{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,3]],"date-time":"2026-04-03T09:54:46Z","timestamp":1775210086701,"version":"3.50.1"},"reference-count":32,"publisher":"Wiley","license":[{"start":{"date-parts":[[2019,4,10]],"date-time":"2019-04-10T00:00:00Z","timestamp":1554854400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Ophthalmology"],"published-print":{"date-parts":[[2019,4,10]]},"abstract":"<jats:p>Fabry Disease (FD) is a rare X-linked lysosomal storage disorder characterized by systemic and ocular involvement. It has been described an increasing in retinal and conjunctival vessel tortuosity and this feature represents an important marker for the disease. Currently, there is not an objective method to measure and quantify this parameter. We tested a new semi-automatic software measuring retinal and conjunctival vessel tortuosity from eye fundus and conjunctival digital images in a group of FD patients. We performed an observational case-control study evaluating three mathematical parameters describing tortuosity (sum of angle metric [SOAM], product of angle distance [PAD], triangular index [I2e]) obtained from fundus and conjunctival pictures of 11 FD patients and 11 age and sex-matched controls. Both eyes were considered. Mann\u2013Whitney test was used to compare the FD group versus the control group and, within the FD group, male versus female patients. Linear regression analysis was performed to evaluate the possible association of retinal and conjunctival vessels tortuosity parameters with age and with specific markers of systemic disease\u2019s progression. The tortuosity parameters (SOAM, PAD and I2e) were significantly higher in retinal vessels and in conjunctival nasal vessels in FD patients in comparison with the controls (<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M1\"><mml:mrow><mml:mi>p<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn>0.003<\/mml:mn><\/mml:mrow><\/mml:math>, <mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M2\"><mml:mrow><mml:mi>p<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn>0.002<\/mml:mn><\/mml:mrow><\/mml:math>, <mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M3\"><mml:mrow><mml:mi>p<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn>0.001<\/mml:mn><\/mml:mrow><\/mml:math> respectively for retina) (<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M4\"><mml:mrow><mml:mi>p<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn>0.023<\/mml:mn><\/mml:mrow><\/mml:math>, <mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M5\"><mml:mrow><mml:mi>p<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn>0.014<\/mml:mn><\/mml:mrow><\/mml:math>, <mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M6\"><mml:mrow><mml:mi>p<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn>0.001<\/mml:mn><\/mml:mrow><\/mml:math> respectively for nasal conjunctiva). No significant association was found between retinal and conjunctival tortuosity parameters and increasing age or systemic involvement markers. Vessel tortuosity represents an important clinical manifestation in FD. A computer-assisted analysis of retinal and conjunctival vasculature demonstrated an increased vessels tortuosity in patients affected by Fabry disease. This non-invasive technique might be useful to help the diagnosis in early stages, to establish disease severity and monitor its progression.<\/jats:p>","DOI":"10.1155\/2019\/4696429","type":"journal-article","created":{"date-parts":[[2019,4,10]],"date-time":"2019-04-10T20:04:48Z","timestamp":1554926688000},"page":"1-6","source":"Crossref","is-referenced-by-count":12,"title":["Quantitative Analysis of Conjunctival and Retinal Vessels in Fabry Disease"],"prefix":"10.1155","volume":"2019","author":[{"given":"Andrea","family":"Sodi","sequence":"first","affiliation":[{"name":"Department of Surgery and Translational Medicine, Eye Clinic, Careggi Teaching Hospital, University of Florence, Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9954-0407","authenticated-orcid":true,"given":"Chiara","family":"Lenzetti","sequence":"additional","affiliation":[{"name":"Department of Surgery and Translational Medicine, Eye Clinic, Careggi Teaching Hospital, University of Florence, Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniela","family":"Bacherini","sequence":"additional","affiliation":[{"name":"Department of Surgery and Translational Medicine, Eye Clinic, Careggi Teaching Hospital, University of Florence, Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1986-045X","authenticated-orcid":true,"given":"Lucia","family":"Finocchio","sequence":"additional","affiliation":[{"name":"Department of Surgery and Translational Medicine, Eye Clinic, Careggi Teaching Hospital, University of Florence, Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tommaso","family":"Verdina","sequence":"additional","affiliation":[{"name":"Institute of Ophthalmology, University of Modena and Reggio Emilia, Modena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isabella","family":"Borg","sequence":"additional","affiliation":[{"name":"Department of Pathology, The Medical School, University of Malta, Msida, Malta"},{"name":"Department of Pathology, Medical Genetics Unit, Mater Dei Hospital, Msida, Malta"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca","family":"Cipollini","sequence":"additional","affiliation":[{"name":"Department of Surgery and Translational Medicine, Eye Clinic, Careggi Teaching Hospital, University of Florence, Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fatema Ullah","family":"Patwary","sequence":"additional","affiliation":[{"name":"Department of Surgery and Translational Medicine, Eye Clinic, Careggi Teaching Hospital, University of Florence, Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilaria","family":"Tanini","sequence":"additional","affiliation":[{"name":"Referral Center for Cardiomyopathies, Careggi Teaching Hospital, Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Zoppetti","sequence":"additional","affiliation":[{"name":"Department of Informatic Engineering, University of Siena, Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stanislao","family":"Rizzo","sequence":"additional","affiliation":[{"name":"Department of Surgery and Translational Medicine, Eye Clinic, Careggi Teaching Hospital, University of Florence, Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gianni","family":"Virgili","sequence":"additional","affiliation":[{"name":"Department of Surgery and Translational Medicine, Eye Clinic, Careggi Teaching Hospital, University of Florence, Florence, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","reference":[{"key":"1","doi-asserted-by":"publisher","DOI":"10.1186\/1479-7364-2-5-297"},{"key":"2","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1051\/jbio\/2002196020161","volume":"196","year":"2002","journal-title":"Journal de la Socie\u0301te\u0301 de Biologie"},{"issue":"3","key":"3","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1001\/jama.281.3.249","volume":"281","year":"1999","journal-title":"JAMA"},{"issue":"6","key":"4","doi-asserted-by":"crossref","first-page":"425","DOI":"10.7326\/0003-4819-146-6-200703200-00007","volume":"146","year":"2007","journal-title":"Annals of Internal Medicine"},{"issue":"1","key":"5","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1186\/1750-1172-5-30","volume":"5","year":"2010","journal-title":"Orphanet Journal of Rare Diseases"},{"issue":"2","key":"6","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1097\/00005792-200203000-00003","volume":"81","year":"2002","journal-title":"Medicine (Baltimore)"},{"issue":"2","key":"7","first-page":"71","volume":"24","year":"2001","journal-title":"Journal of Inherited Metabolic Disease"},{"issue":"4","key":"8","doi-asserted-by":"crossref","first-page":"338","DOI":"10.7326\/0003-4819-138-4-200302180-00014","volume":"138","year":"2003","journal-title":"Annals of Internal Medicine"},{"key":"9","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e3181f13b75"},{"key":"10","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2796.2004.01300.x"},{"key":"11","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2014.01.014"},{"key":"12","doi-asserted-by":"publisher","DOI":"10.1016\/j.braindev.2012.12.002"},{"key":"13","doi-asserted-by":"publisher","DOI":"10.1159\/000308723"},{"key":"14","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbspin.2003.10.015"},{"issue":"2","key":"15","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1136\/bjo.2006.100602","volume":"91","year":"2007","journal-title":"The British Journal of Ophthalmology"},{"key":"16","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9394(72)90071-2"},{"key":"17","doi-asserted-by":"publisher","DOI":"10.1076\/opge.24.3.129.15609"},{"key":"18","first-page":"625","volume":"3","year":"1980","journal-title":"Journal Fran\u00e7ais D\u2019Ophtalmologie"},{"key":"19","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1001\/archopht.1979.01020010327008","volume":"97","year":"1979","journal-title":"Archives of Ophthalmology"},{"issue":"6","key":"20","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1001\/archopht.1965.00970040762005","volume":"74","year":"1965","journal-title":"Archives of Ophthalmology"},{"key":"21","doi-asserted-by":"publisher","DOI":"10.1111\/j.1442-9071.2005.00990.x"},{"key":"22","first-page":"299","volume-title":"The eye in Fabry disease","year":"2010"},{"issue":"12","key":"23","doi-asserted-by":"crossref","first-page":"4734","DOI":"10.1167\/iovs.05-0646","volume":"46","year":"2005","journal-title":"Investigative Opthalmology & Visual Science"},{"key":"24","doi-asserted-by":"publisher","DOI":"10.1001\/archopht.125.11.1523"},{"key":"25","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.07-1353"},{"key":"26","doi-asserted-by":"publisher","DOI":"10.1111\/j.1755-3768.2008.01448.x"},{"key":"27","doi-asserted-by":"publisher","DOI":"10.1007\/bf02173368"},{"key":"28","doi-asserted-by":"publisher","DOI":"10.1111\/j.1755-3768.2012.02576.x"},{"key":"29","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2004.00219.x"},{"key":"30","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2362.2004.01309.x"},{"issue":"1","key":"31","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/S0181-5512(05)81024-1","volume":"28","year":"2005","journal-title":"Journal Fran\u00e7ais D\u2019Ophtalmologie"},{"key":"32","doi-asserted-by":"publisher","DOI":"10.1111\/j.1442-9071.2006.01233.x"}],"container-title":["Journal of Ophthalmology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/downloads.hindawi.com\/journals\/joph\/2019\/4696429.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/joph\/2019\/4696429.xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/joph\/2019\/4696429.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,4,10]],"date-time":"2019-04-10T20:04:56Z","timestamp":1554926696000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.hindawi.com\/journals\/joph\/2019\/4696429\/"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,4,10]]},"references-count":32,"alternative-id":["4696429","4696429"],"URL":"https:\/\/doi.org\/10.1155\/2019\/4696429","relation":{},"ISSN":["2090-004X","2090-0058"],"issn-type":[{"value":"2090-004X","type":"print"},{"value":"2090-0058","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,4,10]]}}}