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Phenotypes for disease #00783 (PVNH4 (heterotopia, periventricular, Ehlers-Danlos variant, type 4 (PVNH-4)), OMIM:300537)
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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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Date
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17 entries on 1 page. Showing entries 1 - 17.
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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
0000024773
skin hyperelasticity, joint hypermobility, elbow and scapulohumeral dislocation, subarachnoid haemorrhage, hiatus hernia, mitral prolapse
-
-
Familial
-
-
-
-
-
Yu Sun
00028751
0000024774
skin hyperelasticity, joint hypermobility, shoulder and knee dislocations, high arched palate, lumbar scoliosis, pectus excavatum
-
-
Familial
-
-
-
-
-
Yu Sun
00028752
0000024775
skin hyperelasticity, joint hypermobility, knee dislocations, bilateral inguinal hernias, pyloric stenosis, high arched palate, lumbar hyperlordosis
-
-
Familial
-
-
-
-
-
Yu Sun
00028753
0000024884
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028747
0000024885
-
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028748
0000024886
###
-
-
Isolated (sporadic)
-
-
-
-
-
Yu Sun
00028749
0000058791
5y diagnosed with EDS based on soft and slightly hyperextensible skin, diaphragmatic eventration, umbilical hernia, joint hypermobility (Beighton score 8/9); mild pectus carinatum, mild lumbar scoliosis; normal palate/uvula; normal wound healing, normal scarring, normal bruising, no joint dislocations; EMG and elastin staining skin biopsy normal; 15y-MRI paucinodular PH,headaches, episodes of dizziness/poor balance, no associated cortical malformations; early childhood development, subsequent learning, intelligence, mainstream school progress normal; ECG mild mitral valve prolapse with thickened mitral valve leaflets, normal aortic valve, aortic diameter sinuses level of Valsalva 31mm (normal range 28mm); MRA cerebral arteries normal; 16y-admitted for spontaneous pneumothorax
-
-
Familial, X-linked
16y
05y
-
-
-
M Walsh
00074612
0000073493
joint hypermobility (7/9) (HP:0001382), seizures (HP:0001250), pulmonary root dilatation (HP:0004927), normal aorta (-HP:0001679), normal valves (-HP:0001654), thin skin with visible vessels (HP:0000963), no abnormalities craniofacial (-HP:0000271, -HP:0000234)
-
-
Isolated (sporadic)
-
-
>00y
-
-
Johan den Dunnen
00095101
0000073494
no abnormalities aorta (-HP:0001679), aneurysm right subclavian artery (HP:0002617), no abnormalities valves (-HP:0001654), easy bruising (HP:0000978), soft skin (HP:0000977), normal wound healing (-HP:0001058), normal scars (-HP:0000987), no skin hyper extensibility (-HP:0008067), single uvula (-HP:0000172), normal palate (-HP:0008067), no dysmorphic features (-HP:0001999), normal habitus (-HP:0004323), joint hypermobility (7/9) (HP:0001382), no dislocations (-HP:0001373), joint pain (HP:0002829), normal genetic bone survey (HP:0000924), no seizures (-HP:0001250), periventricular nodular heterotopia (HP:0007165)
-
-
Familial, X-linked dominant
-
-
?
-
-
Johan den Dunnen
00095100
0000073495
Generalised joint laxity (HP:0002761), no seizures (-HP:0001250), Dilatation Aorta ascendens (HP:0005111), Thin skin (HP:0000963), Abnormality of the aortic valve (HP:0001646), Patent ductus arteriosus (ligated at 0y3m)(HP:0001643), Cognitive impairment (HP:0100543)
-
-
Familial, X-linked recessive
-
-
?
-
-
Johan den Dunnen
00095102
0000073496
joint hypermobility (HP:0001382), Seizures (HP:0001250), periventricular nodular heterotopia, mitralic valve prolapse (HP:0001634), ventriculomegaly (HP:0002119), soft thin skin (HP:0000963), normal elasticity, normally healed scars, agenesis of corpus collosum (HP:0001338), cognitive impairment (HP:0100543)
-
-
Familial, X-linked recessive
15y
-
-
-
-
Johan den Dunnen
00095103
0000073499
Congenital hip dysplasia (HP:0001385), JH (8/9) (HP:0001382), Seizures (HP:0001250), periventricular nodular heterotopia (HP:0007165), Aortic root dilatation (HP:0002616), pulmonary artery dilatation (HP:0004927), Mild skin thinning (HP:0000963), ‘criss crossing’ of palmar skin (HP:0011356), Non-dysmorphic (-HP:0001999) , Umbilical hernia (HP:0001537)
-
-
Familial, X-linked dominant
-
-
?
-
-
Johan den Dunnen
00095106
0000073500
joint hypermobility (HP:0001382), no dislocations (-HP:0001373), joint pain (HP:0002829), aortic aneurysm (HP:0004942), sinus of valsalva aneurysm (HP:0011645), diffuse ectasia (HP:?), normal valves (-HP:0001654), easy bruising (HP:0000978), soft skin (HP:0000977), normal wound healing (-HP:0001058), normal scars (HP:0001075, HP:0001073), no skin hyperextensibility (-HP:0000974), stretch marks (HP:0001065), normal palate (-HP:0000174), single uvula (HP:0000172), no dysmorphic features (-HP:0001999)
-
-
Familial, X-linked dominant
-
-
<38y
-
-
Johan den Dunnen
00095099
0000073503
no abnormality of habitus (HP:0004323), joint hypermobility (HP:0001382), seizures (HP:0001250), periventricular heterotopia (HP:0002282), mitral valve prolapse (HP:0001634), thin skin (HP:0000963), doughy skin (HP:0001027), no skin hyperextensibility (-HP:0000974), no poor wound healing (-HP:0001058), non dysmorphic face (-HP:0001999), high arched palate (HP:0002705), supraumbilical hernia (HP:0001537); brain MRI periventricular heterotropia (PH); ECG prolapse mitral valve; 8y supra-umbilical hernia repaired; 1m small atrial septal defect; normal wound healing
-
-
Familial, X-linked dominant
10y
-
00y01m
-
-
Johan den Dunnen
00095107
0000073504
Joint laxity (HP:0002761), No seizure (HP:0001250), Periventricular heterotopia (HP:0002282), Hypoplasia of corpus callosum (HP:0007370), Syringomyelia (HP:0003396), Dilation of ascending aorta (HP:0005111), Dilation of abdominal aorta (HP:0005112), Dysplastic aortic (HP:0005176), Dysplastic mitral valve (HP:0001633), Dysplastic tricuspid valves (HP:0001702), Tortuous supra-aortic vessels , Elastic skin (HP:0000974), Non dysmorphic face (HP:0001999), Normal palate (HP:0000174), Intestinal malrotation (HP:0002566), Abnormality of elastic fibers
-
-
Familial, X-linked dominant
-
-
00y00m?
-
-
Johan den Dunnen
00095108
0000073508
no joint hypermobility, seizures, periventricular nodular heterotopia, ECG normal, thin skin, normal scar formation, non-dysmorphic,
-
-
Unknown
41y
-
-
-
-
Johan den Dunnen
00095113
0000106397
PVNH myxomatous and prolapsed mitral valve with moderate regurgitation tricuspid aortic valve prolapse with mild regurgitation brachycephaly, telecanthus, epicanthal folds, periorbital fullness and infraorbital creases bilaterally and low-set, posteriorly rotated ears skin laxity Increased mobility across all joints brachydactyly with proximally placed thumbs, flat feet with sandal gap and medially deviated great toes tall vertebral bodies
-
-
Isolated (sporadic)
-
04y
-
-
-
Elyssa Cannaerts
00133647
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