PEDIATRICS Vol. 94 No. 1 July 1994, pp. 111-112
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Senior-Loken Syndrome: Revisited

Bradley A. Warady MD1, Gerhard Cibis MD1, Uri Alon MD1, Douglas Blowey MD1, and Stanley Hellerstein MD1

1 Dept of Pediatrics, Sections of Nephrology and Ophthalmology, The Children's Mercy Hospital, Kansas City, MO 64108

The combination of familial juvenile nephronophthisis (FJN) and congenital amaurosis of Leber, the so called Senior-Loken Syndrome, was first described by Senior et al in 1961.1 Despite recognition of the syndrome for > 30 years, its occurrence is relatively rare and the basis for the oculorenal association remains unresolved. Our recent experience with two patients who had end-stage renal disease (ESRD) as a manifestation of the syndrome prompted this review.

CASE HISTORIES

A.M. is an 11-year-old girl who complained of generalized fatigue over the previous 3 weeks. The patient had been diagnosed with Leber's amaurosis at 3 months of age.

Submitted on September 1, 1993
Accepted on November 18, 1993