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PLASTIC BRONCHITIS - IS IT A SYNDROMIC DISEASE?

LIDIJA SRKOČ MAJČICA ; Opća bolnica Zabok i Bolnica hrvatskih veterana, Odjel za pedijatriju, Zabok, Hrvatska
DOROTEA BARTONIČEK ; Klinika za pedijatriju KBC Zagreb, Zavod za pedijatrijsku kardiologiju, Zagreb, Hrvatska
DRAŽEN BELINA ; Zavod za kardijalnu kirurgiju KBC Zagreb, Zagreb, Hrvatska
SVEN SEIWERTH ; Zavod za patologiju, Medicinski fakultet Sveučilišta u Zagrebu, Zagreb, Hrvatska
IVAN MALČIĆ ; Klinika za pedijatriju KBC Zagreb, Zavod za pedijatrijsku kardiologiju, Zagreb, Hrvatska

Sažetak

Plastic or cast bronchitis is a very rare and severe condition that occurs as a late complication with increasing frequency in children after the establishment of the Fontan’s circulation. The case study presents an 11-year-old boy with an initial diagnosis of hypoplastic left heart syndrome (HLHS) who developed plastic bronchitis features with typical clinical, macroscopic and microscopic fi ndings. Possible etiological factors are considered in the disease pathogenesis, from some of the borderline original criteria (hypoplasia of large pulmonary blood vessels) to numerous operating and interventional procedures. Regardless of the fact that there is no unique etiological factor, the disease is marked by an underlying disorder in the lymphatic drainage. Possible etiological factors are evaluated in the paper, with extensive analysis of a possible lymphatic fl ow disorder in cardiopulmonary relationship with Fontan’s circulation. Furthermore, other possible late complications with Fontan’s circulation are presented, as well as a comprehensive and successful therapeutic approach to the plastic bronchitis syndrome.

Ključne riječi

Fontan’s circulation; late complications; plastic bronchitis; children; etiology; treatment

Hrčak ID:

245740

URI

https://hrcak.srce.hr/245740

Datum izdavanja:

6.11.2020.

Podaci na drugim jezicima: hrvatski

Posjeta: 1.604 *