The FBN1 mutations database - List of Phenotypic information


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SymptomSeverity
C-Aneurysm asc.aorta
C-Aortic regurgitation (m/-)
moderate
surgery
C-Aortic root aneurysm (m/-)
surgery
C-Aortic root dilation (M/M)
borderline
slight
surgery
C-Aortic root dissection (M/M)
C-Aortic root replacement
C-Aortic valve insufficiency
moderate
severe
surgery
C-Aortic valve prolapse
C-Arhythmia
C-Atrial septal defect
C-Bicuspidy
C-Bradycardy
C-Cardiomegaly
C-Congestive heart failure
C-Coronary artery aneurysm
C-Dilation asc. aorta (M/M)

borderline
moderate
severe
surgery
C-Dilation desc. aorta (-/m)
C-Dilation of atria
C-Dilation pulmonary art. (-/m)
mild
C-Dissecting aneurysm
surgery
C-Dissection abdominal aorta (M/m)
C-Dissection asc. aorta (M/M)
surgery
C-Dissection desc. Aorta (M/m)
surgery
C-Dysrythmia (m/-)
C-Enlarged heart
C-Incomplete description
C-Mitral endocarditis
C-Mitral regurgitation
borderline
mild
moderate
severe
C-Mitral valve insufficiency
moderate
severe
surgery
C-Mitral valve prolapse (m/m)
mild
surgery
C-Mixomatous mitral valve
C-Multiple aneurysms
C-Myocardial mass
C-Pulmonary emphysema
C-Pulmonary valve insufficiency
C-Thoracic des. aorta neurysm
C-Tricuspid regurgitation
mild
C-Tricuspid valve insufficiency
moderate
C-Tricuspid valve prolapse
moderate
C-Ventricular hypertrophy
CNS-Dural ectasia (M/M)
suspected
CNS-Enlarged cisterna magna (m/-)
CNS-Lombosacral meningocele (m/-)
CNS-Mental retardation
CNS-Verbal expression pb (m/-)
CNS-Widening of the lumbosacral canal
L-Apical blebs (m/m)
L-hemidiaphragm eventration
L-Pneumectomy
L-Pulmonary emphysema
L-Spontaneous pneumothorax (m/m)
bilateral
no clinical data
O-Adhesion of iris with cornea
O-Amblyopia
O-Aphakia
bilateral
O-Asthigmatism
O-Bluish sclerae
O-Cataract
O-Coloboma
O-Congenital miosis
O-Corneal detachment
O-Divergent strabismus
O-Ectopia lentis (M/M)
bilateral
surgery
O-Flat cornea (<42 dp) (m/m)
bilateral
slight
O-Glaucoma
O-Incomplete description
O-Increased axial length of globe (m/m)
O-Iridodonesis
O-Iris dilatator hypoplasia (-/m)
O-Lens extraction
bilateral
O-Megalocornea
O-Microphthalmus
O-Microspherophakia
O-Miosis
O-Myopia (m/-)
early
mild
severe
O-No implication
O-Not examined
O-Peripheral iris atrophy
O-Retinal ablation
O-Retinal aplasiabilateral
O-Retinal degeneration
bilateral
O-Retinal detachment (m/-)
bilateral
surgery
O-Shallow anterior chambers
O-Spherophakia
O-Strabismus
S-Abnormal ears
S-Abnormal face
S-Arachnodactyly (m/M)
borderline
moderate
severe
S-Arm span/height >1.05 (-/M)
S-Camptodactyly
S-Chest deformity (unspecified) (m/m)
S-Chondromalacia Patellae
S-Craniosynostosis
S-Crumpled ears
S-Cubitus valgus
S-Dental crowding (m/m)
moderate
S-Dolichocephalia (-/m)
S-Dolichostenomelia (m/-)
extreme
moderate
severe
S-Downward palpebral fissures (-/m)
S-Enophthalmus (-/m)
S-Flat feet
S-Foot deformity
S-Incomplete description
S-Increased body length (m/-)
S-Infantile hypotonia
S-Joint contractures (m/-)
mild
S-Joint dislocation
S-Joint hyper-mobility (m/m)
moderate
S-Knees dislocation
S-Kyphosis (m/-)
S-Leg deformity
S-Long bone over growth
moderate
S-Lordosis (m/-)
severe
S-Malar hypoplasia (-/m)
S-Micrognathia
S-Muscular hypotonia
severe
S-Narrow palatal arch (m/m)
moderate
surgery
S-Patella dislocation
S-Pectus carinatum (m/M)
S-Pectus excavatum moderate (m/m)
S-Pectus excavatum severe (m/M)surgery
S-Pes planovalgus
S-Pes planus (-/M)
bilateral
S-Protusio acetabulæ (m/M)
bilateral
S-Reduced extension of the elbows (-/M)
S-Repeating twists
S-Retrognathia (-/m)
S-Scoliosis (m/M)

mild
severe
surgery
thoracic
S-Short stature
S-US/LS ratio <0.87 (-/M)
S-Valgus elbowbilateral
SI-Bleeding tendency
SI-Inguinal hernia (m/m)

bilateral
SI-Loose, redundant skin
SI-Other herniae (m/m)
diaphragmatic
Hiatal
ombilical
vertebral
SI-Skin hyperextensibility
SI-Striae distensae (m/m)
SI-Tendency to ecchymosis
SI-Translucent skin
SI-Varicose veins