Senior syndrome (senior-loken syndrome; tubulointerstitial nephropathy syndrome; loken-senior syndrome; renal-retinal syndrome; renal dysplasia and retinal aplasia)

General: Autosomal recessive trait; pleiotropic gene with variable expression; death before adulthood by renal failure with uremia; both sexes affected; onset in early childhood.

Ocular: Visual loss progressing to complete blindness; progressive tapetoretinal degeneration that becomes manifest in early infancy; retinitis pigmentosa; rubeosis iridis; bilateral or unilateral cataracts; spherophakia; anterior lenticonus; hydrophthalmos; corneal opacities; pallor of optic disk; narrowed arterioles; bone spicule formation.

Clinical: Nephropathy (tubulointerstitial type); growth and mental retardation; nocturia; thirst; polyuria; short stature; hearing loss; brain maldevelopment; convulsions; osteomalacia; deafness; nephronophthisis; osteomalacia; arterial hypertension; aortic insufficiency.

Fillastre JP, et al. Familial chronic interstitial nephropathy and retinal dystrophy (Senior-Loken syndrome). Nouv Presse Med 1974; 3:309.

Olmer M, et al. Letterer-Senior-Loken's syndromes. Nouv Presse Med 1974; 3:1032.

Rosenthal G, et al. Senior's syndrome (hereditary renal retinal dysplasia) associated with cataracts. Ann Ophthalmol 1992; 24:104-l05.

Schuman JS, et al. Senior-Loken syndrome and Coats' disease. Am J Ophthalmol 1985; 100:822-827.

Senior B, et al. Juvenile familial nephropathy with tapetoretinal degeneration: a new oculo-renal dystrophy. Am J Ophthalmol 1961; 52:625.