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Pediatr Pulmonol. 2012 Sep;47(9):923-5. doi: 10.1002/ppul.22522. Epub 2012 Feb 10.

Laryngeal obstruction in congenital plasminogen deficiency.

Author information

1
Department of Otolaryngology/Head and Neck Surgery, Hadassah Medical Center, Hebrew University, Jerusalem, Israel.

Abstract

Type 1 congenital plasminogen deficiency (CPD) is a rare autosomal recessive disease which causes formation of fibrin pseudomembranes that affect multiple systems/organs: the eyes, respiratory system, urinary and genital systems, gastrointestinal system, and the central nervous system. We present a rare manifestation of the disease-severe upper airway obstruction due to a rapidly growing mass in the supraglottic region-6 months after dental treatment under general anesthesia. The management of such a manifestation has not been discussed in the current literature. Due to deterioration in his clinical status, the patient eventually underwent both a tracheotomy in order to bypass the obstruction, and excision of the supraglottic mass. Within a few days the mass recurred with complete obstruction of the upper airway.

PMID:
22328462
DOI:
10.1002/ppul.22522
[Indexed for MEDLINE]

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