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Fundal Findings in a Female Carrier of X-linked Ocular Albinism FREE

Ahmad M. Mansour, MD; Mark J. Greenwald, MD; Lee M. Jampol, MD; Nicholas Hrisomalos, MD
Arch Ophthalmol. 1987;105(6):750-751. doi:10.1001/archopht.1987.01060060036019
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To the Editor.  —Detection of female carriers of X-linked genetic disorders is essential for proper diagnosis and genetic counseling. In ocular albinism, iris transillumination defects, fundal changes, electrophysiological testing, and skin biopsy to detect macromelanosomes can complement the search for the carrier state.1-4 The characteristic fundal changes in female heterozygotes rarely have been described. We present the first documentation, to our knowledge, of fluorescein angiographic findings in a carrier of ocular albinism.

Report of a Case.  —A 15-year-old white female adolescent presented with a decrease in vision of the left eye of a few days' duration. Examination revealed visual acuities of 20/20 in the right eye and 20/100 in the left eye; this latter acuity improved to 20/40 with hyperopic astigmatic correction. There was no iris transillumination. Funduscopy of each eye revealed a stippled macula and patchy pigmentation of the retinal pigment epithelium anterior to the arcades (Figs 1

REFERENCES

Goodman G, Ripps H, Siegel IM:  Sex-linked disorders: Trait expressivity in males and carrier females . Arch Ophthalmol 1965;;73:387-398.
Krill AE:  X chromosomal-linked diseases affecting the eye: Status of the heterozygote female . Trans Am Ophthalmol Soc 1969;;67:548-602.
O'Donnell FE Jr, Hambrick GW Jr, Green WR, et al:  X-linked ocular albinism: An oculocutaneous macromelanosomal disorder . Arch Ophthalmol 1976;;94:1883-1892.
Garner A, Jay BS:  Macromelanosomes in X-linked ocular albinism . Histopathology 1980;;4:243-254.
Lyon MF:  Sex chromatin and gene action in the mammalian X chromosome . Am J Hum Genet 1962;;14:135-148.

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Goodman G, Ripps H, Siegel IM:  Sex-linked disorders: Trait expressivity in males and carrier females . Arch Ophthalmol 1965;;73:387-398.
Krill AE:  X chromosomal-linked diseases affecting the eye: Status of the heterozygote female . Trans Am Ophthalmol Soc 1969;;67:548-602.
O'Donnell FE Jr, Hambrick GW Jr, Green WR, et al:  X-linked ocular albinism: An oculocutaneous macromelanosomal disorder . Arch Ophthalmol 1976;;94:1883-1892.
Garner A, Jay BS:  Macromelanosomes in X-linked ocular albinism . Histopathology 1980;;4:243-254.
Lyon MF:  Sex chromatin and gene action in the mammalian X chromosome . Am J Hum Genet 1962;;14:135-148.

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