Polyendocrinopathy and multisystem involvement are common phenotypic features of Kearns-Sayre syndrome

  Dear Editor,   We were interested to read the article by Amergoolov et al. on two patients with Kearns-Sayre Syndrome (KSS) due to single mtDNA deletions who had phenotypic endocrine disorders among other features.1 Patient 1, a 20-year-old female, was diagnosed with hypogonadism, diabetes and...

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Main Author: Josef Finsterer
Format: Article
Language:English
Published: PAGEPress Publications 2025-04-01
Series:European Journal of Translational Myology
Subjects:
Online Access:https://www.pagepressjournals.org/bam/article/view/13634
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author Josef Finsterer
author_facet Josef Finsterer
author_sort Josef Finsterer
collection DOAJ
description   Dear Editor,   We were interested to read the article by Amergoolov et al. on two patients with Kearns-Sayre Syndrome (KSS) due to single mtDNA deletions who had phenotypic endocrine disorders among other features.1 Patient 1, a 20-year-old female, was diagnosed with hypogonadism, diabetes and osteoporosis, and patient 2, a 22-year-old male, was diagnosed with impaired glucose tolerance and osteoporosis.1 It was found that the severity of clinical manifestations increases with the size of the mtDNA deletion, but that other factors such as heteroplasmy, mtDNA duplications or pleioplasmia can also determine the severity of the disease.1 The study is impressive, but some points should be discussed. [...]
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issn 2037-7452
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publishDate 2025-04-01
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series European Journal of Translational Myology
spelling doaj-art-ac26ac5f89aa437db3f7bdfd16497d7f2025-08-20T03:08:28ZengPAGEPress PublicationsEuropean Journal of Translational Myology2037-74522037-74602025-04-0110.4081/ejtm.2025.13634Polyendocrinopathy and multisystem involvement are common phenotypic features of Kearns-Sayre syndromeJosef Finsterer0Neurology Department, Neurology & Neurophysiology Center, Vienna  Dear Editor,   We were interested to read the article by Amergoolov et al. on two patients with Kearns-Sayre Syndrome (KSS) due to single mtDNA deletions who had phenotypic endocrine disorders among other features.1 Patient 1, a 20-year-old female, was diagnosed with hypogonadism, diabetes and osteoporosis, and patient 2, a 22-year-old male, was diagnosed with impaired glucose tolerance and osteoporosis.1 It was found that the severity of clinical manifestations increases with the size of the mtDNA deletion, but that other factors such as heteroplasmy, mtDNA duplications or pleioplasmia can also determine the severity of the disease.1 The study is impressive, but some points should be discussed. [...] https://www.pagepressjournals.org/bam/article/view/13634mtDNAdeletionKearn-Sayre syndromemitochondrial disorderendocrine involvement
spellingShingle Josef Finsterer
Polyendocrinopathy and multisystem involvement are common phenotypic features of Kearns-Sayre syndrome
European Journal of Translational Myology
mtDNA
deletion
Kearn-Sayre syndrome
mitochondrial disorder
endocrine involvement
title Polyendocrinopathy and multisystem involvement are common phenotypic features of Kearns-Sayre syndrome
title_full Polyendocrinopathy and multisystem involvement are common phenotypic features of Kearns-Sayre syndrome
title_fullStr Polyendocrinopathy and multisystem involvement are common phenotypic features of Kearns-Sayre syndrome
title_full_unstemmed Polyendocrinopathy and multisystem involvement are common phenotypic features of Kearns-Sayre syndrome
title_short Polyendocrinopathy and multisystem involvement are common phenotypic features of Kearns-Sayre syndrome
title_sort polyendocrinopathy and multisystem involvement are common phenotypic features of kearns sayre syndrome
topic mtDNA
deletion
Kearn-Sayre syndrome
mitochondrial disorder
endocrine involvement
url https://www.pagepressjournals.org/bam/article/view/13634
work_keys_str_mv AT joseffinsterer polyendocrinopathyandmultisysteminvolvementarecommonphenotypicfeaturesofkearnssayresyndrome