{"orpha":357332,"name":"Syndactyly-camptodactyly and clinodactyly of fifth fingers-bifid toes syndrome","type":"Malformation syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["Synactyly-camptodactyly and clinodactyly of fifth fingers-bifid halluces syndrome","Wahab syndrome"],"definition":"A rare, genetic, congenital limb malformation syndrome characterized by a unique combination of bilateral, symmetrical camptodactyly and clinodactyly of 5th fingers, mesoaxial camptodactyly of toes, and ulnar deviation of 3rd fingers. Additional variable manifestations include bifid toes and severe syndactyly, or synpolydactyly, involving all digits of hands and feet.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-357332/","orphanet_url":"https://www.orpha.net/en/disease/detail/357332","names":{"fr":{"name":"Syndrome de syndactylie-camptodactylie et clinodactylie de l'auriculaire-gros orteils bifides","synonyms":["Syndrome de Wahab"],"definition":"Anomalie congénitale des membres d'origine génétique rare caractérisée par une combinaison unique de camptodactylie et de clinodactylie bilatérales et symétriques des 5<sup>es</sup> doigts, de camptodactylie mésoaxiale des orteils et de déviation ulnaire des 3<sup>es</sup> doigts. Les autres manifestations, inconstantes, incluent des orteils bifides et une syndactylie sévère, ou une synpolydactylie, affectant l'ensemble des doigts et des orteils."},"de":{"name":"Syndaktylie - Kamptodaktylie und Klinodaktylie des fünften Fingers - bifide Zehen","synonyms":["Wahab-Syndrom"]},"es":{"name":"Síndrome de sindactilia-camptodactilia y clinodactilia del quinto dedo de la mano-dedos de los pies bífidos","synonyms":["Síndrome de Wahab"],"definition":"Es un síndrome malformativo de las extremidades, congénito, genético y poco frecuente, caracterizado por una combinación única de camptodactilia y clinodactilia bilateral y simétrica de los quintos dedos de la mano, camptodactilia mesoaxial de los dedos de los pies y desviación cubital de los terceros dedos de la mano. Otros hallazgos variables observados incluyen dedos de los pies bífidos y grave sindactilia,o sinpolidactilia que afecta a todos los dedos de las manos y de los pies."},"it":{"name":"Sindattilia - camptodattilia e clinodattilia del quinto dito - dita dei piedi bifide","definition":"È una malformazione congenita rara degli arti, di origine genetica, caratterizzata dall'associazione tra camptodattilia simmetrica bilaterale e clinodattilia del quinto dito della mano, camptodattilia mesoassiale delle dita dei piedi e deviazione ulnare del terzo dito della mano. Altri segni clinici variabili sono le dita bifide e la sindattilia grave dei piedi, o la sinpolidattilia, che interessano tutte le dita delle mani e dei piedi."},"nl":{"name":"Syndactylie - camptodactylie en clinodactylie van vijfde vingers - gespleten tenen-syndroom","synonyms":["Syndactylie - camptodactylie en clinodactylie van vijfde vingers - gespleten halluces-syndroom","Syndroom van Wahab"],"definition":"Een zeldzaam, genetisch, congenitaal malformatiesyndroom van de ledematen dat gekarakteriseerd wordt door een unieke combinatie van bilaterale, symmetrische camptodactylie en clinodactylie van de vijfde vingers, mesoaxiale camptodactylie van tenen, en ulnaire deviatie van de derde vingers. Bijkomende variabele manifestaties zijn onder meer gespleten tenen en ernstige syndactylie, of synpolydactylie, met betrokkenheid van alle vingers en tenen."},"pt":{"name":"Síndrome de sindactilia-camptodactilia e clinodactilia do quinto dedo-dedos dos pés bífidos"},"pl":{"name":"Syndaktylia - kamptodaktylia i klinodaktylia piątych palców - rozszczep palców stóp","synonyms":["Syndaktylia - kamptodaktylia i klinodaktylia piątych palców - rozszczep dużych palców stóp","Zespół Wahaba"]},"cs":{"name":"Syndrom zahrnující syndaktylii, kamptodaktylii a klinodaktylii pátých prstů ruky a rozštěpení palce u nohy","synonyms":["Syndaktylie, kamptodaktylie a klinodaktylie pátých prstů ruky a bifidní palce","Wahabův syndrom"]},"uk":{"name":"Синдром синдактилії-камптодактилії та клінодактилії пятих пальців-роздвоєння пальців на ногах","synonyms":["Синдром синактилії-камптодактилії та клінодактилії пятих пальців - подвоєних великих пальців","Синдром Вахабу"],"definition":"A rare, genetic, congenital limb malformation syndrome characterized by a unique combination of bilateral, symmetrical camptodactyly and clinodactyly of 5th fingers, mesoaxial camptodactyly of toes, and ulnar deviation of 3rd fingers. Additional variable manifestations include bifid toes and severe syndactyly, or synpolydactyly, involving all digits of hands and feet."},"zh":{"name":"并指畸形-第五指屈曲和侧弯-双足趾综合征","synonyms":["Wahab综合征"],"definition":"本病是一种罕见的遗传性先天性肢体畸形综合征，其特征是第五指的双侧对称的上睑和斜指的独特组合，脚趾的中轴性上肢指和第三指的尺骨偏斜。其他的临床表现可包括脚趾分叉和严重并指或多指，累及手脚的所有手（趾）指。"},"tr":{"name":"Sindaktili-beşinci parmak kamptodaktili ve klinodaktilisi-bifid ayak sendromu","synonyms":["Beşinci parmak-bifid halüsis sendromunun sözdizimsel-kamptodaktili ve klinodaktili","Wahab sendromu"]}},"xrefs":[{"source":"OMIM","code":"615170","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"Q87.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0014069","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3554611","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":294959,"name":"Syndrome with limb duplication, polydactyly, syndactyly, and/or hyperphalangy","classifications":[147,156,199]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal recessive"],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":26.0,"area":"Worldwide","status":"Validated","source":"20683984[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}