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Descripteurs (mots clés)
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Sterol O-acyltransferase
An enzyme that catalyzes the formation of cholesterol esters by the direct transfer of the fatty acid group from a fatty acyl CoA derivative. This enzyme has been found in the adrenal gland, gonads, liver, intestinal mucosa, and aorta of many mammalian species. EC 2.3.1.26.
Synonyme(s)
Acyl-CoA Cholestérol acyltransférase; Cholestérol acyltransféraseRelation(s)
- voir aussi au terme générique : [Descripteurs (mots clés)] Acyltransferases
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Case Report: Management of Corneal Clouding from Lecithin: Cholesterol Acyltransferase Deficiency / Christine Zemsky in OVS : Optometry & Vision Science, vol. 96, 02 (Février 2019)
[article]
in OVS : Optometry & Vision Science > vol. 96, 02 (Février 2019)
Titre : Case Report: Management of Corneal Clouding from Lecithin: Cholesterol Acyltransferase Deficiency Type de document : article de périodique Auteurs : Christine Zemsky ; Suzanne Sherman ; Hermann Schubert Année de publication : 2019 Langues : Français (fre) Descripteurs (mots clés) : [Thésaurus HELB]:Paramédical:carences
[Thésaurus Mesh]Cornée
[Thésaurus Mesh]Lécithines
[Thésaurus Mesh]Opacification de la capsule postérieure
[Thésaurus Mesh]Sterol O-acyltransferase
[Thésaurus Mesh]Transplantation de cornéeMots-clés : kératoplastie Résumé : SIGNIFICANCE Given that there are few reported cases of lecithin:cholesterol acyltransferase (LCAT) deficiency, recognition of the condition with proper management is notable. Long-term follow-up and contact lens fitting after penetrating keratoplasty provide best possible outcomes.
PURPOSE The purpose of this study was to report a case of LCAT deficiency successfully treated with penetrating keratoplasty and longer-term follow-up with contact lens fitting.
CASE REPORT A 43-year-old white woman of Italian descent presented with corneal clouding and trouble with night vision. The patient had a history of LCAT deficiency, irritable bowel syndrome, gastroesophageal reflux disease, osteoporosis, and hemolytic anemia. Slit-lamp examination demonstrated corneal haze throughout the corneal layers. The corneas had normal pachymetry. Given the opacity of each cornea (right greater than left) and decreased night vision, penetrating keratoplasty was performed on the right eye. At post-operative month 16, the corneal graft remained clear. The patient was able to achieve a best-corrected visual acuity of 20/30+ with a scleral lens.
CONCLUSIONS Penetrating keratoplasty may be necessary to provide better quality of vision in LCAT deficiency patients, specifically to enhance one's contrast sensitivity, despite relatively good Snellen visual acuity.Permalink : https://bibliotheque.helb-prigogine.be/opac_css/index.php?lvl=notice_display&id= [article]Exemplaires
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