Visual functions in phenylketonuria-evaluating the dopamine and long-chain polyunsaturated fatty acids depletion hypotheses.

Background: In phenylketonuria presymptomatic treatment following newborn screening prevents severe mental and physical impairment. The reasons for subtle impairments of cerebral functions despite early treatment remain unclear. We assessed a broad spectrum of visual functions in early-treated patients with phenylketonuria and evaluated two hypotheses-the dopamine and the long-chain polyunsaturated fatty acids (LCPUFAs) depletion hypotheses.

Severe Arthrographis kalrae keratomycosis in an immunocompetent patient.

Purpose: To describe a severe case of keratomycosis caused by Arthrographis kalrae requiring repeated keratoplasty.

Methods: A 42-year-old otherwise healthy soft contact lens wearer developed a unilateral central corneal ulcer. Treatment with topical and systemic voriconazole is described.

Accumulation of A2-E in mitochondrial membranes of cultured RPE cells.

Background: Lipofuscin occurs in association with various blinding diseases, including ARMD. Formation of lipofuscin is considered to be initiated by the inability of the RPE lysosome to degrade constituents of phagocytosed material resulting in its intralysosomal accumulation. Thus, the deposition of abnormal retinoid adducts causing the autofluorescent properties of RPE lipofuscin originates from abnormal products of the retinoid cycle contained in phagocytosed photoreceptor outer segments.