Manifestações oculares da síndrome de Scheie: Relato de cinco casos

Manifestações oculares da síndrome de Scheie: Relato de cinco casos

Autores:

Diane Ruschel Marinho,
Silvana Gattani,
James Marchiori,
Carlos Roberto Galia,
Samuel Rymer

ARTIGO ORIGINAL

Arquivos Brasileiros de Oftalmologia

versão impressa ISSN 0004-2749versão On-line ISSN 1678-2925

Arq. Bras. Oftalmol. vol.60 no.5 São Paulo out. 1997

http://dx.doi.org/10.5935/0004-2749.19970024

SUMMARY

The Scheie's Syndrome (Mucopolyssacharidosis I-S) is an autossomal recessive disease characterized by the absence of the alpha-L-iduronidase enzyme which causes an accumulation of glycosaminoglycans throughout the connective tissue cells. The patients’ intelligence and their life expectancy are often normal. The individual functional capacity is usually reduced by ocular involvement.

The authors report five cases of Scheie's Syndrome, the results of corneal keratoplasty in three eyes of two patients and the management of high intraocular pressure. The etiology of glaucoma in this Syndrome is discussed.

Key-words: Scheie's Syndrome; Mucopolyssacharidosis; Keratoplasty

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