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Phenotypes for disease #01941 (biotinidase deficiency, OMIM:253260)
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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93 entries on 1 page. Showing entries 1 - 93.
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Legend
How to query
Phenotype ID
Phenotype details
Diagnosis/Initial
Diagnosis/Definite
Inheritance
Age/Examination
Age/Diagnosis
Age/Onset
Phenotype/Onset
Protein
Owner
Individual ID
0000083851
enzyme activity: 43%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105944
0000083852
enzyme activity: 54%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105945
0000083853
enzyme activity: 80%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105946
0000083854
enzyme activity: 109%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105947
0000083855
enzyme activity: 84%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105948
0000083856
enzyme activity: 106%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105949
0000083857
enzyme activity: 56%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105950
0000083858
enzyme activity: 52%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105951
0000083859
enzyme activity: 44%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105952
0000083860
enzyme activity: 54%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105953
0000083861
enzyme activity: 61%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105954
0000083862
enzyme activity: 58%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105955
0000083863
enzyme activity: 87%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105956
0000083864
enzyme activity: 56%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105957
0000083865
enzyme activity: 17%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105958
0000083866
enzyme activity: 26%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105959
0000083867
enzyme activity: 29%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105960
0000083868
biotinidase deficiency, partial
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105984
0000083869
biotinidase deficiency, partial
-
-
Unknown
-
-
-
-
-
Jasper Saris
00105986
0000083870
biotinidase deficiency, partial
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106001
0000083871
biotinidase deficiency, partial
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106008
0000083872
biotinidase deficiency, partial
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106024
0000083873
biotinidase deficiency, partial
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106035
0000083874
multiple carboxylase deficiency
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106036
0000083875
biotinidase deficiency, partial
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106041
0000083876
multiple carboxylase deficiency
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106050
0000083877
biotinidase deficiency, partial
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106053
0000083878
multiple carboxylase deficiency
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106060
0000083879
enzyme activity: 14%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106075
0000083880
enzyme activity: 44%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106076
0000083881
enzyme activity: 28%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106077
0000083882
enzyme activity: 32%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106078
0000083883
enzyme activity: 31%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106079
0000083884
enzyme activity: 18%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106080
0000083885
enzyme activity: 17%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106081
0000083886
enzyme activity: 27%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106082
0000083887
enzyme activity: 8%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106083
0000083888
enzyme activity: 34%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106084
0000083889
enzyme activity: 28%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106085
0000083890
enzyme activity: 24%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106086
0000083891
enzyme activity: 30%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106087
0000083892
enzyme activity: 20%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106088
0000083894
enzyme activity: 23%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106090
0000083895
enzyme activity: 33%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106091
0000083896
enzyme activity: 5%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106092
0000083897
enzyme activity: 26%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106093
0000083898
enzyme activity: 30%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106094
0000083899
enzyme activity: 19%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106095
0000083900
enzyme activity: 20%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106096
0000083901
enzyme activity: 16%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106097
0000083902
enzyme activity: 27%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106098
0000083903
enzyme activity: 30%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106099
0000083904
enzyme activity: 23%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106100
0000083905
enzyme activity: 23%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106101
0000083906
enzyme activity: 23%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106102
0000083907
enzyme activity: 15%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106103
0000083908
enzyme activity: 16%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106104
0000083909
enzyme activity: 30%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106105
0000083910
enzyme activity: 30%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106106
0000083911
enzyme activity: 18%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106107
0000083912
enzyme activity: 24%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106108
0000083913
enzyme activity: 21%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106109
0000083914
enzyme activity: 31%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106110
0000083915
enzyme activity: 23%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106111
0000083916
enzyme activity: 21%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106112
0000083917
enzyme activity: 22%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106113
0000083918
enzyme activity: 34%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106114
0000083919
enzyme activity: 10%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106115
0000083920
enzyme activity: 26%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106116
0000083921
enzyme activity: 30%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106117
0000083922
enzyme activity: 28%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106118
0000083923
enzyme activity: 39%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106119
0000083924
enzyme activity: 27%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106120
0000083925
enzyme activity: 29%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106121
0000083926
enzyme activity: 28%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106122
0000083927
enzyme activity: 26%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106123
0000083928
enzyme activity: 29%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106124
0000083929
enzyme activity: 28%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106125
0000083930
enzyme activity: 34%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106126
0000083931
enzyme activity: 28%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106127
0000083932
enzyme activity: 0%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106128
0000083933
enzyme activity: 4%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106129
0000083934
enzyme activity: 5%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106130
0000083935
enzyme activity: 6%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106131
0000083936
enzyme activity: 5%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106132
0000083937
enzyme activity: 8%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106133
0000083938
enzyme activity: 14%
-
-
Unknown
-
-
-
-
-
Jasper Saris
00106134
0000339530
-
Biotinidase deficiency
Biotinidase deficiency
Familial, autosomal recessive
00y00m03d
00y00m27d
03y06m
-
-
Miriam Erandi Reyna-Fabián
00450470
0000340287
Attention deficit hyperactivity disorder
Biotinidase deficiency
Biotinidase deficiency
Familial, autosomal recessive
-
00y00m01d
-
-
-
Miriam Erandi Reyna-Fabián
00451616
0000340288
Bicoronal synostosis, congenital pyloric atresia
Biotinidase deficiency
Biotinidase deficiency
Familial, autosomal recessive
-
00y00m01d
-
-
-
Miriam Erandi Reyna-Fabián
00451617
0000340312
Mild intellectual disability, Poor visual, Nerve optic atrophy, Pectus excavatum.
Biotinidase deficiency
Biotinidase deficiency
Familial, autosomal recessive
-
05y
-
-
-
Miriam Erandi Reyna-Fabián
00451651
0000340317
Epilepsy, Dolichocephaly, Prominent glabella, Broad nasal bridge, Cupped ears, Antihelix, stem, underdeveloped, Scoliosis
Biotinidase deficiency
Biotinidase deficiency
Familial, autosomal recessive
-
00y11m
-
-
-
Miriam Erandi Reyna-Fabián
00451656
0000351613
partial biotinidase deficiency
partial biotinidase deficiency
BTD
Familial, autosomal recessive
06y
-
00y02m
-
-
Maria-Pilar Lopez-Garrido
00466075
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