Chronic suppurative lung disease in sisters mimicking cystic fibrosis

Abstract

Two sisters are described with fatal chronic obstructive lung disease of unknown origin. The lung condition was clinically and histologically indistinguishable from cystic fibrosis and the salivary glands showed pathological changes expected in cystic fibrosis. Neither child had abnormal sweat electrolytes, they had no bowel symptoms, and the pancreas was normal at necropsy.