Ophthalmologic manifestations of adult patients with cystic fibrosis

Doctoral Dissertation uoadl:2959502 113 Read counter

Unit:
Faculty of Medicine
Library of the School of Health Sciences
Deposit date:
2021-08-23
Year:
2021
Author:
Giannakouras Panagiotis
Dissertation committee:
Κουτσανδρέα Χρυσάνθη, Ομότιμη Καθηγήτρια, Ιατρική Σχολή, ΕΚΠΑ
Παπακωνσταντίνου Δημήτριος, Καθηγητής, Ιατρική Σχολή, ΕΚΠΑ
Μπρούζας Δημήτριος, Καθηγητής, Ιατρική Σχολή, ΕΚΠΑ
Γεωργάλας Ηλίας, Καθηγητής (επιβλέπων), Ιατρική Σχολή, ΕΚΠΑ
Χατζηστεφάνου Κλειώ, Αναπληρώτρια Καθηγήτρια, Ιατρική Σχολή, ΕΚΠΑ
Χατζηράλλη Ειρήνη, Επίκουρη Καθηγήτρια, Ιατρική Σχολή, ΕΚΠΑ
Δρούτσας Κωνσταντίνος, Επίκουρος Καθηγητής, Ιατρική Σχολή, ΕΚΠΑ
Original Title:
Συμβολή στη μελέτη των επιπτώσεων της κυστικής ίνωσης στους οφθαλμούς
Languages:
Greek
Translated title:
Ophthalmologic manifestations of adult patients with cystic fibrosis
Summary:
Introduction: Cystic fibrosis (CF) is the most common life-shortening recessive genetic disease in Caucasians, affecting primarily the lungs. The objective of our study was to investigate potential ophthalmologic involvement in adult patients with CF.
Methods: Fifty adult patients with cystic fibrosis and 60 age- and sex-matched controls underwent complete ophthalmologic examination including tear-film Break-Up Time (BUT), Macular Thickness and peripapillary Retinal Nerve Fibre Layer (pRNFL) thickness measurements using Spectral Domain-OCT.
Results: CF patients had significantly lower nasal-inferior pRNFL thickness (median 82 IQR 67-102 vs 92.5 IQR 82-107, p=0.005) and lower percentage of normal tear Break-Up Time (56.0% vs 96.7%, p=0.001) than healthy controls. All CF patients with BUT <10 seconds were diagnosed with blepharitis at the time of our assessement. The subgroup of patients homozygous for the most common CF mutation, F508del, had lower nasal-inferior pRNFL thickness (p=0.014) and lower percentage of normal tear Break-Up Time (p=0.001) compared to the control group. Additional findings, present in the CF group only, were punctuate retinal haemorrhages (four patients), vessel tortuosity (four patients), snail-track degeneration and retinal tufts (two patients without refractive error). There were no significant differences in visual acuity, refractive errors, gonioscopic findings, or intraocular pressure between the groups.
Conclusions: Our study is, to the best of our knowledge, the largest ophthalmologic study of patients with cystic fibrosis. We found that CF patients had significantly decreased inferior-quadrant peripapillary retinal nerve fibre layer thickness and decreased tear-film break-up time compared to controls. We highlight the importance of careful regular ophthalmologic assessment and follow-up of these patients.
Main subject category:
Health Sciences
Keywords:
Cystic fibrosis, Ophthalmologic manifestations, Diagnostic techniques, Genetics
Index:
No
Number of index pages:
0
Contains images:
Yes
Number of references:
232
Number of pages:
158
File:
File access is restricted only to the intranet of UoA.

Giannakouras Panagiotis PhD.pdf
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