Posterior staphyloma in oculocutaneous albinism: another possible cause of reduced visual acuity

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Oculocutaneous albinism

Oculocutaneous albinism (OCA) is a group of inherited disorders of melanin biosynthesis characterized by a generalized reduction in pigmentation of hair, skin and eyes. The prevalence of all forms of albinism varies considerably worldwide and has been estimated at approximately 1/17,000, suggesting that about 1 in 70 people carry a gene for OCA. The clinical spectrum of OCA ranges, with OCA1A b...

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Oculocutaneous albinism represents a group of inherited skin disorders characterized by a generalized reduction of cutaneous, ocular, and pilar pigmentation from the time of birth. Oculocutaneous albinism types I and II are the most common, with several other types described. A defect in the melanin synthesis pathway, resulting in reduced formation of melanin, is responsible for oculocutaneous ...

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Visual deficits in Nepalese patients with oculocutaneous albinism.

BACKGROUND Albinism poses a significant threat to visual functions and causes remarkable ocular morbidity often resulting in visual disabilities. The study aimed at describing the visual status in patients with diagnosed cases of complete oculocutaneous albinism (OCA) attending to a tertiary eye hospital in Nepal. METHODS This was a cross-sectional descriptive hospital-based study of all diag...

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Distribution of oculocutaneous albinism in Zimbabwe.

A survey of 1.3 million schoolchildren in Zimbabwe identified a total of 278 pupils with oculocutaneous albinism (OCA), giving a prevalence of 1 in 4728. Pupils with OCA were identified in every province of the country, but the distribution was not even. In certain areas, notably the capital, Harare, and the eastern province of Manicaland, the frequency was significantly higher than in others. ...

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ژورنال

عنوان ژورنال: Journal of American Association for Pediatric Ophthalmology and Strabismus

سال: 2015

ISSN: 1091-8531

DOI: 10.1016/j.jaapos.2015.06.009