About: Otocephaly

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Otocephaly, also known as agnathia–otocephaly complex, is a very rare and lethal cephalic disorder characterized by the absence of the mandible (agnathia), with the ears fused together just below the chin (synotia). It is caused by a disruption to the development of the first branchial arch. It occurs in every 1 in 70,000 embryos.

Attributes	Values
rdf:type	Thing Disease yago:WikicatCongenitalDisordersOfEye,Ear,FaceAndNeck yago:WikicatNeurologicalDisorders yago:Abnormality114501726 yago:Abstraction100002137 yago:Anomaly114505821 yago:Attribute100024264 yago:BirthDefect114465048 yago:Condition113920835 yago:Defect114464005 yago:Disease114070360 yago:Disorder114052403 yago:IllHealth114052046 yago:Illness114061805 yago:Imperfection114462666 yago:NervousDisorder114084880 yago:PathologicalState114051917 yago:PhysicalCondition114034177 disease yago:State100024720 umbel-rc:AilmentCondition
rdfs:label	اتحاد الأذنين (ar) Otozephalie (de) Otocefalia (es) Otocéphalie (fr) Otocephaly (en) Octocefalia (pt)
rdfs:comment	اتحاد الأذنين Otocephaly (من اليونانية عبارة οτο ، وهي تعني «الأذن»، و " κεφάλη "، وهي تعني «الرأس») هو نوع من الاضطراب في الرأس. هذه هي حالة فتاكة فيها المميز الرئيسي الغياب التام أو الظاهري فك السفلي (شذوذ إنمائي يسمى انعدام الفك).و"oto" في الاسم يشير إلى العلاقة بين الأذنين على الوجه في هذا الاضطراب. تعتبر حالة قاتلة بسبب سوء سير عمل مجرى الهواء. في اتحاد الأذنين، قد يحدث انعدام الفك بمفرده أو مع اندماج مقدم الدماغ. (ar) Die Otozephalie (von altgriechisch ὄτο oto, deutsch ‚Ohr‘, und κέφαλος kephalos, deutsch ‚Kopf‘) ist eine sehr seltene angeborene, häufig letale Fehlbildung des Gesichtsschädels, bei welcher der Unterkiefer vollständig oder teilweise nicht angelegt ist (Agnathie). Häufig besteht eine Kombination mit einer Holoprosencephalie und einem Situs inversus. Die Erstbeschreibung stammt aus dem Jahre 1983 durch die US-amerikanischen Ärzte Richard M. Pauli und Mitarbeiter. (de) La Otocefalia es un trastorno letal, cuya característica principal es la agnacia o agnatia, la cual se refiere a una anomalía del desarrollo embrionario caracterizada por la ausencia total o virtual de la mandíbula inferior. Esta patología se considera incompatible con la vida o letal debido al mal funcionamiento de la vía respiratoria. En la otocefalia, la agnacia puede ocurrir en forma independiente o en conjunto con la holoprosencefalia. (es) Otocephaly, also known as agnathia–otocephaly complex, is a very rare and lethal cephalic disorder characterized by the absence of the mandible (agnathia), with the ears fused together just below the chin (synotia). It is caused by a disruption to the development of the first branchial arch. It occurs in every 1 in 70,000 embryos. (en) L'otocéphalie est une pathologie congénitale caractérisée par une non-migration (durant l'embryogenèse) des oreilles en position crâniale. Celles-ci restent donc en position caudale, c'est-à-dire au niveau des joues, ou selon les cas, au centre du bas du visage. Il est par ailleurs très souvent remarqué qu'en plus de cette non-migration des oreilles, il n'existe qu'un seul œil central chez l'enfant (cyclope).C'est une maladie relativement rare, qui relève du domaine de la tératologie. * Portail de la médecine (fr) A Octocefalia é um trastorno letal, cuja característica principal é a uma anomalia do desenvolvimento caracterizada pela ausência total ou virtual da mandíbula inferior. Considera-se letal devido ao mau funcionamento da via respiratória. Na octocefalia, a agnasia pode ocorrer em forma independente ou em conjunto com a holoprosencefalia. (pt)
foaf:name	Otocephaly (en)
name	Otocephaly (en)
foaf:depiction
dcterms:subject	Congenital disorders of eye, ear, face and neck
Wikipage page ID	3720089 (xsd:integer)
Wikipage revision ID	1119304319 (xsd:integer)
Link from a Wikipage to another Wikipage	Proboscis (anomaly) Encephalocele Enteral administration History of medicine Cyclopia Viscera De novo mutation Intrauterine growth restriction Medical ultrasound Oral and maxillofacial surgery Cleft lip Cleft palate Greek language Miscarriage Theodor Kerckring Stillbirth Treacher Collins syndrome Medical genetics Micrognathism Microstomia Agenesis of the corpus callosum Tracheal intubation Anencephaly Chromosome 1 Goldenhar syndrome Premature birth Guinea pig Hypoplasia Ectopic kidney Holoprosencephaly Congenital disorders of eye, ear, face and neck Pierre Robin sequence Animal embryonic development Mesencephalon Micrognathia Neural tube defect Organ transplantation Cephalic disorder Sewall Wright Mandible Situs inversus New Latin Gestation Möbius syndrome Pharyngeal arch XK aprosencephaly Reconstructive surgery PRRX1 Autosomal trisomy Carnegie stage First branchial arch
Link from a Wikipage to an external page	https://web.archive.org/web/20080920160408/http:/www.ninds.nih.gov/disorders/cephalic_disorders/detail_cephalic_disorders.htm
sameAs	Otocephaly Otocephaly Otocephaly Otocephaly

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