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Phenotypes for disease #04164 (NEM (myopathy, nemaline (NEM)))
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
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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Date
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Date
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all entries before the year 2020
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all entries on or after June 15th, 2020
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Date
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all entries in 2019 or 2020, and before March, 2020
Numeric
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all entries exactly matching 23 or 24
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Numeric
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all entries not exactly matching 23
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all entries lower than 23
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all entries lower than, or equal to, 23
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all entries with values from 20 to 29, but not equal to 23
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all entries containing 'South Asian', but not containing 'South East Asian'
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470 entries on 5 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
0000023225
severe congenital nemaline myopathy; deceased (neonatal period, <28d); polyhydramnios, preterm delivery (30/40), arthrogryposis, fractures (bilateral femoral)
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027154
0000023226
severe congenital nemaline myopathy; deceased (neonatal period, <28d); preterm delivery (36/40)
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027155
0000023227
severe congenital nemaline myopathy; decreased fetal movements, breech presentation, arthrogryposis, ophthalmoplegia
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027156
0000023228
severe congenital nemaline myopathy; deceased (neonatal period); polyhydramnios, decreased fetal movements, contractures
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027157
0000023229
severe congenital nemaline myopathy; alive 4m, lost to follow-up
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027158
0000023230
severe congenital nemaline myopathy; alive 2m, lost to follow-up; polyhydramnios, decreased fetal movements, subdural hematoma
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027159
0000023231
severe congenital nemaline myopathy; alive 10m, lost to follow-up; polyhydramnios, decreased fetal movements, fetal edema, preterm delivery (32/40), microcephaly, contractures
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027160
0000023232
severe congenital nemaline myopathy; alive 1y7m, lost to follow-up; polyhydramnios, decreased fetal movements, ophthalmoplegia
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027161
0000023233
severe congenital nemaline myopathy; deceased (neonatal period)
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027162
0000023234
severe congenital nemaline myopathy; deceased (4m); polyhydramnios, decreased fetal movements, preterm delivery (34/40), ophthalmoplegia, contractures
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027163
0000023235
severe congenital nemaline myopathy; deceased (6w); polyhydramnios, decreased fetal movements, preterm delivery (35/40), breech presentation, ophthalmoplegia, arthrogryposis, fractures (bilateral humeral)
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027164
0000023236
severe congenital nemaline myopathy; deceased (5m); polyhydramnios, arthrogryposis
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027165
0000023237
severe congenital nemaline myopathy; deceased (2m); absent fetal movements, preterm delivery (31/40), breech presentation, arthrogryposis
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027166
0000023238
severe congenital nemaline myopathy; deceased (3m); polyhydramnios, breech presentation, ophthalmoplegia, kyphosis
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027167
0000023239
typical congenital nemaline myopathy; alive (10y); polyhydramnios, decreased fetal movements, bulbar weakness, ophthalmoplegia, percutaneous endoscopic gastrostomy, nocturnal noninvasive ventilation.; walks independently; normal cardiac assessment and echocardiogram
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027168
0000023240
severe congenital nemaline myopathy; alive 1m, lost to follow-up; polyhydramnios, contractures
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027169
0000023241
severe congenital nemaline myopathy; deceased (neonatal period)
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027170
0000023242
severe congenital nemaline myopathy; deceased (neonatal period); preterm delivery (33/40), arthrogryposis
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027171
0000023243
severe congenital nemaline myopathy; affected fetus
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027172
0000023244
severe congenital nemaline myopathy; deceased (neonatal period);polyhydramnios, decreased fetal movements.
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027173
0000023245
typical congenital nemaline myopathy; alive (4y); polyhydramnios, bulbar weakness, percutaneous endoscopic gastrostomy, nocturnal noninvasive ventilation; walks with truncal support; nNormal cardiac assessment and echocardiogram
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00027174
0000059393
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079680
0000059394
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079681
0000059395
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079682
0000059396
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079683
0000059397
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079684
0000059398
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079685
0000059399
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079686
0000059400
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079687
0000059401
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079688
0000059402
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079689
0000059403
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079690
0000059404
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079691
0000059405
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079692
0000059406
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079693
0000059407
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079694
0000059408
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079695
0000059409
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079696
0000059410
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079697
0000059411
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079698
0000059412
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079699
0000059413
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079700
0000059414
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079701
0000059415
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079702
0000059416
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079703
0000059417
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079704
0000059418
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079705
0000059419
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079706
0000059420
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079707
0000059421
Familial, autosomal recessive
-
-
Familial, autosomal recessive
-
-
-
-
-
Gianina Ravenscroft
00079708
0000084023
NEM requested
-
-
Isolated (sporadic)
-
-
-
-
-
Kristen Nowak
00106208
0000084137
mild; intranuclear rods; Disease severity and fact biopsy has intranuclear rods in email from Isabelle Penisson-Besnier to Nigel Laing 6.9.04
-
-
Familial, autosomal dominant
-
-
-
-
-
Kristen Nowak
00106322
0000084162
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106358
0000084163
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106359
0000084164
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106360
0000084165
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106361
0000084166
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106362
0000084167
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106363
0000084168
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106364
0000084169
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106365
0000084170
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106366
0000084171
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106367
0000084172
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106368
0000084173
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106369
0000084174
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106370
0000084175
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106371
0000084176
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106372
0000084177
NEM requested
-
-
Familial, autosomal recessive
-
-
-
-
-
Kristen Nowak
00106373
0000084178
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106374
0000084179
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106375
0000084180
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106376
0000084181
NEM requested
-
-
Familial, autosomal recessive
-
-
-
-
-
Kristen Nowak
00106377
0000084182
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106378
0000084183
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106379
0000084184
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106380
0000084185
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106381
0000084186
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106382
0000084187
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106383
0000084188
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106384
0000084189
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106385
0000084190
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106386
0000084191
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106387
0000084192
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106388
0000084193
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106389
0000084194
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106390
0000084195
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106391
0000084196
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106392
0000084197
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106393
0000084198
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106394
0000084199
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106395
0000084200
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106396
0000084201
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106397
0000084202
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106398
0000084203
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106399
0000084204
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106400
0000084205
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106401
0000084206
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106402
0000084207
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106403
0000084218
actin intranuclear rod and nemaline myopathy; diagnosed as actin myopathy, based on the cytoplasmic accumulation of thin filament aggregates. Intranuclear rods and dispersed, tiny nemaline bodies were also seen. n addition, the patient also showed supraorbital cranial sclerosis and striation of the iliac bone, which were reminiscent of the findings in osteopathia striata-cranial sclerosis. Hepatomegaly, undescended testis, right ureter-bladder constriction, and dilatation of lateral ventricles with signal change in the periventicular white matter were also noted. These may contribute to broaden the spectrum of actinopathy phenotypes.
-
-
Isolated (sporadic)
1y9m
-
1y9m
birth-marked hypotonia, respiratory insufficiency
-
Kristen Nowak
00106414
0000084219
NEM requested
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106415
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