FAMILIAL DYSAUTONOMIA: PULMONARY MANIFESTATIONS

Abstract

Pulmonary manifestations of significance were encountered in 13 of 20 children with familial autonomic dysfunction. Study of the nature of the respiratory difficulties suggests that bronchial hypersecretion with secondary obstruction are of primary etiologic importance, although infection may supervene. The symptoms and signs, including fever, are most frequently the result of autonomic dysfunction. Roentgenographic findings in the chest have been described as consisting principally of varying combinations of diffuse infiltrations, atelectasis and emphysema. The similarity of the roentgenographic findings to those of fibrocystic disease of the pancreas has been noted and ascribed to the common factors of bronchial obstruction and secondary infection. Other diagnostic features relating to the clinical aspects of familial autonomic dysfunction have been noted.