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Descriptor English: Kearns-Sayre Syndrome
Descriptor Spanish: Síndrome de Kearns-Sayre
Descriptor síndrome de Kearns-Sayre
Entry term(s) síndrome de Kearns
Scope note: Trastorno mitocondrial que se caracteriza por la triada constituida por OFTALMOPLEJIA EXTERNA crónica progresiva, enfernedad miocárdica (MIOCARDIOPATÍAS) con bloqueo de la conducción (BLOQUEO CARDÍACO) y RETINITIS PIGMENTOSA. El comienzo de la enfermedad ocurre en la primera o segunda décadas de la vida. Puede haber también incremento del contenido de proteínas en el LCR, sordera neurosensorial, convulsiones y signos piramidales. En la biopsia muscular se encuentran fibras rojas rotas. (Adams et al., Principles of Neurology, 6th ed, p984)
Descriptor Portuguese: Síndrome de Kearns-Sayre
Descriptor French: Syndrome de Kearns-Sayre
Entry term(s): CPEO with Myopathies
CPEO with Myopathy
CPEO with Ragged Red Fibers
Chronic Progressive External Ophthalmoplegia with Myopathy
Cpeo With Ragged-Red Fibers
Cytopathy, Kearn-Sayre Mitochondrial
Kearn Sayre Mitochondrial Cytopathy
Kearn Syndrome
Kearn-Sayre Mitochondrial Cytopathy
Kearns Sayre Shy Daroff Syndrome
Kearns Sayre Syndrome
Kearns Syndrome
Kearns' Syndrome
Kearns-Sayre Mitochondrial Cytopathy
Kearns-Sayre-Shy-Daroff Syndrome
Mitochondrial Cytopathy, Kearn-Sayre
Myopathies, CPEO with
Myopathy, CPEO with
Oculocraniosomatic Syndrome
Oculocraniosomatic Syndromes
Ophthalmoplegia Plus Syndrome
Ophthalmoplegia Plus Syndromes
Ophthalmoplegia, Pigmentary Degeneration of Retina, and Cardiomyopathy
Ophthalmoplegia, Progressive External, With Ragged-Red Fibers
Ophthalmoplegia-Plus Syndrome
Sayre Syndrome, Kearns
Syndrome, Kearns
Syndrome, Kearns Sayre
Syndrome, Kearns'
Syndrome, Kearns-Sayre
Syndrome, Kearns-Sayre-Shy-Daroff
Syndrome, Oculocraniosomatic
Syndrome, Ophthalmoplegia Plus
Tree number(s): C05.651.460.700.500
C10.292.562.750.250.500
C10.597.622.447.511.500
C10.668.491.500.700.500
C11.590.472.250.500
C11.768.585.658.500.627
C14.280.238.510
C18.452.660.560.700.500
C23.550.291.500.688.500
C23.888.592.636.447.511.500
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D007625
Scope note: A mitochondrial disorder featuring the triad of chronic progressive EXTERNAL OPHTHALMOPLEGIA, cardiomyopathy (CARDIOMYOPATHIES) with conduction block (HEART BLOCK), and RETINITIS PIGMENTOSA. Disease onset is in the first or second decade. Elevated CSF protein, sensorineural deafness, seizures, and pyramidal signs may also be present. Ragged-red fibers are found on muscle biopsy. (Adams et al., Principles of Neurology, 6th ed, p984)
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Previous Indexing: Heart Block (1966-1982)
Ophthalmoplegia (1966-1982)
Retinitis Pigmentosa (1966-1982)
Public MeSH Note: 2010; see KEARNS-SAYER SYNDROME 2000-2009, see KEARNS SYNDROME 1991-2004, see OPHTHALMOPLEGIA 1983-1990
History Note: 2010 (1983)
DeCS ID: 28115
Unique ID: D007625
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1991/01/01
Date of Entry: 1982/04/26
Revision Date: 2015/06/22
Kearns-Sayre Syndrome - Preferred
Concept UI M0011934
Scope note A mitochondrial disorder featuring the triad of chronic progressive EXTERNAL OPHTHALMOPLEGIA, cardiomyopathy (CARDIOMYOPATHIES) with conduction block (HEART BLOCK), and RETINITIS PIGMENTOSA. Disease onset is in the first or second decade. Elevated CSF protein, sensorineural deafness, seizures, and pyramidal signs may also be present. Ragged-red fibers are found on muscle biopsy. (Adams et al., Principles of Neurology, 6th ed, p984)
Preferred term Kearns-Sayre Syndrome
Entry term(s) CPEO with Myopathies
CPEO with Myopathy
CPEO with Ragged Red Fibers
Chronic Progressive External Ophthalmoplegia with Myopathy
Cpeo With Ragged-Red Fibers
Cytopathy, Kearn-Sayre Mitochondrial
Kearn Sayre Mitochondrial Cytopathy
Kearn Syndrome
Kearn-Sayre Mitochondrial Cytopathy
Kearns Sayre Shy Daroff Syndrome
Kearns Sayre Syndrome
Kearns Syndrome
Kearns' Syndrome
Kearns-Sayre Mitochondrial Cytopathy
Kearns-Sayre-Shy-Daroff Syndrome
Mitochondrial Cytopathy, Kearn-Sayre
Myopathies, CPEO with
Myopathy, CPEO with
Oculocraniosomatic Syndrome
Oculocraniosomatic Syndromes
Ophthalmoplegia Plus Syndrome
Ophthalmoplegia Plus Syndromes
Ophthalmoplegia, Pigmentary Degeneration of Retina, and Cardiomyopathy
Ophthalmoplegia, Progressive External, With Ragged-Red Fibers
Ophthalmoplegia-Plus Syndrome
Sayre Syndrome, Kearns
Syndrome, Kearns
Syndrome, Kearns Sayre
Syndrome, Kearns'
Syndrome, Kearns-Sayre
Syndrome, Kearns-Sayre-Shy-Daroff
Syndrome, Oculocraniosomatic
Syndrome, Ophthalmoplegia Plus



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