Kearns-Sayre Syndrome [Kearnsův-Sayreův syndrom]

topical
11
Terms

Kearnsův syndrom
Kearnsův-Sayrův syndrom
Kearnův-Sayrův syndrom
KSS

 

Chronic Progressive External Ophthalmoplegia with Myopathy
CPEO with Myopathy
CPEO with Ragged Red Fibers
Cpeo With Ragged-Red Fibers
Kearn-Sayre Mitochondrial Cytopathy
Kearns Sayre Syndrome
Kearns Syndrome
Kearns-Sayre Mitochondrial Cytopathy
Kearns-Sayre-Shy-Daroff Syndrome
Kearns' Syndrome
Oculocraniosomatic Syndrome
Ophthalmoplegia Plus Syndrome
Ophthalmoplegia-Plus Syndrome
Ophthalmoplegia, Pigmentary Degeneration of Retina, and Cardiomyopathy
Ophthalmoplegia, Progressive External, With Ragged-Red Fibers

Persistent link   https://www.medvik.cz/link/D007625
Definition

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)

DUI
D007625 MeSH Browser
CUI
M0011934
Previous indexing
Heart Block (1966-1982); Ophthalmoplegia (1966-1982); Retinitis Pigmentosa (1966-1982)
History note
2010 (1983)
Public note
2010; see KEARNS-SAYER SYNDROME 2000-2009, see KEARNS SYNDROME 1991-2004, see OPHTHALMOPLEGIA 1983-1990

Allowable subheadings

BL
blood
CF
cerebrospinal fluid
CI
chemically induced
CL
classification
CO
complications 1
DI
diagnosis 5
DG
diagnostic imaging
DH
diet therapy
DT
drug therapy
EC
economics
EM
embryology
EN
enzymology
EP
epidemiology
EH
ethnology
ET
etiology 3
GE
genetics 2
HI
history
IM
immunology
ME
metabolism 1
MI
microbiology
MO
mortality
NU
nursing
PS
parasitology
PA
pathology
PP
physiopathology 2
PC
prevention & control
PX
psychology
RT
radiotherapy
RH
rehabilitation
SU
surgery
TH
therapy
UR
urine
VE
veterinary
VI
virology

C Diseases
C05 Musculoskeletal Diseases 1 120
C05.651 Muscular Diseases 553
C05.651.460 Mitochondrial Myopathies 40
C05.651.460.700 Ophthalmoplegia, Chronic Progressive External 4
C05.651.460.700.500 Kearns-Sayre Syndrome 11
C10 Nervous System Diseases 1 878
C10.292 Cranial Nerve Diseases 60
C10.292.562 Ocular Motility Disorders 101
C10.292.562.750 Ophthalmoplegia 33
C10.292.562.750.250 Ophthalmoplegia, Chronic Progressive External 4
C10.292.562.750.250.500 Kearns-Sayre Syndrome 11
C10.597 Neurologic Manifestations 1 052
C10.597.622 Paralysis 280
C10.597.622.447 Ophthalmoplegia 33
C10.597.622.447.511 Ophthalmoplegia, Chronic Progressive External 4
C10.597.622.447.511.500 Kearns-Sayre Syndrome 11
C10.668 Neuromuscular Diseases 330
C10.668.491 Muscular Diseases 553
C10.668.491.500 Mitochondrial Myopathies 40
C10.668.491.500.700 Ophthalmoplegia, Chronic Progressive External 4
C10.668.491.500.700.500 Kearns-Sayre Syndrome 11
C11 Eye Diseases 1 489
C11.590 Ocular Motility Disorders 101
C11.590.472 Ophthalmoplegia 33
C11.590.472.250 Ophthalmoplegia, Chronic Progressive External 4
C11.590.472.250.500 Kearns-Sayre Syndrome 11
C11.768 Retinal Diseases 402
C11.768.585 Retinal Degeneration 75
C11.768.585.658 Retinal Dystrophies 6
C11.768.585.658.500 Retinitis Pigmentosa 55
C11.768.585.658.500.627 Kearns-Sayre Syndrome 11
C11.768.585.658.500.813 Usher Syndromes 8
C14 Cardiovascular Diseases 10 507
C14.280 Heart Diseases 2 867
C14.280.238 Cardiomyopathies 1 057
C14.280.238.028 Arrhythmogenic Right Ventricular Dysplasia 60
C14.280.238.057 Cardiomyopathy, Alcoholic 16
C14.280.238.070 Cardiomyopathy, Dilated 340
C14.280.238.100 Cardiomyopathy, Hypertrophic 505
C14.280.238.160 Cardiomyopathy, Restrictive 71
C14.280.238.190 Chagas Cardiomyopathy 5
C14.280.238.235 Diabetic Cardiomyopathies 68
C14.280.238.281 Endocardial Fibroelastosis 20
C14.280.238.406 Endomyocardial Fibrosis 31
C14.280.238.458 Glycogen Storage Disease Type IIb 11
C14.280.238.510 Kearns-Sayre Syndrome 11
C14.280.238.615 Myocardial Reperfusion Injury 225
C14.280.238.625 Myocarditis 377
C14.280.238.719 Peripartum Cardiomyopathy
C14.280.238.812 Sarcoglycanopathies 2
C14.280.238.906 Takotsubo Cardiomyopathy 76
C18 Nutritional and Metabolic Diseases 172
C18.452 Metabolic Diseases 1 196
C18.452.660 Mitochondrial Diseases 159
C18.452.660.560 Mitochondrial Myopathies 40
C18.452.660.560.700 Ophthalmoplegia, Chronic Progressive External 4
C18.452.660.560.700.500 Kearns-Sayre Syndrome 11
C23 Pathological Conditions, Signs and Symptoms 451
C23.550 Pathologic Processes 195
C23.550.291 Disease Attributes 1 208
C23.550.291.500 Chronic Disease 5 045
C23.550.291.500.688 Ophthalmoplegia, Chronic Progressive External 4
C23.550.291.500.688.500 Kearns-Sayre Syndrome 11
C23.888 Signs and Symptoms 3 919
C23.888.592 Neurologic Manifestations 1 052
C23.888.592.636 Paralysis 280
C23.888.592.636.447 Ophthalmoplegia 33
C23.888.592.636.447.511 Ophthalmoplegia, Chronic Progressive External 4
C23.888.592.636.447.511.500 Kearns-Sayre Syndrome 11

Mitochondrial cytopathy Disease MeSH Browser

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