricle. Sections from various parts of the lung indicated a generalized involvement of both lungs. The alveolar walls were thickened with an interstitial infiltrate of chronic inflammatory cells mainly lymphocytes with occasional plasma cells and a few polymorphonuclear leukocytes; there was accompanying increase in connective tissue elements, especially fibroblasts. An increase of reticulum fibers was demonstrated by reticulum stains. The alveolar spaces were poorly formed and indistinct in many areas. There was desquamation of alveolar cells into the alveoli; the majority of these alveolar cells did not show any PAS+ve granules in the cytoplasm. Active proliferation with mitotic figures was seen. The smaller bronchioles showed patchy loss of cilia and cuboidal metaplasia of the epithelium. There was no change in the wall structure. The larger bronchioles demonstrated goblet cells in the epithelium, indicative of hypersecretion. There was no evidence of obliterative bronchiolitis. There was no significant arteriolar hypertrophy nor was there any evidence of obliterative endarteritis. The Hamman Rich syndrome is now a well established clinical entity. First reported by Hamman and Rich (1935), it is now known to be most frequent during ages 30 to 50 yr and affects males slightly more frequently. The Hamman Rich syndrome is rare in children, the youngest case reported being a child of 3 mth. In a review of the literature, Bradley (1956) could only trace 25 typical and 20 less typical cases of this condition in children. The present case seems to be the first report of this condition occurring almost immediately after birth.