
 Phenotype ID
|

 Phenotype details
|

 Diagnosis/Initial
|

 Diagnosis/Definite
|

 Inheritance
|

 Age/Examination
|

 Age/Diagnosis
|

 Age/Onset
|

 Phenotype/Onset
|

 Protein
|

 Owner
|

 Individual ID
|
0000010387 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00011700 |
0000010388 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00011700 |
0000010389 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christopher Watson |
00003297 |
0000015957 |
see paper |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00017598 |
0000015958 |
see paper; normal situs, EM revealed that, typically, both inner and outer dynein arms are absent |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00017599 |
0000017529 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
A. Mesut Erzurumluoglu |
00019723 |
0000036748 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00050136 |
0000036749 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00050137 |
0000036750 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00050138 |
0000036751 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00050139 |
0000036752 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00050140 |
0000036753 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00050141 |
0000036754 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00050142 |
0000036755 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00050143 |
0000036756 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00050144 |
0000036757 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00050145 |
0000045705 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Marie Legendre |
00059189 |
0000046736 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00059190 |
0000081919 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Sandra Cindrić |
00103983 |
0000129765 |
see paper; ... |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00164726 |
0000129766 |
see paper; ... |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00164727 |
0000129767 |
see paper; ... |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00164728 |
0000129768 |
see paper; ... |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00164729 |
0000129769 |
see paper; ... |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00164730 |
0000129770 |
see paper; ... |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00164731 |
0000129771 |
see paper; ... |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00164732 |
0000144058 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Rahma MANI |
00183321 |
0000144060 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Rahma MANI |
00183322 |
0000144061 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Rahma MANI |
00183323 |
0000144062 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Rahma MANI |
00183324 |
0000144063 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Rahma MANI |
00183325 |
0000144064 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Rahma MANI |
00183326 |
0000144065 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Rahma MANI |
00183327 |
0000144066 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Rahma MANI |
00183328 |
0000144067 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Rahma MANI |
00183329 |
0000144068 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Rahma MANI |
00183330 |
0000144069 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Rahma MANI |
00183331 |
0000157201 |
neonatal respiratory distress, bronchiectasis sinusitis, otitis media, situs solitus |
CILD |
CILD |
Familial, autosomal recessive |
11y |
02y |
- |
- |
- |
Marie Legendre |
00208525 |
0000159284 |
situs inversus totalis, mild respiratory, septo-optic dysplasia |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00210722 |
0000159285 |
no respiratory phenotype, situs inversus totalis |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00210723 |
0000159286 |
mild respiratory phenotype, situs inversus totalis |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00210724 |
0000159287 |
situs ambiguous with complex heart defect (left atrial isomerism, atrioventricular canal defect, interrupted vena cava inferior, anomalous pulmonary vein connection, persistent ductus
arteriosus); 8d-died from cardiac defect |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00210725 |
0000159288 |
mild respiratory phenotype, heterotaxy with situs inversus, interrupted inferior vena cava with azygous continuation, right aortic arch with mirror image head and neck vessel branching, liver and
gall bladder on left, multiple
splenules on right side, hydrocephalus, ventriculoperitoneal shunt after group B streptococcal sepsis as infant |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00210726 |
0000159662 |
no neonatal distress, rhinosinusitis, wet cough, no otitis media, no bronchiectasis, situs inversus, infertility unknown, protein losing enteropathy, complex congenital heart disease (unbalanced atrioventricular septal defect, congenitally corrected transposition great arteries, inferior vena cava conduit stenosis required stenting |
- |
- |
Familial, autosomal recessive |
08y |
- |
- |
- |
- |
Johan den Dunnen |
00211179 |
0000159663 |
no neonatal distress, rhinosinusitis, wet cough with infection, no otitis media, no bronchiectasis, situs inversus, infertility unknown |
- |
- |
Familial, autosomal recessive |
02y |
- |
- |
- |
- |
Johan den Dunnen |
00211180 |
0000159664 |
no neonatal distress, rhinosinusitis, wet cough with infection, no otitis media, no bronchiectasis, situs inversus, infertility unknown, |
- |
- |
Familial, autosomal recessive |
09y |
- |
- |
- |
- |
Johan den Dunnen |
00211181 |
0000159665 |
no neonatal distress, rhinosinusitis, wet cough, no otitis media, no bronchiectasis, situs inversus, infertility |
- |
- |
Familial, autosomal recessive |
13y |
- |
- |
- |
- |
Johan den Dunnen |
00211182 |
0000167657 |
situs solitus, ciliary ultra-structural defect, no neo RDS, Bxsis, sinusitis, otitis media; nNO 42.7 (nl/min) |
- |
- |
Isolated (sporadic) |
- |
14y |
- |
- |
- |
Maimoona Zariwala |
00219103 |
0000167658 |
situs solitus, ciliary ultra-structural defect, neo RDS, Bxsis, sinusitis, otitis media; nNO 5.9 (nl/min) |
- |
- |
Isolated (sporadic) |
- |
3y |
- |
- |
- |
Maimoona Zariwala |
00219105 |
0000167659 |
history of recurrent lower respiratory tract infections, chronic wet cough, persistent segmental collapse left lower lobe with likely bronchiectasis on CT thorax and documented hearing deficit. He also has GSD III. Analysis of nasal brushings showed that all cilia were static or dyskinetic. TEM revealed a transposition defect with absence of the central pair. |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Jillian Casey |
00219106 |
0000167660 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219107 |
0000167661 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219108 |
0000167662 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219109 |
0000167663 |
- |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219110 |
0000167664 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219111 |
0000167665 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219112 |
0000167666 |
situs solitus, ciliary ultra-structural defect, neo RDS, Bxsis, sinusitis, otitis media; nNO 22 (nl/min) |
- |
- |
Familial, autosomal recessive |
- |
6y |
- |
- |
- |
Maimoona Zariwala |
00219115 |
0000167667 |
situs solitus, ciliary ultra-structural defect, neo RDS, sinusitis, otitis media; nNO 10.5 (nl/min) |
- |
- |
Familial, autosomal recessive |
- |
10y |
- |
- |
- |
Maimoona Zariwala |
00219116 |
0000167668 |
situs solitus, ciliary ultra-structural defect, neo RDS, Bxsis, sinusitis, otitis media; nNO 17.8 (nl/min) |
- |
- |
Isolated (sporadic) |
- |
25y |
- |
- |
- |
Maimoona Zariwala |
00219117 |
0000167669 |
situs solitus, ciliary ultra-structural defect, neo RDS, Bxsis, no otitis media; nNO 22.6 (nl/min) |
- |
- |
Isolated (sporadic) |
- |
17y |
- |
- |
- |
Maimoona Zariwala |
00219118 |
0000167670 |
situs solitus, ciliary ultra-structural defect, neo RDS, Bxsis, sinusitis, otitis media; nNO 10 (nl/min) |
- |
- |
Isolated (sporadic) |
- |
9y |
- |
- |
- |
Maimoona Zariwala |
00219119 |
0000167671 |
situs solitus, ciliary ultra-structural defect, neo RDS, Bxsis, sinusitis, otitis media; nNO 24 (nl/min) |
- |
- |
Isolated (sporadic) |
- |
34y |
- |
- |
- |
Maimoona Zariwala |
00219120 |
0000167672 |
situs solitus, neo RDS, Bxsis, sinusitis, otitis media; nNO na (nl/min) |
- |
- |
Familial, autosomal recessive |
- |
37y |
- |
- |
- |
Maimoona Zariwala |
00219121 |
0000167673 |
situs solitus ; nNO na (nl/min) |
- |
- |
Familial, autosomal recessive |
- |
37y |
- |
- |
- |
Maimoona Zariwala |
00219122 |
0000167674 |
situs solitus, ciliary ultra-structural defect, neo RDS, Bxsis, sinusitis, otitis media; nNO 15.2 (nl/min) |
- |
- |
Isolated (sporadic) |
- |
10y |
- |
- |
- |
Maimoona Zariwala |
00219123 |
0000167675 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219124 |
0000167676 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219127 |
0000167677 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219128 |
0000167678 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219131 |
0000167679 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219132 |
0000167680 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219133 |
0000167681 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00219134 |
0000168012 |
neonatal respiratory distress, no bronchiectasis, sinusitis, otitis media, situs solitus, infertile |
- |
- |
Familial, autosomal recessive |
56y |
- |
- |
- |
- |
Johan den Dunnen |
00222891 |
0000170576 |
see paper; ..., chronic chest infections |
primary ciliary dyskinesia |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00225461 |
0000170577 |
see paper; ..., fetus lethal congenital heart disease (2 pregnancies terminated) |
primary ciliary dyskinesia |
- |
Familial, autosomal recessive |
<00y00m00d |
- |
- |
- |
- |
Johan den Dunnen |
00225462 |
0000170578 |
see paper; ... |
primary ciliary dyskinesia |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00225463 |
0000180452 |
see paper; ..., asthenozoospermia |
male infertility |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00240387 |
0000210675 |
see paper; ..., infertile |
primary ciliary dyskinesia |
- |
Familial, autosomal recessive |
24y |
- |
- |
- |
- |
Johan den Dunnen |
00276078 |
0000210676 |
see pape; ..., asthma bronchiectasis rhino-sinusitis |
primary ciliary dyskinesia |
- |
Familial, autosomal recessive |
50y |
- |
- |
- |
- |
Johan den Dunnen |
00276079 |
0000210677 |
see pape; ..., neonatal respiratory distress, bronchiectasis, pneumopathies, rhino-sinusitis otitis |
primary ciliary dyskinesia |
- |
Familial, autosomal recessive |
11y |
- |
- |
- |
- |
Johan den Dunnen |
00276080 |
0000210678 |
see paper; ..., infertile, bronchitis, rhino-sinusitis |
primary ciliary dyskinesia |
- |
Familial, autosomal recessive |
33y |
- |
- |
- |
- |
Johan den Dunnen |
00276081 |
0000339027 |
HP:0001651, HP:0012265, HP:0002110, HP:0004469, HP:0011861, HP:0001696 |
- |
CILD28 |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Marketa Wayhelova |
00449879 |
0000343407 |
see paper; ..., cilia beating pattern unsyncronized, wavy |
primary ciliary dyskinesia |
CILD5 |
Familial, autosomal recessive |
25y |
- |
- |
- |
- |
Johan den Dunnen |
00454796 |
0000343408 |
see paper; ..., cilia beat frequency 10.8 Hz, cilia beating pattern stiff, static |
primary ciliary dyskinesia |
CILD5 |
Familial, autosomal recessive |
18y |
- |
- |
- |
- |
Johan den Dunnen |
00454797 |
0000343409 |
see paper; ..., cilia beat frequency 0 Hz, cilia beating pattern stiff, static |
primary ciliary dyskinesia |
CILD5 |
Familial, autosomal recessive |
24y |
- |
- |
- |
- |
Johan den Dunnen |
00454798 |
0000343410 |
see paper; ..., cilia beat frequency 0 Hz, cilia beating pattern static |
primary ciliary dyskinesia |
CILD5 |
Familial, autosomal recessive |
24y |
- |
- |
- |
- |
Johan den Dunnen |
00454799 |
0000349815 |
heterotaxy, asplenia, midline liver, pulmonary stenosis, interrupted inferior vena cava, bilateral superior vena cava, and right aortic arch |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462316 |
0000349816 |
dextrocardia, pulmonary stenosis, respiratory infections; family members with congenital heart anomalies |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462317 |
0000349817 |
32wg-birth prematurity, persistent atelectasis |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462318 |
0000349818 |
primary ciliary dyskinesia |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462319 |
0000349819 |
primary ciliary dyskinesia |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462320 |
0000349820 |
heterotaxy syndrome (isomerism) |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462321 |
0000349821 |
recurrent sinusitis |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462322 |
0000349822 |
sinopulmonary infections (including chronic otorrhea) from early infancy with ultrastructural defects in the cilia (significant microtubular disorganizations, including distorted dynein arms, absent inner dynein arms) |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462323 |
0000349823 |
Joubert syndrome |
primary ciliary dyskinesia |
JBTS25 |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462324 |
0000349824 |
bronchiectasis, chronic sinusitis; mother recurrent abortions, ectopic pregnancy |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462325 |
0000349825 |
chronic respiratory infections, ultrastructural defects cilia (significant microtubular disorganizations, including distorted outer dynein arms) |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462326 |
0000349826 |
primary ciliary dyskinesia |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462327 |
0000349827 |
recurrent respiratory infections from childhood,non-motile sperms |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462328 |
0000349828 |
chronic sinusitis |
primary ciliary dyskinesia |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00462329 |