Ten days of orotracheal intubation with successful extubation in an infant with junctional epidermolysis bullosa.
OBJECTIVE: The most severe form of generalized junctional epidermolysis bullosa, the Herlitz variant, is associated with a number of extracutaneous manifestations. We report on a 45-day-old infant with laryngotracheobronchial mucosa involvement who underwent successful tracheal extubation after 10 days of orotracheal intubation and mechanical ventilatory support. Issues regarding airway management and mechanical ventilatory support in the pediatric intensive care unit are discussed.
The'
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Division of Critical Care Medicine, Department of Pediatrics (Drs. The', McLaughlin, Kuluz, and Schleien) and the Department of Dermatology (Dr. Schachner), University of Miami School of Medicine, Miami, FL.
McLaughlin
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Kuluz
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Schachner
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Schleien
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CL