holoprosencefalie [Holoprosencephaly]

tematický
9
Termíny

alobární holoprosencefalie
alobární holoprozencefalie
arhinencefalie
arinencefalie
holoprosencefalie lobárního typu
holoprozencefalie
lobární holoprosencefalie
lobární holoprozencefalie
semilobární holoprosencefalie
semilobární holoprozencefalie

 

Alobar Holoprosencephaly
Arhinencephaly
Holoprosencephaly, Familial Alobar
Lobar Holoprosencephaly
Semilobar Holoprosencephaly

Perzistentní odkaz   https://www.medvik.cz/link/D016142
Definice

Vývojová vada charakterizovaná chyběním středových kraniofaciálních struktur, která má variabilní expresivitu (je přítomen pouze jeden přední zub, jedna společná dutina nosní, jedno oko (kyklopismus); splynutí mozkových hemisfér (alobární holoprosencefalie); nebo chybí bulbus a tractus olfactorius a corpus callosum. (cit. Wikiskripta 2015 http://www.wikiskripta.eu/)

Anterior midline brain, cranial, and facial malformations resulting from the failure of the embryonic prosencephalon to undergo segmentation and cleavage. Alobar prosencephaly is the most severe form and features anophthalmia; cyclopia; severe INTELLECTUAL DISABILITY; CLEFT LIP; CLEFT PALATE; SEIZURES; and microcephaly. Semilobar holoprosencepaly is characterized by hypotelorism, microphthalmia, coloboma, nasal malformations, and variable degrees of INTELLECTUAL DISABILITY. Lobar holoprosencephaly is associated with mild (or absent) facial malformations and intellectual abilities that range from mild INTELLECTUAL DISABILITY to normal. Holoprosencephaly is associated with CHROMOSOME ABNORMALITIES.

DUI
D016142 MeSH Prohlížeč
CUI
M0024659
Předchozí užití
Abnormalities, Multiple (1966-1990); Brain/abnormalities (1966-1990)
Historická pozn.
91
Veřejná pozn.
91

C Nemoci
C05.660.207.103 syndrom delece 22q11 2
C05.660.207.219 Costellův syndrom 3
C05.660.207.240 kraniosynostózy 56
C05.660.207.325 Donohueův syndrom 1
C05.660.207.410 holoprosencefalie 9
C05.660.207.532 Loeysův-Dietzův syndrom 7
C05.660.207.536 megalencefalie 7
C05.660.207.620 mikrocefalie 78
C05.660.207.690 Noonanové syndrom 52
C05.660.207.707 plagiocefalie 3
C05.660.207.720 platybazie 12
C10.500.034.500 syndrom corpus callosum 1
C10.500.034.687 Aicardiho syndrom
C10.500.034.875 holoprosencefalie 9
C10.500.034.937 septo-optická dysplazie 15
C16.131 vrozené vady 1 753
C16.131.077.019 syndrom delece 22q11 2
C16.131.077.065 Alagillův syndrom 19
C16.131.077.095 Angelmanův syndrom 35
C16.131.077.121 Barthův syndrom 6
C16.131.077.137 Bloomův syndrom 11
C16.131.077.229 Carneyův komplex 8
C16.131.077.245 ciliopatie 9
C16.131.077.250 Cockayneův syndrom 4
C16.131.077.256 Costellův syndrom 3
C16.131.077.272 de Langeové syndrom 4
C16.131.077.299 hluchoslepota 65
C16.131.077.313 Donohueův syndrom 1
C16.131.077.327 Downův syndrom 510
C16.131.077.350 ektodermální dysplazie 33
C16.131.077.371 Fraserův syndrom 2
C16.131.077.393 Gardnerův syndrom 21
C16.131.077.401 heterotaxe 7
C16.131.077.410 holoprosencefalie 9
C16.131.077.445 incontinentia pigmenti 19
C16.131.077.537 Loeysův-Dietzův syndrom 7
C16.131.077.550 Marfanův syndrom 105
C16.131.077.578 Moebiův syndrom 10
C16.131.077.592 moniletrichosis 1
C16.131.077.619 Nethertonův syndrom 2
C16.131.077.696 Cantrellova pentalogie 1
C16.131.077.703 POEMS syndrom 35
C16.131.077.730 Praderův-Williho syndrom 92
C16.131.077.735 nedostatek prolidázy
C16.131.077.740 Proteův syndrom 8
C16.131.077.745 prune belly syndrom 3
C16.131.077.790 zarděnky vrozené 20
C16.131.077.889 Sotosův syndrom 4
C16.131.077.919 Patauův syndrom 6
C16.131.077.929 syndrom trizomie 18 5
C16.131.077.938 Waardenburgův syndrom 10
C16.131.077.970 Zellwegerův syndrom 10
C16.131.260.019 syndrom delece 22q11 2
C16.131.260.040 Angelmanův syndrom 35
C16.131.260.210 de Langeové syndrom 4
C16.131.260.260 Downův syndrom 510
C16.131.260.380 holoprosencefalie 9
C16.131.260.440 Jacobsenův syndrom 1
C16.131.260.700 Praderův-Williho syndrom 92
C16.131.260.905 Sotosův syndrom 4
C16.131.260.923 Patauův syndrom 6
C16.131.260.932 syndrom trizomie 18 5
C16.131.260.940 syndrom WAGR 3
C16.131.621.207.103 syndrom delece 22q11 2
C16.131.621.207.207 kleidokraniální dysplazie 7
C16.131.621.207.231 kraniofaciální dysostóza 28
C16.131.621.207.240 kraniosynostózy 56
C16.131.621.207.410 holoprosencefalie 9
C16.131.621.207.532 megalencefalie 7
C16.131.621.207.540 maxilofaciální abnormality 28
C16.131.621.207.620 mikrocefalie 78
C16.131.621.207.690 Noonanové syndrom 52
C16.131.621.207.700 orofaciodigitální syndromy 7
C16.131.621.207.707 plagiocefalie 3
C16.131.621.207.720 platybazie 12
C16.131.621.207.850 Rubinsteinův-Taybiho syndrom 12
C16.131.666.034 ageneze corpus callosum 12
C16.131.666.034.500 syndrom corpus callosum 1
C16.131.666.034.687 Aicardiho syndrom
C16.131.666.034.875 holoprosencefalie 9
C16.131.666.034.937 septo-optická dysplazie 15
C16.320.180.019 syndrom delece 22q11 2
C16.320.180.040 Angelmanův syndrom 35
C16.320.180.210 de Langeové syndrom 4
C16.320.180.260 Downův syndrom 510
C16.320.180.380 holoprosencefalie 9
C16.320.180.440 Jacobsenův syndrom 1
C16.320.180.700 Praderův-Williho syndrom 92
C16.320.180.905 Sotosův syndrom 4
C16.320.180.923 Patauův syndrom 6
C16.320.180.932 syndrom trizomie 18 5
C16.320.180.940 syndrom WAGR 3

Dysgnathia complex Disease MeSH Prohlížeč

Genoa syndrome Disease MeSH Prohlížeč

Holoprosencephaly 10 Disease MeSH Prohlížeč

Holoprosencephaly 2 Disease MeSH Prohlížeč

Holoprosencephaly 3 Disease MeSH Prohlížeč

Holoprosencephaly 4 Disease MeSH Prohlížeč

Holoprosencephaly 5 Disease MeSH Prohlížeč

Holoprosencephaly 6 Disease MeSH Prohlížeč

Holoprosencephaly 7 Disease MeSH Prohlížeč

Holoprosencephaly 8 Disease MeSH Prohlížeč

Holoprosencephaly 9 Disease MeSH Prohlížeč

Holoprosencephaly with Fetal Akinesia-Hypokinesia Sequence Disease MeSH Prohlížeč

Holoprosencephaly, Ectrodactyly, and Bilateral Cleft Lip-Palate Disease MeSH Prohlížeč

Holoprosencephaly, recurrent infections, and monocytosis Disease MeSH Prohlížeč

Lambotte syndrome Disease MeSH Prohlížeč

Microgastria limb reduction defect Disease MeSH Prohlížeč

Nonsyndromic Holoprosencephaly Disease MeSH Prohlížeč

Pseudotrisomy 13 syndrome Disease MeSH Prohlížeč

Steinfeld Syndrome Disease MeSH Prohlížeč

cyclopia sequence Disease MeSH Prohlížeč