
 Phenotype ID
|

 Phenotype details
|

 Diagnosis/Initial
|

 Diagnosis/Definite
|

 Inheritance
|

 Age/Examination
|

 Age/Diagnosis
|

 Age/Onset
|

 Phenotype/Onset
|

 Protein
|

 Owner
|

 Individual ID
|
| 0000028056 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Frans Cremers |
00037535 |
| 0000028070 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Frans Cremers |
00037549 |
| 0000220799 |
"Best-corrected visual acuity: no light pursuit; 0.05
Optometry: NA
intraocular pressure (mmHg): NA; 16
Axial length: NA
Corneal: CO; Normal
Lens: Opacity; IOL
Vitreous: NA; Normal
Fundus: NA; CA
Ultrasonography: Atrophy; NA
FFA (fundus fluorescein angiography): NA; NA" |
- |
FEVR |
Familial |
44y |
- |
- |
- |
- |
Dong Sun |
00287050 |
| 0000220800 |
"Best-corrected visual acuity: no light pursuit; hand movement/10 cm
Optometry: NA
intraocular pressure: NA; 8
Axial length: NA
Corneal: CO; Normal
Lens: NA;Opacity
Vitreous: NA; NA
Fundus: NA; NA
Ultrasonography: Atrophy; membrane connected with optic disc
FFA(fundus fluorescein angiography): NA; NA" |
- |
FEVR |
Familial |
40y |
- |
- |
- |
- |
Dong Sun |
00287052 |
| 0000220801 |
"best-corrected visual acuity:1.0; 1.0
Optometry: -0.75DS/-3.50 DC*10; +1.25DS/ +1.50DC*80
intraocular pressure (mmHg):12; 12
Axial length: NA
Corneal: Normal Normal
Lens: Normal Normal
Vitreous: vitreous hemorrhage; vitreous hemorrhage
Fundus: brush-like vessels; brush-like vessels
Ultrasonography: vitreous hemorrhage; vitreous hemorrhage
FFA: avascular zone; neovascularization" |
- |
FEVR |
Familial |
13y |
- |
06y |
- |
- |
Dong Sun |
00287053 |
| 0000308407 |
- |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416895 |
| 0000308408 |
- |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416896 |
| 0000308409 |
- |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416897 |
| 0000308410 |
0m: retina: avascular temporal peripheries and vascular shunts at the site of the joining of the vascularized and avascularized tissue similar to that observed in retinopathy of prematurity; treated with cryotherapy, maintained their sight; myopia |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
15y |
- |
- |
- |
- |
LOVD |
00416898 |
| 0000308411 |
0m: retina: avascular temporal peripheries and vascular shunts at the site of the joining of the vascularized and avascularized tissue similar to that observed in retinopathy of prematurity; treated with cryotherapy, maintained their sight; myopia |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
11y |
- |
- |
- |
- |
LOVD |
00416899 |
| 0000308412 |
blind in the right eye by 13y; right eye: phthisis, atrophy of the iris, a dense cataract and nystagmus; left eye: visual acuity: 4/60, large falciform retinal fold; 18y: left retina detached with a large yellow exudate present underneath the retina; fibrous tissue and vitreous thickening were with neovascularization leading to leakage; deterioration of sight in the left eye treated by cryocoagulation and the patient has subsequently maintained the visual acuity in this eye |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
33y |
- |
- |
- |
- |
LOVD |
00416900 |
| 0000308413 |
- |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416901 |
| 0000308414 |
- |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416902 |
| 0000308415 |
- |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416903 |
| 0000308416 |
right eye enucleated due to intraocular tumor suspicion after recurrent iritis, histological examination: severe intraocular inflammation without any malignancy; visual acuity on the left eye remained unchanged until adulthood; last few years a slight decrease of visual acuity; 29y: visual acuity: 20/300 with medium myopia ( -3.0 diopters) and nystagmus; anterior segment: normal; retina: severe temporal dragging of the vessels, including the macula where scar with peripheral hyperpigmentation and central depigmentation was noticed; no exudates present, it cannot be excluded that areas of central and peripheral scars have been partly induced by retinal exudates; peripheral retina: pigmentation between the equator and the ora serrata on the temporal side, nasally, equatorial degenerations present |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
29y |
- |
0m |
low visual acuity |
- |
LOVD |
00416904 |
| 0000308418 |
whole family description: characteristic ophthalmological findings of Norrie disease; but no mental retardation or hearing abnormality |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416906 |
| 0000308419 |
whole family description: characteristic ophthalmological findings of Norrie disease; but no mental retardation or hearing abnormality |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416907 |
| 0000308420 |
whole family description: characteristic ophthalmological findings of Norrie disease; but no mental retardation or hearing abnormality |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416908 |
| 0000308421 |
whole family description: characteristic ophthalmological findings of Norrie disease; but no mental retardation or hearing abnormality |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416909 |
| 0000308422 |
whole family description: characteristic ophthalmological findings of Norrie disease; but no mental retardation or hearing abnormality |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416910 |
| 0000308423 |
3 months for poor visual development; term infant, birth weight of 3,000 g, otherwise healthy; pendular nystagmus; corneae: clear, anterior chambers: shallow; ophthalmoscopy: large retinal folds and a white temporal mass of apparent exudate that pulled the retinal vessels toward the temporal ora serrata and attached near the temporal posterior edge of the lens; bilateral congenital retinal folds initially diagnosed |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
3m |
3m |
- |
poor visual development |
- |
LOVD |
00416911 |
| 0000308424 |
all relatives of the proband description: symptoms of retinal traction, peripheral vitreous opacities, and subretinal and intraretinal exudates |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416912 |
| 0000308425 |
all relatives of the proband description: symptoms of retinal traction, peripheral vitreous opacities, and subretinal and intraretinal exudates |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416913 |
| 0000308426 |
all relatives of the proband description: symptoms of retinal traction, peripheral vitreous opacities, and subretinal and intraretinal exudates |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416914 |
| 0000308427 |
all relatives of the proband description: symptoms of retinal traction, peripheral vitreous opacities, and subretinal and intraretinal exudates |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416915 |
| 0000308428 |
all relatives of the proband description: symptoms of retinal traction, peripheral vitreous opacities, and subretinal and intraretinal exudates |
- |
familial exudative vitreoretinopathy |
Familial, X-linked recessive |
- |
- |
- |
- |
- |
LOVD |
00416916 |
| 0000308429 |
whole family description: congenital blindness and hearing loss; diagnosed as having Norrie disease; proband born in to healthy parents with an uncomplicated delivery; maternal uncle, maternal grandmother and maternal grand uncles had a history of congenital blindness and hearing loss; 14d: bilateral tractional retinal detachments andvitreous hemorrhage with normal anterior segments and clear lenses; mother phenotypically completely normal |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
- |
- |
- |
LOVD |
00416918 |
| 0000308430 |
whole family description: congenital blindness and hearing loss; diagnosed as having Norrie disease |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
- |
- |
- |
LOVD |
00416919 |
| 0000308431 |
whole family description: congenital blindness and hearing loss; diagnosed as having Norrie disease |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
- |
- |
- |
LOVD |
00416920 |
| 0000308432 |
whole family description: congenital blindness and hearing loss; diagnosed as having Norrie disease |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
- |
- |
- |
LOVD |
00416921 |
| 0000308433 |
whole family description: congenital blindness and hearing loss; diagnosed as having Norrie disease |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
- |
- |
- |
LOVD |
00416922 |
| 0000308434 |
whole family description: congenital blindness and hearing loss; diagnosed as having Norrie disease |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
- |
- |
- |
LOVD |
00416923 |
| 0000308435 |
whole family description: congenital blindness and hearing loss; diagnosed as having Norrie disease |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
- |
- |
- |
LOVD |
00416924 |
| 0000308436 |
full term pregnancy, born to healthy non-consanguinous parents with an uncomplicated delivery; ophthalmic examinalion: retinal fold extending towards the retinal periphery, an intravitreous fibrous mass and membrane, vitreous detachment and peripheral exudates at the junction between the avascular and vascular retina; no exposure to supplemental oxygen |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
- |
- |
- |
LOVD |
00416925 |
| 0000308454 |
1y: fundoscopy (general anaesthesia): inferotemporal peripheral vascular and gliotic changes in the right eye and a dragged macula with retinal exudation in the left eye; right eye treated with retinal laser photocoagulation and the left developed a total retinal detachment |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
2y |
- |
1y |
left esotropia and an abnormal red reflex in the left eye |
- |
LOVD |
00416943 |
| 0000308477 |
no hearing impairment |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
4y |
- |
- |
LOVD |
00416966 |
| 0000308478 |
no hearing impairment |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
3y |
- |
- |
LOVD |
00416967 |
| 0000308480 |
no hearing impairment; additional disease - glucose-6-phosphate dehydrogenase deficiency |
- |
persistent fetal vasculature syndrome |
Familial, X-linked |
- |
- |
3m |
- |
- |
LOVD |
00416969 |
| 0000308481 |
no hearing impairment |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
3m |
- |
- |
LOVD |
00416970 |
| 0000308488 |
no hearing impairment |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
- |
- |
01y06m |
- |
- |
Anna Tracewska |
00416977 |
| 0000308496 |
best corrected visual acuity right/left eye: no light pursuit/0.05; optometry: not available; intraocular pressure right, left eye: (mmHg): not available, 16; cornea right, left eye: corneal opacity, normal; lens right, left eye: opacity, intraocular lens; vitreous: not available; normal; fundus: not available, chorioretinal atrophy; ultrasonography: atrophy, not available; fundus fluorescein: not available, not available |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
>18y |
- |
44y |
- |
- |
LOVD |
00416985 |
| 0000308497 |
best corrected visual acuity right/left eye: no light pursuit/hand movement 10 cm; optometry: not available; intraocular pressure right, left eye: (mmHg): not available, 8; cornea right, left eye: corneal opacity, normal; lens right, left eye: not available, opacity; vitreous: not available, not available; fundus: not available, not available; ultrasonography: atrophy, membrane connected with optic disc; fundus fluorescein: not available, not available |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
>18y |
- |
40y |
- |
- |
LOVD |
00416986 |
| 0000308498 |
best corrected visual acuity right/left eye: 1/1; optometry: -0.75DS/-3.50DC*10; +1.25DS/+1.50DC*80; intraocular pressure right, left eye: (mmHg): 12, 12; cornea right, left eye: normal, normal; lens right, left eye: normal, normal; vitreous: vitreous hemorrhage, vitreous hemorrhage; fundus: brush-like vessels, brush-like vessels; ultrasonography: vitreous hemorrhage, vitreous hemorrhage; fundus fluorescein: avascular zone, not available |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
6y |
- |
13y |
- |
- |
LOVD |
00416987 |
| 0000308502 |
best corrected visual acuity right/left eye: no light pursuit/0.05; optometry: not available; intraocular pressure right, left eye: (mmHg): not available, 16; cornea right, left eye: corneal opacity, normal; lens right, left eye: opacity, intraocular lens; vitreous: not available; normal; fundus: not available, chorioretinal atrophy; ultrasonography: atrophy, not available; fundus fluorescein: not available, not available |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
>18y |
- |
44y |
- |
- |
LOVD |
00416991 |
| 0000308503 |
best corrected visual acuity right/left eye: no light pursuit/hand movement 10 cm; optometry: not available; intraocular pressure right, left eye: (mmHg): not available, 8; cornea right, left eye: corneal opacity, normal; lens right, left eye: not available, opacity; vitreous: not available, not available; fundus: not available, not available; ultrasonography: atrophy, membrane connected with optic disc; fundus fluorescein: not available, not available |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
>18y |
- |
40y |
- |
- |
LOVD |
00416992 |
| 0000308504 |
best corrected visual acuity right/left eye: 1/1; optometry: -0.75DS/-3.50DC*10; +1.25DS/+1.50DC*80; intraocular pressure right, left eye: (mmHg): 12, 12; cornea right, left eye: normal, normal; lens right, left eye: normal, normal; vitreous: vitreous hemorrhage, vitreous hemorrhage; fundus: brush-like vessels, brush-like vessels; ultrasonography: vitreous hemorrhage, vitreous hemorrhage; fundus fluorescein: avascular zone, not available |
- |
familial exudative vitreoretinopathy |
Familial, X-linked |
6y |
- |
13y |
- |
- |
LOVD |
00416993 |
| 0000308505 |
- |
- |
familial exudative vitreoretinopathy |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
LOVD |
00416994 |
| 0000308506 |
- |
- |
familial exudative vitreoretinopathy |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
LOVD |
00416995 |
| 0000308507 |
- |
- |
familial exudative vitreoretinopathy |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
LOVD |
00416996 |
| 0000308508 |
- |
- |
familial exudative vitreoretinopathy |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
LOVD |
00416997 |
| 0000308509 |
- |
- |
familial exudative vitreoretinopathy |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
LOVD |
00416998 |