Kearns–Sayre syndrome: Two case reports and a review for the primary care physician

Kearns–Sayre syndrome (KSS) is a mitochondrial encephalopathic disorder. Because mitochondria are ubiquitous organelles that are present in almost every human tissue, their dysfunction can affect nearly any organ system and give rise to a wide range of clinical characteristics. 1: As is the case wit...

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Bibliographic Details
Main Authors: Chad Richmond, Leonard Powell, Zachary D. Brittingham, Alison Mancuso
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2023-04-01
Series:Journal of Family Medicine and Primary Care
Subjects:
Online Access:https://journals.lww.com/10.4103/jfmpc.jfmpc_1790_22
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Summary:Kearns–Sayre syndrome (KSS) is a mitochondrial encephalopathic disorder. Because mitochondria are ubiquitous organelles that are present in almost every human tissue, their dysfunction can affect nearly any organ system and give rise to a wide range of clinical characteristics. 1: As is the case with most diseases associated with mitochondrial DNA (mtDNA) mutations, the clinical features of KSS were defined before modern molecular genetic classifications emerged. 2: The exact prevalence of KSS is unknown; however, estimates place it at about 1:100,000 people. Although it is a rather rare syndrome, the ability to recognize or consider KSS as part of a differential diagnosis is crucial. Reported here are two case reports: 1) a 30-year-old Caucasian female patient who presented for evaluation to her primary care physician’s office and, and 2) A 57-year-old Caucasian female patient long-term C care resident. Guidelines are listed for management as a primary care physician as well as signs and symptoms that are often associated with Kearns–Sayre syndrome and other mitochondrial disorders.
ISSN:2249-4863
2278-7135