Global Variome shared LOVD
PFKL (phosphofructokinase, liver)
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Phenotypes for disease #05213 (BAMS (arhinia, choanal atresia, microphthalmia, Bosma type (BAMS)), OMIM:603457)
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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Date
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Date
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Date
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Date
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51 entries on 1 page. Showing entries 1 - 51.
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Phenotype ID
Phenotype details
Diagnosis/Initial
Diagnosis/Definite
Inheritance
Age/Examination
Age/Diagnosis
Age/Onset
Phenotype/Onset
Protein
Owner
Individual ID
0000073650
complete arhinia; microcorneas and corneal opacities, Deformed pupils, Globulous cataract (bilateral), Ectopic lens (R), Ectopic pupil (L), Primitive vitreous persistence (R), Bilateral microphthalmia, Bilateral esotropia, Limited visual acuity, Hypoplasia of the optic nerves; Hypertelorism, Absence of the nasal cavity, nasal bones and of cartilaginous nasal structures, Agenesis of the maxillary, frontal, sphenoidal and ethmoidal sinuses, Hypoplasia of the ethmoid and maxilla, Preauricular sinus (R); normal reproductive system; normal growth; normal psychomotor development; absence of olfactory bulbs and sulci
-
-
Isolated (sporadic)
05y
-
-
-
-
Johan den Dunnen
00095254
0000073651
complete arhinia; microphthalmia, bilateral strabismus; hypertelorism, High arched palate, Choanal atresia, Absent nasolacrimal ducts; delayed menstruation requiring hormone induction at puberty; normal growth; normal psychomotor development
-
-
Isolated (sporadic)
28y
-
-
-
-
Johan den Dunnen
00095255
0000073652
complete arhinia; normal eyes; absence of the nasal cavity, the nasal spine of the frontal bone,the nasal bones, the vomer and the conchae, midline cleft of the cribriform plate, absence of central incisor(s); normal growth; normal psychomotor development; olfactory bulb agenesis
-
-
Isolated (sporadic)
04y
-
-
-
-
Johan den Dunnen
00095256
0000073653
complete arhinia; eye coloboma (L) involving the disc, lens and retina, cataract; bilateral bony choanal atresia. Hypoplasia of maxillary sinuses, mild hypertelorism; normal growth; delayed psychomotor development; small apical ventricular septal defect
-
-
Isolated (sporadic)
03y
-
-
-
-
Johan den Dunnen
00095257
0000073654
arhinia; microphthalmia (R) with severe visual impairment, microphthalmia (L) with sclerocornea; midface hypoplasia, bifid philtral region, small mouth, high palate with soft palate cleft, small preauricular pit (R), hypoplasia of maxillary sinus, nuchal oedema, hypoplasia of nasolacrimal duct (L); micropenis, normal testes, hypogonadotropic hypogonadism; at birth, weight 25th%, head circumference 75th%, length >99th%; growth delayed, weight and height just over 9th%; severe learning difficulties; moderate persistent ductus arteriosus, and tiny persistent foramen ovale at birth
-
-
Isolated (sporadic)
07y
-
-
-
-
Johan den Dunnen
00095258
0000073655
complete arhinia ; chronic epiphora. Cataract (R); facial asymmetry. Mid-face hypoplasia; micropenis. Bilateral undescended testes. Low gonadatropins; has been started on testosterone supplements; height delayed. Weight and OFC normal; learning difficulties; transaminitis associated with hypoalbuminaemia and peripheral oedema, with diffuse echogenic liver consistent with fatty infiltration. Bronchiectasis, fixed flexion contractures hip/knees
-
-
Isolated (sporadic)
14y
-
-
-
-
Johan den Dunnen
00095259
0000073656
complete arhinia ; bilateral microphthalmia. Coloboma of the iris; midface hypoplasia. High arched palate. Hypertelorism. Absent nasolacrimal ducts; micropenis. Hypogonadotropic hypogonadism; 9y-growth normal; autistic and aggressive behavior. Psychomotor developmental quotient moderately delayed. Needs special education program at school; generalized seizures. Agenesis of the olfactory bulbs
-
-
Isolated (sporadic)
13y
-
-
-
-
Johan den Dunnen
00095260
0000073657
complete arhinia (small pit to the left of the columella, no nasal airway); bilateral microphthalmia. Short palpebral fissures. Blepharophimosis. Bilateral corneal opacities. Upbeat nystagmus with poor fixation following reflexes. Flash Visual Evoked Potential evident bilaterally, but no consistent evidence of patent Visual Evoked Potential, suggesting visual impairment; high arched palate, hypertelorism. Bilateral complete absence of the lacrimal apparatus. Absence of nasal cavity and ethmoid sinuses; normal reproductive system; normal growth; normal psychomotor development; floor of the anterior cranial fossa is deficient anteriorly, with an encephalocele.
-
-
Unknown
04y
-
-
-
-
Johan den Dunnen
00095261
0000073658
complete arhinia; eyes normal; telecanthus. Downward slant palpebral fissure. Midface hypoplasia. Obstruction of nasolacrimal passage. Absence of nasal cavity, nasal bone and anterior soft tissue. High arched palate. Paramedian notch of the upper lip (bilateral). Nonpneumatization of the maxillary, sphenoid and ethmoid sinuses; slim penis; normal growth; normal psychomotor development; bilateral olfactory bulb agenesis
-
-
Isolated (sporadic)
03y
-
-
-
-
Johan den Dunnen
00095262
0000073659
partial arhinia (patency of left nostril but complete obstruction of left posterior nare); normal eyes; telecanthus. Upward slant palpebral fissure. Midface hypoplasia. Obstruction of nasolacrimal passage. Nasal bone hypoplasia. High arched palate. Nonpneumatization of the maxillary, sphenoid and ethmoid sinuses; normal reproductive system; normal growth; normal psychomotor development; olfactory bulb agenesis (L).
-
-
Isolated (sporadic)
03y
-
-
-
-
Johan den Dunnen
00095263
0000073660
complete arhinia ; normal eyes; telecanthus. High arched palate. Agenesis of nasal bone. Absence of nasal cavity. Midface hypoplasia; normal reproductive system; normal growth; normal psychomotor development; atrial septal defect
-
-
Isolated (sporadic)
09y
-
-
-
-
Johan den Dunnen
00095264
0000073661
complete arhinia ; bilateral microphthalmia. Ectopic iris. Vision loss (can only see light); hypertelorism. Posterior cleft palate. Gingival cleft of lower arch. Cleft lower lip (operated). Cleft mandibula. Underdeveloped superior ear pinnae, auricular pit (R). Absent auditory canal (R). CT: absence of external auditory canals. Absence of nasal cavity, nasal bone and anterior soft tissue. Midface hypoplasia. Obstruction of nasolacrimal passage; hypoplastic labia majora. No menarche or secondary sex development; tall and strong build; severe psychomotor retardation. Walked at 4.5 years, talked with two word sentences at the age of 4 years then lost vocabulary. Has prominent articulation defect. Never had toilet training. Has sleep disorder. Stereotypic movements. Anxious, aggressive and non-cooperative; bilateral olfactory bulb agenesis
-
-
Isolated (sporadic)
26y
-
-
-
-
Johan den Dunnen
00095265
0000073662
complete arhinia ; bilateral coloboma of the iris; high arched palate. Slight hypertelorism. Absence of paranasal sinuses, nasal bones, cribriform plate and septal structures. Maxillary hypoplasia. Nasolachrymal duct atresia; delayed puberty with low gonadotropins; has been started on estrogen; normal growth; normal psychomotor development; absence of olfactory bulbs and sulci. Fused medial orbital and rectus gyri
-
-
Unknown
17y
-
-
-
-
Johan den Dunnen
00095266
0000073663
complete arhinia ; bilateral microphthalmia. Iris anatomy unknown; hypertelorism. Coarse calcifications along the medial aspect of the globes bilaterally. Narrow high arched palate. Choanal and mastoid air cell atresia bilaterally with a dysmorphic and expansile appearance of the greater wing of the sphenoid bone and infratemporal surface of the maxillary bone bilaterally. Absent paranasal sinuses. Central cleft between the dysmorphic maxilla. Absent cribriform plate and crista galli. No identified osseous plate between the non-pneumatized central nasal passage and the frontal lobes; external genitalia norma; growth deficiency. Thought to have nutritional deficiency. BMI 13.7; significant psychomotor delays. Thought to be related to lack of environmental stimuli; no evidence of olfactory bulbs on CT scan
-
-
Unknown
04y
-
-
-
-
Johan den Dunnen
00095267
0000073674
see paper; …
-
-
Isolated (sporadic)
44y
-
-
-
-
Johan den Dunnen
00095277
0000073675
see paper; …
-
-
Unknown
40y
-
-
-
-
Johan den Dunnen
00095278
0000073676
see paper; …
-
-
Unknown
22y
-
-
-
-
Johan den Dunnen
00095279
0000073677
see paper; …
-
-
Unknown
73y
-
-
-
-
Johan den Dunnen
00095280
0000073678
see paper; …
-
-
Unknown
53y
-
-
-
-
Johan den Dunnen
00095281
0000073679
see paper; …
-
-
Unknown
44y
-
-
-
-
Johan den Dunnen
00095282
0000073680
see paper; …
-
-
Unknown
6y
-
-
-
-
Johan den Dunnen
00095283
0000073681
see paper; …
-
-
Unknown
7y
-
-
-
-
Johan den Dunnen
00095284
0000073682
see paper; …
-
-
Unknown
16y
-
-
-
-
Johan den Dunnen
00095285
0000073683
see paper; …
-
-
Isolated (sporadic)
24y
-
-
-
-
Johan den Dunnen
00095286
0000073684
see paper; …
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00095287
0000073685
see paper; …
-
-
Unknown
12y
-
-
-
-
Johan den Dunnen
00095288
0000073686
see paper; …
-
-
Isolated (sporadic)
26y
-
-
-
-
Johan den Dunnen
00095289
0000073687
see paper; …
-
-
Isolated (sporadic)
17y
-
-
-
-
Johan den Dunnen
00095290
0000073688
see paper; …
-
-
Unknown
15y
-
-
-
-
Johan den Dunnen
00095291
0000073689
see paper; …
-
-
Unknown
54y
-
-
-
-
Johan den Dunnen
00095292
0000073690
see paper; …
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00095293
0000073691
see paper; …
-
-
Unknown
45y
-
-
-
-
Johan den Dunnen
00095294
0000073692
see paper; …
-
-
Unknown
3y
-
-
-
-
Johan den Dunnen
00095295
0000073693
see paper; …
-
-
Isolated (sporadic)
3y
-
-
-
-
Johan den Dunnen
00095296
0000073694
see paper; …
-
-
Unknown
9y
-
-
-
-
Johan den Dunnen
00095297
0000073695
see paper; …
-
-
Isolated (sporadic)
51y
-
-
-
-
Johan den Dunnen
00095298
0000073696
see paper; …
-
-
Unknown
20y
-
-
-
-
Johan den Dunnen
00095299
0000073697
see paper; …
-
-
Unknown
13y
-
-
-
-
Johan den Dunnen
00095300
0000073698
see paper; …
-
-
Unknown
1y
-
-
-
-
Johan den Dunnen
00095301
0000073699
see paper; …
-
-
Familial
38y
-
-
-
-
Johan den Dunnen
00095302
0000073700
see paper; …
-
-
Isolated (sporadic)
11y
-
-
-
-
Johan den Dunnen
00095303
0000073701
see paper; …
-
-
Familial
8y
-
-
-
-
Johan den Dunnen
00095304
0000073702
see paper; …
-
-
Isolated (sporadic)
5y
-
-
-
-
Johan den Dunnen
00095305
0000073703
see paper; …
-
-
Unknown
17y
-
-
-
-
Johan den Dunnen
00095306
0000073704
see paper; …
-
-
Familial
10y
-
-
-
-
Johan den Dunnen
00095307
0000073705
see paper; …
-
-
Unknown
20y
-
-
-
-
Johan den Dunnen
00095308
0000073706
see paper; …
-
-
Isolated (sporadic)
4y
-
-
-
-
Johan den Dunnen
00095309
0000073707
see paper; …
-
-
Unknown
8y
-
-
-
-
Johan den Dunnen
00095310
0000185417
-
Bosma arhinia microphthalmia syndrome
BAMS
Familial, autosomal dominant
-
-
-
-
-
Richard Lemmers
00245453
0000185438
-
Bosma arhinia microphthalmia syndrome
BAMS
Familial, autosomal dominant
-
-
-
-
-
Richard Lemmers
00245480
0000185439
-
Bosma arhinia microphthalmia syndrome
BAMS
Familial, autosomal dominant
-
-
-
-
-
Richard Lemmers
00245481
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