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DYSTROPHIE CORNEENNE MACULAIRE [1 record]
Record 1 - internal organization data 2026-01-23
Record 1, English
Record 1, Subject field(s)
- Visual Disorders
Record 1, Main entry term, English
- macular corneal dystrophy
1, record 1, English, macular%20corneal%20dystrophy
correct, noun
Record 1, Abbreviations, English
Record 1, Synonyms, English
- Groenouw type II corneal dystrophy 2, record 1, English, Groenouw%20type%20II%20corneal%20dystrophy
correct, noun
- Groenouw's type II corneal dystrophy 3, record 1, English, Groenouw%27s%20type%20II%20corneal%20dystrophy
correct, noun
- Fehr spotted dystrophy 4, record 1, English, Fehr%20spotted%20dystrophy
correct, noun
Record 1, Textual support, English
Record number: 1, Textual support number: 1 CONT
Macular corneal dystrophy is the only major autosomal recessive stromal dystrophy. It results from the abnormal synthesis of keratin sulfate proteoglycan, occurs early in life, and causes visual loss in the first or second decade of life. ... With accumulation of glycosaminoglycans within stromal keratocytes, the endothelium, and the stroma, there is a diffuse limbus-to-limbus haze in all layers of the stroma ... accompanied by corneal thinning. Central, focal, white deposits are seen in the stroma against a background of variable stroma haze. 5, record 1, English, - macular%20corneal%20dystrophy
Record 1, Key term(s)
- Groenouw type 2 corneal dystrophy
- Groenouw's type 2 corneal dystrophy
Record 1, French
Record 1, Domaine(s)
- Troubles de la vision
Record 1, Main entry term, French
- dystrophie cornéenne maculaire
1, record 1, French, dystrophie%20corn%C3%A9enne%20maculaire
correct, feminine noun
Record 1, Abbreviations, French
Record 1, Synonyms, French
- dystrophie cornéenne de Groenouw de type II 2, record 1, French, dystrophie%20corn%C3%A9enne%20de%20Groenouw%20de%20type%20II
correct, feminine noun
- dystrophie cornéenne de type II de Groenouw 3, record 1, French, dystrophie%20corn%C3%A9enne%20de%20type%20II%20de%20Groenouw
correct, feminine noun
Record 1, Textual support, French
Record number: 1, Textual support number: 1 CONT
La dystrophie cornéenne maculaire [...] est aussi appelée dystrophie cornéenne de Groenouw de type II. Hérédité : autosomique récessive. Début : au cours de la [première] décennie de la vie. Symptômes : détérioration visuelle lentement progressive qui devient sévère au cours des [deuxième] et [troisième] décennies. [...] Signes : au début de l'évolution de la maladie, des opacités blanchâtres superficielles centrales se développent. [...] ces opacités s’étendent de façon périphérique au limbe et au stroma profonde jusqu'à la membrane de Descemet. Le stroma cornéen intermédiaire développe un voile progressif et diffus. [...] Histopathologie : les glycosaminoglycanes(GAG) s’accumulent de manière intracellulaire et dans l'espace extracellulaire [...] 2, record 1, French, - dystrophie%20corn%C3%A9enne%20maculaire
Record 1, Key term(s)
- dystrophie cornéenne de Groenouw de type 2
- dystrophie cornéenne de type 2 de Groenouw
Record 1, Spanish
Record 1, Textual support, Spanish
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