Global Variome shared LOVD
HPS4 (Hermansky-Pudlak syndrome 4)
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Curator:
William (Bill) Oetting
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Phenotypes for disease #01410 (HKPX (hyperekplexia, hereditary (HKPX)), OMIM:149400)
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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Text
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all entries beginning with 'p.(Arg'
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Date
2020
all entries matching the year 2020
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Date
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all entries matching March or April, 2020
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Date
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Date
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Date
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Date
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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
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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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Numeric
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all entries lower than 23
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Numeric
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all entries lower than, or equal to, 23
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Numeric
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Numeric
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Numeric
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all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
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all entries containing 'South Asian', but not containing 'South East Asian'
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37 entries on 1 page. Showing entries 1 - 37.
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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
0000087766
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112202
0000087767
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112203
0000087768
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112204
0000087769
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112205
0000087770
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112206
0000087771
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112207
0000087772
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112208
0000087773
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112209
0000087774
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112210
0000087775
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112211
0000087776
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112212
0000087777
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112213
0000087778
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112214
0000087779
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112215
0000087780
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112216
0000087781
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112217
0000087782
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112218
0000087783
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112219
0000087784
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112220
0000087785
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112221
0000087786
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112222
0000087787
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112223
0000087788
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112224
0000087789
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112225
0000087790
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112226
0000087791
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112227
0000087792
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112228
0000087793
Symptoms of stiff baby syndrome in the index patient (30-3, age=5 weeks) were diagnosed clinically shortly after birth, After birth, patient 30-3 suffered from severe muscular hypertonia and hyperreflexia.
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112229
0000087794
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112230
0000087795
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112231
0000087796
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112232
0000087797
male infant with generalized stiffness that was observed from the first 3 days of life. He showed marked improvement in the startle response and muscle hypertonia after being administered phenobarbital and clonazepam.
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112233
0000087798
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112234
0000087799
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00112235
0000128941
-
-
-
Unknown
-
-
-
-
-
Ivan Milenkovic
00163755
0000231243
hyperekplexia syndrome
-
-
Unknown
26y
-
-
-
-
Andreas Laner
00305390
0000242045
Abnormality of the nervous system, Motor seizure, Hyperexplexia, father also affected
-
-
Unknown
30y
-
-
-
-
Andreas Laner
00320001
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