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Klin Monbl Augenheilkd 2016; 233(04): 448-452
DOI: 10.1055/s-0041-111802
Klinische Studie
Georg Thieme Verlag KG Stuttgart · New York

Increased Prevalence of Flammer Syndrome in Patients with Retinitis Pigmentosa

Gehäuftes Vorkommen des Flammer-Syndroms bei Patienten mit Retinitis pigmentosa
K. Konieczka
Department of Ophthalmology, University of Basel, Switzerland (Chairman ad interim: Prof. S. Orguel)
,
S. Koch
Department of Ophthalmology, University of Basel, Switzerland (Chairman ad interim: Prof. S. Orguel)
,
A. Schoetzau
Department of Ophthalmology, University of Basel, Switzerland (Chairman ad interim: Prof. S. Orguel)
,
M. G. Todorova
Department of Ophthalmology, University of Basel, Switzerland (Chairman ad interim: Prof. S. Orguel)
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Publication History

Publication Date:
26 April 2016 (online)

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Abstract

Background: “Retinitis pigmentosa” refers to a group of degenerative eye diseases with a genetic background. Flammer syndrome encompasses a set of symptoms and signs, mainly but not exclusively related to dysregulation of blood vessels. The purpose of the present study was to determine, with the help of a questionnaire, whether symptoms of Flammer syndrome occur more often in patients with retinitis pigmentosa than in controls. Methods: 76 patients with retinitis pigmentosa (members of the Swiss patient organization for retinitis pigmentosa) and 274 control subjects answered a questionnaire (Flammer Syndrome Questionnaire) on 15 symptoms and signs of Flammer syndrome. Results: Seven of 15 symptoms and signs of Flammer syndrome were significantly more often positive in retinitis pigmentosa patients than in controls. Six additional symptoms and signs occurred non-significantly more often and 2 non-significantly less often in patients with retinitis pigmentosa. Conclusion: Retinitis pigmentosa patients suffer significantly more often from symptoms and signs of the Flammer syndrome than control subjects. This includes low body mass index, low blood pressure, feeling cold, migraine, increased smell perception and perfectionism. The reason for this association between retinitis pigmentosa and Flammer syndrome and the potential implications need to be determined.

Zusammenfassung

Hintergrund: Retinitis pigmentosa umfasst eine Gruppe genetisch bedingter, degenerativer Augenerkrankungen. Das Flammer-Syndrom umfasst die primäre vaskuläre Dysregulation zusammen mit weiteren vaskulären und nicht-vaskulären Symptomen und Zeichen. Das Ziel dieser Studie war es, mit Hilfe eines Fragebogens zu prüfen, ob die Symptome des Flammer-Syndroms bei Patienten mit Retinitis pigmentosa häufiger oder seltener sind, als bei Kontroll-Personen. Methode: 76 Patienten mit Retinitis pigmentosa und 274 Kontroll-Personen füllten einen Multiple-Choice Fragebogen (Flammer-Syndrom Fragebogen) aus. Es wurde nach 15 Symptomen und Zeichen des Flammer-Syndroms gefragt. Ergebnisse: 7 von 15 Symptomen oder Zeichen des Flammer-Syndroms waren signifikant und 6 weitere nicht signifikant häufiger bei Patienten mit Retinitis pigmentosa als bei Kontroll-Personen. 2 Symptomen oder Zeichen waren nicht signifikant seltener bei Patienten mit Retinitis pigmentosa. Schlussfolgerungen: Patienten mit Retinitis pigmentosa haben Symptome und Zeichen des Flammer-Syndroms häufiger als Kontroll-Personen. Signifikant häufiger sind ein tiefer BMI, tiefer Blutdruck, schnelles Frieren, Migräne, erhöhte Empfindlichkeit auf Gerüche und Perfektionismus. Der Grund dieser Zusammenhänge ist noch unklar und potentielle Folgen sind Gegenstand zukünftiger Untersuchungen.

Key words

Retinitis pigmentosa - Flammer syndrome - endothelin - ocular blood flow - vascular dysregulation

Schlüsselwörter

Retinitis pigmentosa - Flammer-Syndrom - Endothelin - Augendurchblutung - vaskuläre Dysregulation
 

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