Kearns-Sayre syndrome


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Kearns-Sayre syn·drome

(kernz sār), [MIM*530000]
a form of chronic progressive external ophthalmoplegia with associated cardiac conduction defects, short stature, and hearing loss; a sporadically occurring mitochondrial myopathy presenting in childhood.

Kearns-Sayre syndrome

[kernz sār]
Etymology: Thomas P. Kearns, American ophthalmologist, 1922-2011; George P. Sayre, American pathologist, 1911-1991
progressive ophthalmoplegia, pigmentary degeneration of the retina, myopathy, ataxia, and cardiac conduction defect, with onset before the age of 20 years. Almost all patients have large mitochondrial DNA deletions, and ragged red fibers are seen on muscle biopsy. Also called ophthalmoplegia plus.

Kearns-Sayre syndrome

A mitochondrial disease characterized by chronic progressive external ophthalmoplegia–paralysis of ocular muscles and mitochondrial myopathy combined with retinal deterioration, heart disease, hearing loss, DM, renal disease See Mitochondrial disease.

Kearns-Sayre syn·drome

(kĕrnz sār sindrōm)
A form of chronic progressive external ophthalmoplegia with associated cardiac conduction defects, short stature, and hearing loss; a sporadically occurring mitochondrial myopathy presenting in childhood.

Kearns-Sayre syndrome (KSS)

A syndrome caused by major rearrangements of, and often large deletions from, the MITOCHONDRIAL DNA. The condition features paralysis of the eye-moving muscles with double vision; drooping eyelids; degeneration of the retinas; defects in the conducting muscle tissue of the heart; respiratory distress; and in some cases staggering walk, deafness and DIABETES.

Kearns,

Thomas P., U.S. ophthalmologist, 1922–.
Kearns-Sayre syndrome - chronic progressive external ophthalmoplegia with associated cardiac conduction defects, short stature, and hearing loss.

Sayre,

George P., U.S. ophthalmologist, 1911–.
Kearns-Sayre syndrome - see under Kearns

Kearns-Sayre syn·drome

(kĕrnz sār sindrōm) [MIM*530000]
Chronic progressive external ophthalmoplegia with associated cardiac conduction defects, short stature, and hearing loss.
References in periodicals archive ?
Clinical characteristics of eight cases with Kearns-Sayre syndrome in children.
Mitochondrial DNA deletions in progressive external ophthalmoplegia and Kearns-Sayre syndrome.
A novel mitochondrial DNA deletion in a Chinese girl with Kearns-Sayre syndrome.
Kearns-Sayre syndrome – 3 case reports and review of clinical feature.
Patient with Kearns-Sayre syndrome exhibiting abnormal magnetic resonance image of the brain.
Evidence for cardioembolic stroke in a case of Kearns-Sayre syndrome.
Kearns-Sayre syndrome with a phenocopy of choroideremia instead of pigmentary retinopathy.
The atypical pigmentary retinopathy of Kearns-Sayre syndrome.
Macular lesion resembling adult-onset vitelliform macular dystrophy in Kearns-Sayre syndrome with multiple mtDNA deletions.
Histological, enzymatic and mitochondrial DNA studies in patients with Kearns-Sayre syndrome and chronic progressive external ophthalmoplegia.
Deterioration of Kearns-Sayre syndrome following articaine administration for local anesthesia.