PMID- 34469897
OWN - NLM
STAT- MEDLINE
DCOM- 20220303
LR  - 20220303
IS  - 1423-0267 (Electronic)
IS  - 0030-3755 (Linking)
VI  - 245
IP  - 1
DP  - 2022
TI  - Quantitative Optical Coherence Tomography Angiography Biomarkers for Alport 
      Syndrome.
PG  - 41-48
LID - 10.1159/000519242 [doi]
AB  - PURPOSE: The aim of this study was to evaluate microvascular abnormalities of 
      patients with Alport syndrome (AS) using optical coherence tomography angiography 
      (OCT-A) quantitative biomarkers. METHODS: This was cross sectional, prospective 
      evaluation of consecutive patients with AS and healthy subjects. AS diagnosis was 
      performed by the genetic test. All participants underwent a retinal vasculature 
      evaluation by spectral-domain optical coherence tomography (SD-OCT) and OCT-A of 
      the macula. Quantitative analysis included whole vascular density, foveal 
      avascular zone area, fractal dimension (FD), and lacunarity (LAC). RESULTS: 
      Ninety-four eyes were included in this study, 45 eyes from patients with AS and 
      49 eyes from healthy subjects. The pathogenic mutation in the COL4A5 gene on the 
      chromosome X was found in 14 patients; the pathogenic autosomal recessive 
      mutations in the COL4A3 gene were found in 9 patients. Quantitative evaluation 
      demonstrated a significant difference between AS and healthy subjects on LAC of 
      the superficial capillary plexus and deep capillary plexus (DCP) (p < 0.001 and p 
      < 0.001, respectively) and on FD in the DCP (p < 0.001). CONCLUSION: The DCP 
      Alport patients have a higher vessel nonuniformity than DCP of healthy subjects. 
      We hypothesize that endothelial cell lesion in the setting of low resistance at 
      the DCP circuit could lead to long-term structural disorganization.
CI  - (c) 2021 S. Karger AG, Basel.
FAU - Cunha, Ana Maria
AU  - Cunha AM
AD  - Department of Ophthalmology, Centro Hospitalar Universitario de Sao Joao, Porto, 
      Portugal.
FAU - Teixeira, Daniela
AU  - Teixeira D
AD  - Faculty of Medicine, University of Porto, Porto, Portugal.
FAU - Cabral, Diogo
AU  - Cabral D
AD  - Instituto de Oftalmologia Dr. Gama Pinto, Lisboa, Portugal.
FAU - Estrela-Silva, Sergio
AU  - Estrela-Silva S
AD  - Department of Ophthalmology, Centro Hospitalar Universitario de Sao Joao, Porto, 
      Portugal.
AD  - Department of Surgery and Physiology, Faculty of Medicine of University of Porto, 
      Porto, Portugal.
FAU - Falcao-Reis, Fernando
AU  - Falcao-Reis F
AD  - Department of Ophthalmology, Centro Hospitalar Universitario de Sao Joao, Porto, 
      Portugal.
AD  - Department of Surgery and Physiology, Faculty of Medicine of University of Porto, 
      Porto, Portugal.
FAU - Carneiro, Angela
AU  - Carneiro A
AD  - Department of Ophthalmology, Centro Hospitalar Universitario de Sao Joao, Porto, 
      Portugal.
AD  - Department of Surgery and Physiology, Faculty of Medicine of University of Porto, 
      Porto, Portugal.
LA  - eng
PT  - Journal Article
DEP - 20210901
PL  - Switzerland
TA  - Ophthalmologica
JT  - Ophthalmologica. Journal international d'ophtalmologie. International journal of 
      ophthalmology. Zeitschrift fur Augenheilkunde
JID - 0054655
RN  - 0 (Biomarkers)
SB  - IM
MH  - Biomarkers
MH  - Cross-Sectional Studies
MH  - Fluorescein Angiography/methods
MH  - Humans
MH  - *Macula Lutea
MH  - *Nephritis, Hereditary/diagnosis/genetics/pathology
MH  - Retinal Vessels/pathology
MH  - Tomography, Optical Coherence/methods
MH  - Visual Acuity
OTO - NOTNLM
OT  - Alport syndrome
OT  - Optical coherence tomography angiography
OT  - Quantitative biomarkers
OT  - Spectral-domain optical coherence tomography
EDAT- 2021/09/02 06:00
MHDA- 2022/03/04 06:00
CRDT- 2021/09/01 20:13
PHST- 2021/03/28 00:00 [received]
PHST- 2021/08/12 00:00 [accepted]
PHST- 2021/09/02 06:00 [pubmed]
PHST- 2022/03/04 06:00 [medline]
PHST- 2021/09/01 20:13 [entrez]
AID - 000519242 [pii]
AID - 10.1159/000519242 [doi]
PST - ppublish
SO  - Ophthalmologica. 2022;245(1):41-48. doi: 10.1159/000519242. Epub 2021 Sep 1.