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Respiratory complications in children with spondyloepiphyseal dysplasia congenita
Author(s) -
Harding Cary O.,
Green Christopher G.,
Perloff William H.,
Pauli Richard M.
Publication year - 1990
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.1950090112
Subject(s) - medicine , bronchopulmonary dysplasia , pediatrics , respiratory disease , dysplasia , dermatology , lung , pregnancy , genetics , gestational age , biology
Mild respiratory distress at birth is a common occurrence in infants with spondyloepiphyseal dysplasia congenita (SEDC) while severe repiratory insufficency and death have been reported only rarely. We describe three infants with SEDC who experienced severe respiratory complications and required tracheostomies. Two of these infants have also needed long‐term continuous positive airway pressure to maintain adequate ventilation. The features and clinical course of these children are described, previous reports of respiratory complications in SEDC are summarized, mechanisms resulting in respiratory problems in SEDC are postulated, and comparison is made with mechanisms giving rise to respiratory risks in other skeletal dysplasias. Pediatr Pulmonol 1990; 9:49–54 .