Manifestations of the Chronic Granulomatous Disease in ENT Locality
Authors:
M. Navratilova 1,2; G. Bugová 1; M. Jeseňák 3; T. Balhárek 4; M. Janíčková 5; A. Hajtman 1
Authors‘ workplace:
Klinika otorinolaryngológie a chirurgie hlavy a krku, Jesseniova lekárska fakulta v Martine, Univerzita, Komenského v Bratislave, Univerzitná nemocnica Martin
1; Oddelenie otorinolaryngológie FNsP F. D. Roosevelta, Banská Bystrica
2; Centrum pre vrodené poruchy imunity, Klinika detí a dorastu, Jesseniova lekárska fakulta v Martine, Univerzita Komenského v Bratislave, Univerzitná nemocnica Martin
3; Ústav patologickej anatómie, Jesseniova lekárska fakulta v Martine, Univerzita Komenského v Bratislave, Univerzitná nemocnica Martin
4; Klinika stomatológie a maxilofaciálnej chirurgie, Jesseniova lekárska fakulta v Martine, Univerzita, Komenského v Bratislave, Univerzitná nemocnica Martin
5
Published in:
Otorinolaryngol Foniatr, 67, 2018, No. 4, pp. 119-123.
Category:
Case Reports
Overview
Chronic granulomatous disease falls into a group of primary disorders of the immune system. The disease is caused by a genetic defect of one of the components of the multienzyme NADPH - oxidase complex in phagocytes, which leads to an insufficient formation of reactive forms of oxygen and to a disorder of respiratory burst. Decreased immunity is manifested in early age by recurrent infections (especially by deep skin infections, organ abscesses, lymphadenitis). The case history demonstrates an 8 years old child with genetically verified, autosomal recessive hereditary, chronic granulomatous disease. The aim is to point out the course of the disease and its clinical complications, while focusing especially on the manifestations in the ENT field and on highlighting the importance of an interdisciplinary approach to the diagnosis and treatment of childhood patients with congenital immune system disorder.
KEYWORDS
chronic granulomatous disease (CGD), primary immunodeficiency, phagocytosis, recurrent infections, respiratory burst
Sources
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Labels
Audiology Paediatric ENT ENT (Otorhinolaryngology)Article was published in
Otorhinolaryngology and Phoniatrics
2018 Issue 4
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