Laryngeal obstruction in congenital plasminogen deficiency

Pediatric Pulmonology
Jonathan CohenDavid Shoseyov

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.

References

Apr 15, 1986·American Journal of Ophthalmology·R C EagleH D Perry
Jun 1, 1969·The British Journal of Ophthalmology·J C McGrandJ Harry
Jul 10, 2003·Survey of Ophthalmology·Volker Schuster, Stefan Seregard
Apr 21, 2005·Thrombosis and Haemostasis·Francis J Castellino, Victoria A Ploplis
Jan 15, 2009·Haemophilia : the Official Journal of the World Federation of Hemophilia·R Mehta, A D Shapiro

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Citations

Jan 28, 2015·Haemophilia : the Official Journal of the World Federation of Hemophilia·M Granito, B Schmidt

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