Congenital Hypertropthy of Retinal Pigment Epithelium Associated with Familial Adenomatous Polyposis Coli
Journal of the Korean Ophthalmological Society
;
: 1187-1193, 1992.
Artigo
em Coreano
| WPRIM
| ID: wpr-139098
ABSTRACT
Congenital hypertrophy of the retinal pigment epithelium (CHRPE) has been described in association with systemic disorders such as Gardner's syndrome (intestinal polyposis, osteomas, and benign soft-tissue tumors), Turgot's syndrome (glioma-polyposis syndrome) and familial adenomatous polyposis (FAP) of the colon. FamiIial adenomatous polyposis is an autosomal dominant disorder characterized by hundreds of polyps throughout the entire colon, and in all patients carcinoma of the color, develops at the age of 40 to 50 years. We describe a family with familial adenomatous polylpcsis coli and congenital hypertrophy of the retinal pigment epithelium. All of them except mother showed flat, weII-demarcated, round to oval pigmented patches of CHRPE. We emphasize the importance of CHRPE as a clinical marker in identifying patients With FAP since they are at risk for cancer.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Osteoma
/
Pólipos
/
Retinaldeído
/
Síndrome de Gardner
/
Biomarcadores
/
Colo
/
Polipose Adenomatosa do Colo
/
Epitélio Pigmentado da Retina
/
Hipertrofia
/
Mães
Limite:
Humanos
Idioma:
Coreano
Revista:
Journal of the Korean Ophthalmological Society
Ano de publicação:
1992
Tipo de documento:
Artigo
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