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Phenotypes for disease #01848 (CPSID (carbamoylphosphate synthetase I deficiency), OMIM:237300)
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
How to query this table
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Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
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94 entries on 1 page. Showing entries 1 - 94.
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How to query
Phenotype ID
Phenotype details
Diagnosis/Initial
Diagnosis/Definite
Inheritance
Age/Examination
Age/Diagnosis
Age/Onset
Phenotype/Onset
Protein
Owner
Individual ID
0000087214
onset 3d, death 1y
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111166
0000087215
onset 1d, death 4d
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111164
0000087216
no mental retardation
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110917
0000087217
onset<1w, death <1w
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110918
0000087218
onset 13y, alive 18y; no mental reatardation
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111165
0000087219
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110920
0000087220
onset 1d, death 20d
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111167
0000087221
neonatal, died at 11d
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00110923
0000087222
survival 3d
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111156
0000087223
late onset (27y)
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110926
0000087224
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110928
0000087225
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110929
0000087226
late onset (15y)
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110935
0000087227
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110938
0000087228
onset 2d, survives with liver transplantation, good mental status
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111162
0000087229
neonatal onset
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110942
0000087230
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110945
0000087231
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110948
0000087232
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110949
0000087233
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110950
0000087234
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110951
0000087235
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110952
0000087236
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111161
0000087237
onset 3d, alive; severe mental retardation
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111168
0000087238
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110966
0000087239
onset 2d, death 14d
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111169
0000087240
late onset
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110973
0000087241
late onset (1m)
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110974
0000087242
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110976
0000087243
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110978
0000087244
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110979
0000087245
late onset (1y)
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110980
0000087246
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110983
0000087247
onset 2d, death 24d
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110984
0000087248
onset 2d, death 36d
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111163
0000087249
late onset
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110987
0000087250
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110989
0000087251
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110990
0000087252
neonatal, survived
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110991
0000087253
onset 1d, survived
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00110994
0000087254
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111000
0000087255
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111010
0000087256
onset 0d, death 13d
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111155
0000087257
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111011
0000087258
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111017
0000087259
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111019
0000087260
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111027
0000087261
late onset
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111028
0000087262
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111033
0000087263
onset 1y, alive 18y
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111040
0000087264
patient died as neonate
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111044
0000087265
onset 2d patient, onset 45y in grandfather of patient
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111045
0000087266
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111048
0000087267
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111050
0000087268
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111060
0000087269
late onset
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111066
0000087270
onset 1d, death 12d
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111170
0000087271
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111069
0000087272
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111079
0000087273
late onset
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111086
0000087274
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111089
0000087275
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111095
0000087276
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111096
0000087277
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111097
0000087278
neonatal presentation with exitus
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111100
0000087279
late onset (31y)
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111159
0000087280
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111105
0000087281
late onset
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111107
0000087282
onset 9d (lethargy, grunting, severe hyperammonemia), severely retarded 27m
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111109
0000087283
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111114
0000087284
late onset
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111117
0000087285
onset 2d, death 4d
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00111118
0000087286
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111119
0000087287
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111120
0000087288
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111160
0000087289
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111126
0000087290
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111127
0000087291
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111128
0000087292
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111132
0000087293
onset 3d, death 28d
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111135
0000087294
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111139
0000087295
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111140
0000087296
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111143
0000087297
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111148
0000087298
late onset
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111150
0000087299
neonatal
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00111151
0000187294
see paper; …
CPS1 deficiency
-
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00248285
0000187295
see paper; …
CPS1 deficiency
-
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00248286
0000187296
see paper; …
CPS1 deficiency
-
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00248287
0000187297
see paper; …
CPS1 deficiency
-
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00248288
0000232050
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Sha Hong
00306202
0000312024
-
-
-
Familial
-
-
-
-
-
Gemeinschaftspraxis für Humangenetik Dresden
00151993
0000325777
fever, poor feeding, poor response, hyperammonemia
-
carbamoylphosphate synthetase I deficiency
Familial, autosomal recessive
00y00m03d
-
00y00m03d
fever, poor feeding and poor response
-
Faye Li
00435592
0000325778
hypothermia, drowsiness
-
-
Familial, autosomal recessive
00y00m02d
-
00y00m02d
hypothermia, drowsiness
-
Faye Li
00435593
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