Kearns-Sayre syndrome presenting as renal tubular acidosis.

Eviatar, L; Shanske, S; Gauthier, B; Abrams, C; Maytal, J; Slavin, M; Valderrama, E; DiMauro, S · Neurology · 1990

case_report · Level V

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Abstract

Renal tubular acidosis and tetany were the 1st manifestations of Kearns-Sayre syndrome in a 5-year-old child. Subsequently, he developed progressive external ophthalmoplegia, ptosis, retinopathy, heart block, and endocrinopathy. There was a 7.5-kb deletion of mitochondrial DNA documented in muscle, kidney, skin fibroblasts, and leukocytes, providing evidence for a multisystem mitochondrial cytopathy.

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