{"orpha":675,"name":"Annular pancreas","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A distinct form of duodenal atresia in which the head of the pancreas forms a ring around the second portion of the duodenum.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-675/","orphanet_url":"https://www.orpha.net/en/disease/detail/675","names":{"fr":{"name":"Pancréas annulaire","definition":"Forme distincte d'atrésie duodénale dans laquelle la tête du pancréas forme un anneau autour du deuxième duodénum."},"de":{"name":"Pankreas anularis","definition":"Das Pancreas anulare (PA) ist eine distinkte Form der Duodenalatresie. Das Pankreas bildet dabei einen Ring um den distalen Anteil des Duodenums."},"es":{"name":"Páncreas anular","definition":"Es una forma de atresia duodenal en la cual la cabeza del páncreas forma un anillo alrededor de la segunda porción del duodeno."},"it":{"name":"Pancreas ad anello"},"nl":{"name":"Annulaire pancreas","synonyms":["Pancreas annulare","Ringvormige alvleesklier"],"definition":"Een aparte vorm van duodenumatresie, waarbij de kop van de pancreas een ring vormt rond het tweede deel van het duodenum."},"pt":{"name":"Pâncreas anular"},"pl":{"name":"Trzustka obrączkowata"},"cs":{"name":"Anulární pankreas"},"uk":{"name":"Кільцева підшлункова залоза","definition":"A distinct form of duodenal atresia in which the head of the pancreas forms a ring around the second portion of the duodenum."},"zh":{"name":"环状胰腺"},"tr":{"name":"Dairesel pankreas"}},"xrefs":[{"source":"OMIM","code":"167750","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"Q45.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LB21.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1311285827"},{"source":"MONDO","code":"0008183","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C536376","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0149955","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"705","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10071757","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":108971,"name":"Non-syndromic visceral malformation of the liver, biliary tract, pancreas or spleen","classifications":[147,156,182,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant","Not applicable"],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 100 000","value":1.8,"area":"Europe","status":"Validated","source":"[EXPERT]_EUROCAT European surveillance of congenital anomalies 2010[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-5 / 10 000","value":29.4,"area":"Austria","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2009[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 100 000","value":2.9,"area":"Belgium","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2011[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-5 / 10 000","value":21.3,"area":"Denmark","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2011[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 100 000","value":7.9,"area":"France","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2011[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 100 000","value":5.9,"area":"Germany","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2011[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 100 000","value":1.0,"area":"Hungary","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2009[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 100 000","value":3.6,"area":"Ireland","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2011[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 100 000","value":5.7,"area":"Italy","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2011[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-5 / 10 000","value":24.9,"area":"Malta","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2010[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-5 / 10 000","value":17.1,"area":"Netherlands","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2010[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.7,"area":"Poland","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2010[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 100 000","value":3.1,"area":"Spain","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2010[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.8,"area":"United Kingdom","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2010[OTHER]"},{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 100 000","value":3.2,"area":"Ukraine","status":"Validated","source":"EUROCAT European surveillance of congenital anomalies 2010[OTHER]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":4.5,"area":"Worldwide","status":"Validated","source":"38310080[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0001734","label":"Annular pancreas","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100867","label":"Duodenal stenosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005250","label":"High intestinal obstruction","frequency":"Frequent (79-30%)","criteria":null}]}}