Global Variome shared LOVD
LOVD v.3.0 Build 30b [
Current LOVD status
]
Register as submitter
|
Log in
View all genes
Create a new gene entry
View all transcripts
Create a new transcript information entry
View all variants
View all variants affecting transcripts
Create a new data submission
View active genomic custom columns
Enable more genomic custom columns
View all individuals
Create a new data submission
View active custom columns
Enable more custom columns
View all diseases
Create a new disease information entry
View available phenotype columns
View all screenings
Create a new data submission
View active custom columns
Enable more custom columns
Submit new data
Phenotypes for disease #00777 (PVNH1 (heterotopia, nodular, periventricular, type 1), OMIM:300049)
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
All list views have search fields which can be used to search data. You can search for a complete word or you can search for a part of a search term. If you enclose two or more words in double quotes, LOVD will search for the combination of those words only exactly in the order you specify. Note that search terms are case-insensitive and that wildcards such as * are treated as normal text! For all options, like "and", "or", and "not" searches, or searching for prefixes or suffixes, see the table below.
Operator
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'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
54 entries on 1 page. Showing entries 1 - 54.
10 per page
25 per page
50 per page
100 per page
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
0000024727
death 3d, severe systemic bleeding and organ failure, severe arrest myeloid/erythroid differentiation in bone marrow, lymphoid depletion in thymus
-
-
Familial
-
-
-
-
-
Yu Sun
00028669
0000024728
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028671
0000024729
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028672
0000024730
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028673
0000024731
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028674
0000024732
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028675
0000024733
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028677
0000024734
severe, non-lethal; shortened digits, syndactyly, clinodactyly; immune compromise, recurrent infections; small corpus callosum, hypoplastic cerebellum
-
-
Familial
-
-
-
-
-
Yu Sun
00028681
0000024736
Developmental Regression, West Syndrome
-
-
Familial
-
-
-
-
-
Yu Sun
00028684
0000024737
Developmental Regression, West Syndrome
-
-
Familial
-
-
-
-
-
Yu Sun
00028685
0000024738
Developmental Regression, West Syndrome
-
-
Familial
-
-
-
-
-
Yu Sun
00028686
0000024739
BPNH, scattered nodules along lateral ventricle wall, slightly vertically oriented posterior hippocampal region, mega cisterna magna, complex partial seizure onset 20y,rare, remission; EEG mild R sided slowing, verbal IQ74/performance IQ97/full-scale IQ81
-
-
Familial
-
-
-
-
-
Yu Sun
00028691
0000024740
BPNH, scattered nodules along lateral ventricle wall, slightly vertically oriented posterior hippocampal region, mega cisterna magna, complex partial seizure onset at 20yrs,rare,remission; EEG shows mild R sided slowing, verbal IQ/performance IQ/full-scale IQ=74/97/81
-
-
Familial
-
-
-
-
-
Yu Sun
00028692
0000024741
NH, scattered nodules along lateral ventricle wall, slightly vertically oriented posterior hippocampal region, mega cisterna magna, complex partial/secondarily generalized seizure onset at 14yrs,once in 4-5 mth,no remission; EEG shows L frontotemporal slow wave, use antiepileptic drugs:carbamazepine,topiramate;verbal IQ/performance IQ/full-scale IQ=70/81/74
-
-
Familial
-
-
-
-
-
Yu Sun
00028693
0000024755
BPNH, mega cisterna magna, complex partial/secondarily generalized seizure onset at 30yrs,rare,remission;use antiepileptic drugs:phenobarbital;verbal IQ/performance IQ/full-scale IQ=71/72/70
-
-
Familial
-
-
-
-
-
Yu Sun
00028725
0000024756
BPNH, mega cisterna magna, simple partial/partial secondarily generalized seizure onset at 14yrs,rare,remission;use antiepileptic drugs:phenobarbital;verbal IQ/performance IQ/full-scale IQ=100/69/83
-
-
Familial
-
-
-
-
-
Yu Sun
00028726
0000024776
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028754
0000024777
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028755
0000024778
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028756
0000024779
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028757
0000024851
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028835
0000024852
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028836
0000024853
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028839
0000024854
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028842
0000024855
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028843
0000024856
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028848
0000024857
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028849
0000024858
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028850
0000024859
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028851
0000024868
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028867
0000024869
-
-
-
Familial
-
-
-
-
-
Yu Sun
00028868
0000024877
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028679
0000024878
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028680
0000024882
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028722
0000024883
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028723
0000024888
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028837
0000024889
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028838
0000024890
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028840
0000024891
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028841
0000024892
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028844
0000024893
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028845
0000024894
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028846
0000024895
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028847
0000024896
indistinguishable from females have FLN1 mutation. Symmetrical nodules, enlarged cisterna magna behind cerebellum, mild to moderate seizure, no MR
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028854
0000024897
milder in MRI than females with FLN1 mutations, few heterotopic nodules only in right hemisphere. No MR. Not intractable seizure
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028855
0000024898
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028864
0000024906
periventricular heterotopia, type 1/frontometaphyseal dysplasia; delayed closure anterior fontanel, craniofacial anomalies, marked genua valga necessitating surgical corrections; 5y-MRI cranial bilateral PVNHs; no seizures, normal motor/mental development; 8y-radiological FMD
-
-
Unknown
-
-
-
-
-
Yu Sun
00028666
0000024909
-
-
-
Unknown
-
-
-
-
-
Yu Sun
00028670
0000024910
-
-
-
Unknown
-
-
-
-
-
Yu Sun
00028676
0000024911
-
-
-
Unknown
-
-
-
-
-
Yu Sun
00028678
0000024916
epilepsy
-
-
Unknown
-
-
-
-
-
Yu Sun
00028690
0000024929
BPNH, mega cisterna magna, complex partial/secondarily generalized seizure onset at 9yrs,rare,no remission;use antiepileptic drugs:phenobarbital;verbal IQ/performance IQ/full-scale IQ=66/85/74
-
-
Unknown
-
-
-
-
-
Yu Sun
00028724
0000034212
see paper; classical X-linked periventricular nodular heterotopia , ...
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00046330
0000242926
(+) Abnormality of the nervous system,(+) Aortic regurgitation,(+) EEG abnormality,(+) Abnormality of the cerebral cortex,(+) Cortical tubers,(+) Cerebral hamartoma,(+) Hamartoma. Positive famliy history regarding sympotoms mentioned.
-
-
Familial, X-linked dominant
28y
-
-
-
-
Andreas Laner
00324385
10 per page
25 per page
50 per page
100 per page
Legend
How to query
Screenscraping/webscraping (downloading large amounts of data using scripts) is strictly prohibited.
Use our
APIs
to retrieve data.
Powered by
LOVD v.3.0
Build 30b
LOVD software ©2004-2024
Leiden University Medical Center
Database contents © by their respective submitters and curators