TY - JOUR
T1 - Ophthalmologic and systemic manifestations of Alström's disease
AU - Millay, Robert H.
AU - Weleber, Richard G.
AU - Heckenlively, John R.
N1 - Funding Information:
From the Departments of Ophthalmology (Drs. Millay and Weleber) and Medical Genetics (Dr. Weleber), Oregon Health Sciences University, Portland, Oregon; and the Jules Stein Eye Institute, Los Angeles, California (Dr. Heckenlively). This study was supported in part by funds from the National Retinitis Pigmentosa Foundation, Fighting Blindness, Baltimore, Maryland.
PY - 1986/10/15
Y1 - 1986/10/15
N2 - Alström's syndrome consists of pigmentary retinopathy, diabetes, obesity, normal mental capacity, and sensorineural deafness. The early retinal findings are optic atrophy, salt-and-pepper pigment epithelial abnormalities, and marked vascular attenuation. Later in the disease process, diffuse areas of chorioretinal atrophy and large clumps of pigment develop. Severe visual loss in the first decade is characteristic and is an important point in the differential diagnosis. Clinically evident renal disease is a variable age-related phenomenon, and is probably the most frequent cause of death.
AB - Alström's syndrome consists of pigmentary retinopathy, diabetes, obesity, normal mental capacity, and sensorineural deafness. The early retinal findings are optic atrophy, salt-and-pepper pigment epithelial abnormalities, and marked vascular attenuation. Later in the disease process, diffuse areas of chorioretinal atrophy and large clumps of pigment develop. Severe visual loss in the first decade is characteristic and is an important point in the differential diagnosis. Clinically evident renal disease is a variable age-related phenomenon, and is probably the most frequent cause of death.
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U2 - 10.1016/0002-9394(86)90078-4
DO - 10.1016/0002-9394(86)90078-4
M3 - Article
C2 - 3766665
AN - SCOPUS:0022973179
SN - 0002-9394
VL - 102
SP - 482
EP - 490
JO - American journal of ophthalmology
JF - American journal of ophthalmology
IS - 4
ER -