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EMPHYSEMA, CONGENITAL LOBAR; CLE

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130710 EMPHYSEMA, CONGENITAL LOBAR; CLE

TABLE OF CONTENTS 

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TEXT

CLE is a well-described cause of respiratory distress in infancy and is characterized by hyperinflation of the affected lobe with compression of surrounding normal lung tissue. Although it can have various 'causes' including extrabronchial vascular compression, hypoplasia of the bronchial cartilages was thought to be the basis in reported familial cases (Wall et al., 1982). Affected sibs were described by Sloan (1953) and Hendren and McKee (1966). Wall et al. (1982) reported affected mother and daughter.

REFERENCES

1. Hendren, W. H.; McKee, D. M. :
Lobar emphysema of infancy. J. Pediat. Surg. 1: 24-39, 1966. 
2. Sloan, H. :
Lobar obstructive emphysema in infancy treated by lobectomy. J. Thorac. Cardiovasc. Surg. 26: 1-20, 1953. 
3. Wall, M. A.; Eisenberg, J. D.; Campbell, J. R. :
Congenital lobar emphysema in a mother and daughter. Pediatrics 70: 131-133, 1982.
PubMed ID : 7088614

 

CLINICAL SYNOPSIS

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CREATION DATE

Victor A. McKusick : 6/4/1986

EDIT HISTORY

mimadm : 9/24/1994
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/26/1989
marie : 3/25/1988
reenie : 6/4/1986

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Date Last Modified: 11/3/99
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