Phenotypes for disease #05213 (BAMS (arhinia, choanal atresia, microphthalmia, Bosma type (BAMS)), OMIM:603457)

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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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