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Descriptor English: Spinal Muscular Atrophies of Childhood
Descriptor Spanish: Atrofias Musculares Espinales de la Infancia
Descriptor atrofias musculares espinales de la infancia
Entry term(s) atrofia muscular espinal infantil
atrofia muscular espinal juvenil
atrofia muscular juvenil espinal
atrofia muscular medular infantil
enfermedad de Kugelberg-Welander
enfermedad de Werdnig-Hoffmann
Scope note: Grupo de enfermedades hereditarias recesivas que se caracterizan por atrofia muscular progresiva e hipotonía. Se clasifican en tipo I (Enfermedad de Werdnig-Hoffman), tipo II (forma intermedia), y tipo III (enfermedad de Kugelberg-Welander). El tipo I es mortal en la infancia, el tipo II tiene un comienzo infantil tardío y se asocia con una supervivencia hasta la segunda o tercera década de la vida. El tipo III tiene su comienzo en la infancia y es lentamente progresiva. (Traducción libre del original: J Med Genet 1996 Apr:33(4):281-3).
Descriptor Portuguese: Atrofias Musculares Espinais da Infância
Descriptor French: Amyotrophies spinales infantiles
Entry term(s): HMN (Hereditary Motor Neuropathy) Proximal Type I
Infantile Muscular Atrophy
Infantile Spinal Muscular Atrophy
Juvenile Muscular Atrophy
Juvenile Spinal Muscular Atrophy
Kugelberg Welander Disease
Kugelberg Welander Syndrome
Kugelberg-Welander Disease
Kugelberg-Welander Syndrome
Muscular Atrophy, Infantile
Muscular Atrophy, Juvenile
Muscular Atrophy, Spinal, Infantile
Muscular Atrophy, Spinal, Infantile Chronic Form
Muscular Atrophy, Spinal, Intermediate Type
Muscular Atrophy, Spinal, Type I
Muscular Atrophy, Spinal, Type II
Muscular Atrophy, Spinal, Type III
Proximal Hereditary Motor Neuropathy Type I
SMA, Infantile Acute Form
Spinal Muscular Atrophy 1
Spinal Muscular Atrophy Type 2
Spinal Muscular Atrophy Type I
Spinal Muscular Atrophy Type II
Spinal Muscular Atrophy Type III
Spinal Muscular Atrophy, Infantile
Spinal Muscular Atrophy, Juvenile
Spinal Muscular Atrophy, Mild Childhood and Adolescent Form
Spinal Muscular Atrophy, Type 3
Spinal Muscular Atrophy, Type I
Spinal Muscular Atrophy, Type II
Spinal Muscular Atrophy, Type III
Type I Spinal Muscular Atrophy
Type II Spinal Muscular Atrophy
Type III Spinal Muscular Atrophy
Werdnig Hoffman Disease
Werdnig Hoffmann Disease
Werdnig-Hoffmann Disease
Tree number(s): C10.228.854.468.800
C10.574.500.812
C10.574.562.500.750
C10.668.467.500.750
C16.320.400.765
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D014897
Scope note: A group of recessive inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3)
Annotation: infantile, childhood or adolescent: for other, MUSCULAR ATROPHY, SPINAL is available
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: Muscular Atrophy (1966-1987)
Spinal Cord Diseases (1966-1987)
Public MeSH Note: 2000; see WERDNIG-HOFFMAN DISEASE 1991-1999, see MUSCULAR ATROPHY, SPINAL 1988-90
History Note: 2000(1988)
Entry Version: SPINAL MUSCULAR ATROPHIES CHILDHOOD
DeCS ID: 24374
Unique ID: D014897
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1991/01/01
Date of Entry: 1987/04/28
Revision Date: 2016/02/08
Spinal Muscular Atrophies of Childhood - Preferred
Concept UI M0022919
Scope note A group of recessive inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3)
Preferred term Spinal Muscular Atrophies of Childhood
Muscular Atrophy, Spinal, Type II - Narrower
Concept UI M0336136
Preferred term Muscular Atrophy, Spinal, Type II
Entry term(s) Spinal Muscular Atrophy Type 2
Spinal Muscular Atrophy Type II
Spinal Muscular Atrophy, Type II
Type II Spinal Muscular Atrophy
HMN (Hereditary Motor Neuropathy) Proximal Type I - Narrower
Concept UI M0336135
Preferred term HMN (Hereditary Motor Neuropathy) Proximal Type I
Entry term(s) Infantile Muscular Atrophy
Infantile Spinal Muscular Atrophy
Muscular Atrophy, Infantile
Muscular Atrophy, Spinal, Infantile
Muscular Atrophy, Spinal, Type I
Proximal Hereditary Motor Neuropathy Type I
SMA, Infantile Acute Form
Spinal Muscular Atrophy 1
Spinal Muscular Atrophy Type I
Spinal Muscular Atrophy, Infantile
Spinal Muscular Atrophy, Type I
Type I Spinal Muscular Atrophy
Werdnig Hoffman Disease
Werdnig Hoffmann Disease
Werdnig-Hoffmann Disease
Juvenile Spinal Muscular Atrophy - Narrower
Concept UI M0336134
Preferred term Juvenile Spinal Muscular Atrophy
Entry term(s) Juvenile Muscular Atrophy
Kugelberg Welander Disease
Kugelberg Welander Syndrome
Kugelberg-Welander Disease
Kugelberg-Welander Syndrome
Muscular Atrophy, Juvenile
Muscular Atrophy, Spinal, Type III
Spinal Muscular Atrophy Type III
Spinal Muscular Atrophy, Juvenile
Spinal Muscular Atrophy, Mild Childhood and Adolescent Form
Spinal Muscular Atrophy, Type 3
Spinal Muscular Atrophy, Type III
Type III Spinal Muscular Atrophy
Muscular Atrophy, Spinal, Infantile Chronic Form - Narrower
Concept UI M0531340
Preferred term Muscular Atrophy, Spinal, Infantile Chronic Form
Entry term(s) Muscular Atrophy, Spinal, Intermediate Type



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