Kearns-Sayre Syndrome Follow-up

Updated: Jul 05, 2019
  • Author: Anna Purna Basu, BMBCh, PhD, MA, NIHR; Chief Editor: Luis O Rohena, MD, MS, FAAP, FACMG  more...
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Follow-up

Further Outpatient Care

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  • Kearns-Sayre syndrome (KSS) can involve many systems and organs. Clinicians must maintain comprehensive surveillance. Be especially alert for signs or symptoms of diabetes mellitus and for heart block; the latter may develop at any stage.

  • Annual ECG, echocardiography, audiometry, and biochemistry to screen for common endocrine disturbances are recommended. [33]

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Complications

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  • Heart block is a significant and preventable cause of mortality.

  • Deterioration has been reported after local anesthesia with articaine. [34]

  • Avoid the use of drugs known to be toxic to mitochondria, such as valproic acid, tetracyclines, biguanides, chloramphenicol, barbiturates, phenothiazines, and zidovudine. [35]

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Prognosis

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  • Kearns-Sayre syndrome is a progressive disorder, and the prognosis for patients with the condition is poor. Death is common in the third or fourth decade of life. [36]

  • Disease progression can be predicted to some extent by the size and location of the deletion and the degree of skeletal muscle heteroplasmy. [37]

  • As in other mtDNA deletion disorders, women who have Kearns-Sayre syndrome have an increased risk of clinically affected offspring. The risk is currently estimated at approximately 1 per 24 births. [16] Genetic counselling is recommended so options for affected women considering pregnancy can be discussed. [38]

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Patient Education

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  • Participation in an exercise-training program can lead to a subjective improvement in muscle-related symptoms, enhanced aerobic exercise capacity, and increased muscle strength.

  • Patients can access the Kearns-Sayre Syndrome Information Page maintained by the National Institute of Neurological Disorders and Stroke for information on the disorder and support organizations.

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