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83rd Annual Meeting of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery

German Society of Oto-Rhino-Laryngology, Head and Neck Surgery

16.05. - 20.05.2012, Mainz

Cyclic Neutropenia: ENT-Symptoms and their treatment

Meeting Abstract

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  • corresponding author presenting/speaker Sabine Nospes - Hals-, Nasen-, Ohrenklinik und Poliklinik, Schwerpunkt Kommunikationsstörungen, Mainz, Germany
  • author Annerose Keilmann - Hals-, Nasen-, Ohrenklinik und Poliklinik, Schwerpunkt Kommunikationsstörungen, Mainz, Germany

German Society of Oto-Rhino-Laryngology, Head and Neck Surgery. 83rd Annual Meeting of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery. Mainz, 16.-20.05.2012. Düsseldorf: German Medical Science GMS Publishing House; 2012. Doc12hno45

DOI: 10.3205/12hno45, URN: urn:nbn:de:0183-12hno457

Published: July 23, 2012

© 2012 Nospes et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.


Outline

Text

Objective: Cyclic neutropenia is an autosomal dominant condition with neutropenia in variable familiar or sporadical expression. The neutrophil count cycles, typically over 21 days.

Material and methods: In a 30 years old women and her 10 years old daughter the typical symptoms and the treatment were observed.

Results: Since her early childhood the mother suffered from recurrent otitis media, resulting in deafness on the right ear and a moderate sensorineural hearing loss on the left ear. Tonsillectomy was conducted because of chronic tonsillitis. Following chronic inflammations/aphthous ulcers/abscesses of the oral mucosa the first dental loss occured at the age of 14, total loss of teeth at the age of 20. During adulthood she came down with two pneumonias and recurrent abscesses of skin. We did a hearing aid fitting and recommended a cochlea implantation. Her daughter suffered from recurrent otitis media/mucocutaneous infections since the first year of life. A retroauricular abscess needed to be operated. In pure tone audiometry a mild sensorineural hearing loss was found on both ears. The diagnosis was confirmed in the age of 19/9 years by hematologic examination. Treated with granulocyte colony stimulating factor both patients showed a decrease of infections, no further progression of hearing loss and a remarked increase of health-related quality of life.

Conclusion: Symptoms associated with congenital cyclic neutropenia are found in the early childhood. In patients with recurrent aphthous oral ulcers and recurrent otitis media we recommend hematologic diagnosis/therapy.


References

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James RM, Kinsey SE. The investigation and management of chronic neutropenia in children. Arch Dis Child. 2006 Oct;91(10):852-8. DOI: 10.1136/adc.2006.094706 External link