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Phenotypes for disease #02439 (RSMD (dystrophy, muscular, rigid spine (RSMD)))
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Phenotype details
: additional information on the phenotype of the individual, preferably use HPO terms only (http://www.human-phenotype-ontology.org/)
Diagnosis/Initial
: initial diagnosis, before molecular testing
Diagnosis/Definite
: phenotype individual after molecular testing (OMIM abbreviation)
Inheritance
: Indicates the inheritance of the phenotype in the family; unknown, familial (autosomal/X-linked, dominant/ recessive), paternal (Y-linked), maternal (mitochondrial), isolated (sporadic) or complex
All options:
Unknown
Familial
Familial, autosomal dominant
Familial, autosomal recessive
Familial, X-linked
Familial, X-linked dominant
Familial, X-linked dominant, male sparing
Familial, X-linked recessive
Paternal, Y-linked
Maternal, mitochondrial
Isolated (sporadic)
Di-genic
Complex
- = Not applicable
Age/Examination
: age at which the individual was examined.
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Diagnosis
: age diagnosis was confirmed
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Onset
: Age first symptoms disease appeared in individual:
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Phenotype/Onset
: individual's phenotype at Age/Onset described using HPO
Protein
: result from protein staining
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all entries in 2019 or 2020, and before March, 2020
Numeric
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126 entries on 2 pages. Showing entries 1 - 100.
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Phenotype ID
Phenotype details
Diagnosis/Initial
Diagnosis/Definite
Inheritance
Age/Examination
Age/Diagnosis
Age/Onset
Phenotype/Onset
Protein
Owner
Individual ID
0000082212
delayed motor milestones, never jump or run, wheelchair bound 34y; IHC SEPN1 low, spotted aggregates; WB no SEPN1; CPK 590
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00104271
0000082213
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104272
0000082214
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104273
0000082215
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104274
0000082216
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104275
0000082217
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104276
0000082219
WB SEPN1 normal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104278
0000082220
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104279
0000082221
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104280
0000082222
s6m, w11m
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00104281
0000082223
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104282
0000082224
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104283
0000082225
WB SEPN1 faint
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104284
0000082233
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104292
0000082234
weak neck muscles infancy; WB SEPN1 faint; ambulant at 45y
-
-
Isolated (sporadic)
-
-
32y
difficulty climbing stairs, excercise intolerance
-
Johan den Dunnen
00104293
0000082235
IHC SEPN1 normal; WB SEPN1 reduced size; CPK 130
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00104294
0000082236
neonatal hypotonia, poor head control, facial weakness; WB SEPN1 faint; w20m
-
-
Isolated (sporadic)
-
18y
-
-
-
Johan den Dunnen
00104295
0000082237
neonatal hypotonia, respiratory failure at birth, facial weakness; WB SEPN1 faint; w13m
-
-
Isolated (sporadic)
-
29y
-
-
-
Johan den Dunnen
00104296
0000082238
neonatal hypotonia, respiratory failure at birth, facial weakness; w15m
-
-
Isolated (sporadic)
-
30y
-
-
-
Johan den Dunnen
00104297
0000082239
delayed motor milestones, feeding difficulties, facial weakness; w18m
-
-
Isolated (sporadic)
-
26y
-
-
-
Johan den Dunnen
00104298
0000082240
lack of head control, delayed motor milestones, facial weakness; w18m
-
-
Isolated (sporadic)
-
15y
-
-
-
Johan den Dunnen
00104299
0000082242
neonatal hypotonia, weak suckling, lack of head control, facial weakness mild; WB SEPN1 faint; w15m
-
-
Isolated (sporadic)
-
17y
-
-
-
Johan den Dunnen
00104301
0000082244
poor head control, delayed motor milestones, failure to thrive, no facial weakness; w15m
-
-
Isolated (sporadic)
-
30y
-
-
-
Johan den Dunnen
00104303
0000082250
neonatal hypotonia, poor head control, facial weakness; w14m
-
-
Isolated (sporadic)
-
23y
-
-
-
Johan den Dunnen
00104309
0000082251
neonatal hypotonia, poor head control, facial weakness; w14m
-
-
Isolated (sporadic)
-
19y
-
-
-
Johan den Dunnen
00104310
0000082254
delayed motor milestones, thorax deformity, facial weakness; w36m
-
-
Isolated (sporadic)
-
41y
-
-
-
Johan den Dunnen
00104313
0000082255
-
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00104314
0000082259
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104327
0000082260
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104328
0000082265
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104333
0000082266
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104334
0000082268
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104336
0000082269
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104337
0000082270
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104338
0000082271
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104339
0000082272
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104340
0000082273
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104341
0000082274
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104342
0000082275
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104343
0000082276
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104344
0000082277
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104345
0000082279
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104347
0000082280
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104348
0000082281
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104349
0000082282
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104350
0000082283
severe, scoliosis 1y, died of restrictive respiratory failure; w14m
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104351
0000082284
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104352
0000082285
SelN level <5%
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104353
0000082286
SelN level 20%
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104354
0000082287
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00104355
0000082288
6y-scoliosis, rigid spine syndrome; 8y-proximal muscle weakness, no progression, walk >4000m
-
-
Isolated (sporadic)
-
-
2m
lack of head control
-
Johan den Dunnen
00104356
0000082289
myopathic with cores; 11y-scoliosis, rigid spine syndrome; 18y-proximal muscle weakness, no progression, walk 30004000m; 13y-ventilation 2-4h/night; FVC 13y 18%
-
-
Isolated (sporadic)
-
-
1y
hypotonia, lack of head control
-
Johan den Dunnen
00104357
0000082290
12y-scoliosis, rigid spine syndrome; 14y-proximal muscle weakness, no progression, walk 1000m; not ventilated; FVC 13y 44%
-
-
Isolated (sporadic)
-
-
1y
hypotonia
-
Johan den Dunnen
00104358
0000082291
myopathic with cores; 15y-scoliosis, rigid spine syndrome; 17y-proximal muscle weakness, slow progression, walk 10002000m; not ventilated; FVC 16.6y 46%
-
-
Isolated (sporadic)
-
-
1y
hypotonia
-
Johan den Dunnen
00104359
0000082292
myopathic with multiminicores; 7y-scoliosis, 8y-rigid spine syndrome; 13y-proximal muscle weakness, no progression, walk 10002000m; not ventilated; FVC 11y 65%
-
-
Isolated (sporadic)
-
-
1y
hypotonia, motor devevelopment delay
-
Johan den Dunnen
00104360
0000082293
myopathic with unspecific changes; 4.5y-rigid spine syndrome; 7y-proximal muscle weakness, no progression, walk 10002000m; not ventilated; FVC 7y 39%
-
-
Isolated (sporadic)
-
-
4y
lack of head control, motor devevelopment delay
-
Johan den Dunnen
00104361
0000082294
myopathic with multiminicores; 12y-scoliosis, rigid spine syndrome; 13y-proximal muscle weakness, slow progression, walk 1000m; 10y-ventilation 8-9h/night; FVC 9y 50.5%
-
-
Isolated (sporadic)
-
-
4y
lack of head control, motor devevelopment delay
-
Johan den Dunnen
00104362
0000082295
myopathic with unspecific changes; 18y-scoliosis, rigid spine syndrome; 21y-proximal muscle weakness, slow progression, walk>4000m; not ventilated; FVC 13y 62%
-
-
Isolated (sporadic)
-
-
1y
hypotonia, lack of head control
-
Johan den Dunnen
00104363
0000082296
5y-proximal muscle weakness, no progression, walk>4000m
-
-
Unknown
-
-
1y
hypotonia, lack of head control
-
Johan den Dunnen
00104364
0000082297
unspecific with fiber size variability; 13y-scoliosis; 15y-proximal muscle weakness, slow progression, walk 5001000m; 10y-intermittend ventilation night; FVC 10y 33%
-
-
Unknown
-
-
2y
motor devevelopment delay
-
Johan den Dunnen
00104365
0000082298
congenital fibertype dysproportion; 11y-scoliosis, rigid spine syndrome, contractures in both elbows; 20y-proximal muscle weakness, no progression, walk>4000m; 12y-intermittend ventilation night; FVC 12y 56%
-
-
Unknown
-
-
1y
hypotonia, motor devevelopment delay, impaired increase inweight
-
Johan den Dunnen
00104366
0000082299
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104367
0000082300
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104368
0000082302
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104370
0000082303
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104371
0000082307
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104375
0000082308
not able to raise head from early childhood on, facies myopathica; w15m; CPK 300 U/l
-
-
Familial, autosomal recessive
-
-
-
-
-
Wolfram Kress
00104376
0000082309
5m
-
-
Isolated (sporadic)
-
-
00y04m
-
-
Varvara Kadnikova
00104377
0000082310
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104378
0000082311
rigid spine syndrome; congenital muscle weakness, proximal, body size and weight below 3rd percentile, 2.5y when diagnosed
-
-
Familial, autosomal recessive
-
-
2y6m
-
-
Wolfram Kress
00104379
0000082343
rigid spine syndrome; 6y-rigid spine, poor head control; CPK normal
-
-
Familial, autosomal recessive
-
-
-
-
-
Wolfram Kress
00104411
0000082344
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104412
0000082345
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104413
0000082346
mildly atrophic fibers.
-
-
Unknown
-
-
-
-
-
Tom Winder
00104414
0000082347
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104415
0000082348
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104416
0000082349
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104417
0000082350
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104418
0000082351
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104419
0000082352
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104420
0000082353
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104421
0000082354
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104422
0000082355
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104423
0000082356
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104424
0000082357
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104425
0000082358
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104426
0000082359
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104427
0000082360
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104428
0000082361
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104429
0000082362
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104430
0000082363
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104431
0000082364
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104432
0000082365
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104433
0000082366
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104434
0000082367
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104435
0000082368
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104436
0000082369
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104437
0000082370
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104438
0000082371
mulitminicore disease/RSMD-1?
-
-
Unknown
-
-
-
-
-
Shu Yau
00104439
0000082372
mulitminicore disease (MmD); dystrophy, muscular, rigid spine (RSMD-1)
-
-
Unknown
-
-
-
-
-
Shu Yau
00104440
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