Global Variome shared LOVD
RYR1 (ryanodine receptor 1 (skeletal))
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Curators:
Jorge Oliveira
and
Johan den Dunnen
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Phenotypes for disease #00388 (KBGS (KBG syndrome (KBGS)), OMIM:148050)
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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all entries not matching March, 2020
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Date
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all entries before the year 2020
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Date
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Date
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all entries after June, 2020
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Date
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all entries on or after June 15th, 2020
combination
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
|
Numeric
23|24
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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all entries higher than 23
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Numeric
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all entries higher than, or equal to, 23
combination
Numeric
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all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
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Matches
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all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
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all entries containing 'Asian' but not containing 'Caucasian'
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all entries containing 'South Asian', but not containing 'South East Asian'
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29 entries on 1 page. Showing entries 1 - 29.
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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
0000017727
-
-
-
Unknown
-
-
-
-
-
Helger Yntema
00019951
0000017728
-
-
-
Unknown
-
-
-
-
-
Helger Yntema
00019952
0000017729
-
-
-
Isolated (sporadic)
8y
-
-
-
-
Helger Yntema
00019953
0000017730
-
-
-
Isolated (sporadic)
25y
-
-
-
-
Helger Yntema
00019954
0000017731
-
-
-
Isolated (sporadic)
6y
-
-
-
-
Helger Yntema
00019955
0000017732
-
-
-
Isolated (sporadic)
38y
-
-
-
-
Helger Yntema
00019956
0000017733
-
-
-
Familial
-
-
-
-
-
Helger Yntema
00019957
0000017734
-
-
-
Isolated (sporadic)
11y
-
-
-
-
Helger Yntema
00019958
0000017735
-
-
-
Isolated (sporadic)
10y
-
-
-
-
Helger Yntema
00019959
0000017736
-
-
-
Isolated (sporadic)
11y
-
-
-
-
Helger Yntema
00019960
0000017737
-
-
-
Isolated (sporadic)
19y
-
-
-
-
Helger Yntema
00019961
0000017738
-
-
-
Familial
-
-
-
-
-
Helger Yntema
00019962
0000060452
KBG syndrome (OMIM:148050)
-
-
Isolated (sporadic)
-
-
-
-
-
Daniel Trujillano
00080883
0000087477
Feeding difficulties, short stature, microcephaly, moderate to severe ID, facial freckling
-
-
Isolated (sporadic)
-
-
-
-
-
Bernt Popp
00111392
0000207384
macrodontia; low anterior and posterior hairlines, brachycephaly, triangular face, synophrys, long palpebral fissures, hypertelorism, ptosis, prominent nasal bridge, anteverted nostrils, long philtrum, large and prominent ears; short hands with clinodactyly of the 5th fingers and ulnar deviation of the 2nd fingers; short stature; seizures, mild-moderate intellectual disability; delayed bone age; accessory cervical ribs; cryptorchidism
KBG syndrome
-
Familial, autosomal dominant
-
-
-
-
-
Johan den Dunnen
00269556
0000207385
macrodontia; low anterior and posterior hairlines, brachycephaly, triangular face, synophrys, long palpebral fissures, hypertelorism, ptosis, prominent nasal bridge, anteverted nostrils, long philtrum, large and prominent ears; short hands with clinodactyly of the 5th fingers and ulnar deviation of the 2nd fingers; short stature; seizures, mild-moderate intellectual disability, attention deficit hyperactivity disorder; delayed bone age; closed spina bifida; cryptorchidism, mild sensorineural hearing loss
KBG syndrome
-
Familial, autosomal dominant
-
-
-
-
-
Johan den Dunnen
00269557
0000207386
macrodontia; low anterior and posterior hairlines, brachycephaly, triangular face, synophrys, long palpebral fissures, hypertelorism, ptosis, prominent nasal bridge, anteverted nostrils, long philtrum, large and prominent ears; short hands with clinodactyly of the 5th fingers and ulnar deviation of the 2nd fingers; short stature; moderate intellectual disability; thoracic kyphosis, closed spina bifida; cryptorchidism, epispadias
KBG syndrome
-
Familial, autosomal dominant
-
-
-
-
-
Johan den Dunnen
00269558
0000207387
macrodontia; triangular face with pointed chin, low anterior hairline, long palpebral fissures, ptosis, anteverted nostrils, long philtrum, prominent ears; short hands with clinodactyly of the 5th fingers and ulnar deviation of the 2nd fingers, cutaneous syndactyly of fingers and toes; short stature; mild intellectual disability; delayed bone age; accessory cervical ribs; cryptorchidism
KBG syndrome
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00269559
0000207388
macrodontia; prominent forehead, brachycephaly, triangular face, synophrys, long palpebral fissures, hypertelorism, anteverted nostrils, posteriorly rotated ears, long philtrum, short and webbed neck; short hands with clinodactyly of the 5th fingers; no short stature; history of developmental delay, moderate intellectual disability; no costovertebral anomalies; cryptorchidism
KBG syndrome
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00269560
0000207389
macrodontia; low anterior and posterior hairlines, triangular face, ptosis, hypertelorism, prominent nasal bridge, anteverted nostrils, long philtrum, large and prominent ears, short and webbed neck; short hands with clinodactyly of the 5th fingers, cutaneous syndactyly of fingers and toes; short stature; moderate intellectual disability, hyperactivity, anxiety, poor concentration; accessory cervical ribs
KBG syndrome
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00269561
0000207390
low anterior and posterior hairlines, triangular face with pointed chin, synophrys, long and downslanting palpebral fissures, ptosis, hypertelorism, prominent nasal bridge, anteverted nostrils, long philtrum, tented upper lip, prominent ears; short hands with clinodactyly of the 5th fingers and ulnar deviation of the 2nd fingers; short stature; moderate intellectual disability; delayed bone age; no costovertebral anomalies; cryptorchidism
KBG syndrome
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00269562
0000272723
-
-
-
Familial
-
-
-
-
-
Ana Latorre
00377570
0000276610
Intellectual disability, Global developmental delay, Absent speech, Seizure, Growth delay, Edema of the dorsum of hands, Edema of the dorsum of feet, Behavioral abnormality
-
-
Isolated (sporadic)
18y
-
-
-
-
Andreas Laner
00382753
0000294760
Delayed speech and language development, Poor speech, Neurological speech impairment, Abnormality of higher mental function, Abnormal nervous system physiology, Abnormality of the face
-
-
Isolated (sporadic)
06y
-
-
-
-
Andreas Laner
00401997
0000306094
Hypospadias, Hypertelorism, Hearing impairment, Long palpebral fissure, Single transverse palmar crease, Motor delay, Neurodevelopmental delay
-
-
Unknown
-
-
-
-
-
Andreas Laner
00414242
0000322861
Generalized non-motor (absence) seizure, Neurodevelopmental delay, Precocious puberty
-
-
Unknown
10y
-
-
-
-
Andreas Laner
00432294
0000325227
High-arch and narrow palate, congenital heart defect; fifth finger brachydactyly, short hands, short feet, scoliosis, intellectual disability, short stature, delayed bone age
Syndromic short stature
KBG syndrome
Complex
7
-
-
-
DYH
Dongye He
00434980
0000332806
Intrauterine growth retardation, Abnormality of prenatal development or birth, Polyhydramnios, Arteria lusoria
prenatal
-
Isolated (sporadic)
21+5
-
-
-
-
Andreas Laner
00443473
0000342112
Neurodevelopmental delay, Microcephaly, Hypertelorism, Autism, Intellectual disability, Infantile muscular hypotonia
-
-
Isolated (sporadic)
06y
-
-
-
-
Andreas Laner
00453447
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