J. Graham Dobbie

Central Areolar Pigment Epithelial Dystrophy

We examined nine members of a family with a unique hereditary macular dystrophy by using stereoscopic color photography, fluorescein angiography, electroretinography, electro-oculography, dark adaptation, H-R-R color plates, and the Farnsworth-Munsell 100-hue test. The disorder was transmitted as an autosomal-dominant trait with increased penetrance and variable expression. Four of the family members, representing three successive generations, exhibited defects in macular pigmentation ranging from a 1-disk diameter excavation to a more subtle central loss of macular pigment. Only one eye with macular hemorrhage had decreased visual acuity; visual acuity in this eye was 20/25 when the hemorrhage resolved. All other retinal function studies were normal. The unusual nonprogressive areolar depigmentation of the central macula together with normal retinal functions made it impossible to classify this disorder, and indicated a new and unreported dominant macular dystrophy, central areolar pigment epithelial dystrophy.

Protein kinases in the subretinal fluid

Abstract Samples of subretinal fluid from patients with retinal detachment of various duration were examined. Protein kinase activity increased with the duration of the detachment. The specific activity of the enzyme increased up to 14 days of retinal detachment, subsequently decreased up to 21 days and thereafter remained constant. Cyclic AMP dependent and cyclic AMP independent protein kinase activities were present. The data suggest that protein kinases may be involved in the degradation of rod outer segments during retinal detachment.

Amines in the Subretinal Fluid and Aqueous

We examined 49 samples of subretinal fluid and nine samples of aqueous from patients with retinal detachments. Seven amines (histamine, dopamine, methylamine, ethanolamine, dimethylamine, pyridoxamine, and piperidine) were positively identified and quantitated. The amines in the subretinal fluid were similar to those present in the aqueous. The presence of histamine in the subretinal fluid, observed only in fresh retinal detachment, suggested an inflammatory process, at least in the early stages of some cases of retinal detachment. This is consistent with the clinical observations that mild uveitis frequently accompanies a rhegmatogenous retinal detachment.