PEDIATRICS Vol. 44 No. 5 November 1969, pp. 709-715
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TRACHEOBRONCHIOMEGALY AND ACQUIRED CUTIS LAXA IN A CHILD

Physiologic and Immunologic Studies

Alan A. Wanderer M.D.1, Elliot F. Ellis M.D.1, Robert W. Goltz M.D.1, and Ernest K. Cotton M.D.1

1 Pediatric Chest Service, Department of Pediatrics, University of Colorado Medical Center, Pediatric Allergy and Clinical Immunology Division, National Jewish Hospital-University of Colorado Medical Center, Department of Dermatology, University of Colorado Medical Center, Denver

Tracheohronchiomegaly and acquired cutis laxa are described in a 14-year-old boy. Marked dilatation of the tracheobronchial tree associated with recurrent lower respiratory tract infections has been reported chiefly in the adult medical literature as the Mounier-Kuhn syndrome. Cutis laxa is a rare elastic tissue disorder that involves the skin and other systems. While the etiology of tracheobronchiomegaly is unknown, the coexistence of two rare conditions in the same patient suggtsts a common fundamental elastic tissue defect.

The investigative studies revealed abnormal elastic fibers in the skin, increased thoracic gas volumes, abnormal pulmonary elastic recoil, and a normal immunologic status except for increased serum and parotid IgA levels.

Submitted on April 10, 1969
Accepted on May 19, 1969