PMID- 32518974
OWN - NLM
STAT- MEDLINE
DCOM- 20210818
LR  - 20210818
IS  - 1435-702X (Electronic)
IS  - 0721-832X (Linking)
VI  - 258
IP  - 11
DP  - 2020 Nov
TI  - Optical coherence tomography (OCT) and OCT angiography allow early identification 
      of sickle cell maculopathy in children and correlate it with systemic risk 
      factors.
PG  - 2551-2561
LID - 10.1007/s00417-020-04764-y [doi]
AB  - PURPOSE: To determine the presence of sickle cell retinopathy and maculopathy and 
      to identify associations between markers of hemolysis and systemic and ocular 
      manifestations in children affected by sickle cell disease. METHODS: Eighteen 
      children with sickle cell disease, aged 5-16 years, underwent complete eye 
      examination including best-corrected visual acuity, slit-lamp biomicroscopy, 
      ophthalmoscopy after pharmacological mydriasis, spectral-domain optical coherence 
      tomography (SD-OCT), and optical coherence tomography angiography (OCTA). Blood 
      test results and clinical history information were collected for each child, 
      including fetal hemoglobin (HbF), hemoglobin (Hb), hematocrit (Htc), mean 
      corpuscular volume (MCV), mean corpuscular hemoglobin concentration (MCHC), 
      reticulocytes percentage (%ret), lactic dehydrogenase (LDH), total and direct 
      bilirubin, glomerular filtration rate, number of painful crises, acute chest 
      syndromes, and splenic sequestration. Therapeutic regimen and transfusion therapy 
      were also evaluated. RESULTS: Sixteen of 36 eyes (44.4%) had non-proliferative 
      sickle cell retinopathy on ophthalmoscopic evaluation. No patients had 
      proliferative sickle cell retinopathy. In 13 of 36 eyes (36.1%), SD-OCT and OCTA 
      detected signs of sickle cell maculopathy. Nine eyes (25%) presented sickle cell 
      retinopathy and maculopathy, 7 eyes (19.4%) sickle cell retinopathy alone, and 4 
      eyes (11.1%) sickle cell maculopathy alone. A statistically significant 
      association was found between sickle cell retinopathy; lower levels of HbF, Hb, 
      and Htc; and higher MCV and percentage of reticulocytes. Sickle cell maculopathy 
      was associated with lower values of H and Htc and higher levels of reticulocytes 
      and total bilirubin. CONCLUSIONS: We identified early signs of sickle cell 
      retinopathy and maculopathy in a pediatric population with SD-OCT and OCTA. These 
      two retinal complications were more frequent in children with higher hemolytic 
      rates.
FAU - Grego, L
AU  - Grego L
AUID- ORCID: 0000-0003-0768-4970
AD  - Department of Medicine- Ophthalmology, University of Udine, Udine, Italy.
FAU - Pignatto, S
AU  - Pignatto S
AD  - Department of Medicine- Ophthalmology, University of Udine, Udine, Italy.
FAU - Alfier, F
AU  - Alfier F
AD  - Department of Medicine- Ophthalmology, University of Udine, Udine, Italy.
FAU - Arigliani, M
AU  - Arigliani M
AD  - Department of Medicine- Paediatrics, University of Udine, Udine, Italy.
FAU - Rizzetto, F
AU  - Rizzetto F
AD  - Department of Ophthalmology, Hospital of Pordenone, Pordenone, Italy.
FAU - Rassu, N
AU  - Rassu N
AD  - Department of Medicine- Ophthalmology, University of Udine, Udine, Italy.
FAU - Samassa, F
AU  - Samassa F
AD  - Department of Medicine- Ophthalmology, University of Udine, Udine, Italy.
FAU - Prosperi, R
AU  - Prosperi R
AD  - Department of Medicine- Ophthalmology, University of Udine, Udine, Italy.
FAU - Barbieri, F
AU  - Barbieri F
AD  - Department of Paediatrics, Hospital of Pordenone, Pordenone, Italy.
FAU - Dall'Amico, R
AU  - Dall'Amico R
AD  - Department of Paediatrics, Hospital of Pordenone, Pordenone, Italy.
FAU - Cogo, P
AU  - Cogo P
AD  - Department of Medicine- Paediatrics, University of Udine, Udine, Italy.
FAU - Lanzetta, P
AU  - Lanzetta P
AD  - Department of Medicine- Ophthalmology, University of Udine, Udine, Italy. 
      paolo.lanzetta@uniud.it.
LA  - eng
PT  - Journal Article
DEP - 20200609
PL  - Germany
TA  - Graefes Arch Clin Exp Ophthalmol
JT  - Graefe's archive for clinical and experimental ophthalmology = Albrecht von 
      Graefes Archiv fur klinische und experimentelle Ophthalmologie
JID - 8205248
SB  - IM
MH  - *Anemia, Sickle Cell/complications/diagnosis
MH  - Child
MH  - Fluorescein Angiography
MH  - Humans
MH  - *Macular Degeneration
MH  - *Retinal Diseases/diagnosis/etiology
MH  - Risk Factors
MH  - Tomography, Optical Coherence
MH  - Visual Acuity
OTO - NOTNLM
OT  - OCT angiography
OT  - Pediatric age
OT  - Sickle cell disease
OT  - Sickle cell maculopathy
OT  - Sickle cell retinopathy
EDAT- 2020/06/11 06:00
MHDA- 2021/08/19 06:00
CRDT- 2020/06/11 06:00
PHST- 2019/11/26 00:00 [received]
PHST- 2020/05/20 00:00 [accepted]
PHST- 2020/05/13 00:00 [revised]
PHST- 2020/06/11 06:00 [pubmed]
PHST- 2021/08/19 06:00 [medline]
PHST- 2020/06/11 06:00 [entrez]
AID - 10.1007/s00417-020-04764-y [pii]
AID - 10.1007/s00417-020-04764-y [doi]
PST - ppublish
SO  - Graefes Arch Clin Exp Ophthalmol. 2020 Nov;258(11):2551-2561. doi: 
      10.1007/s00417-020-04764-y. Epub 2020 Jun 9.