{"orpha":103918,"name":"Tropical pancreatitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["TCP","Tropical calcific chronic pancreatitis"],"definition":"A rare pancreatic disease of juvenile onset occurring mainly in tropical developing countries and characterized by chronic non-alcoholic pancreatitis manifesting with abdominal pain, steatorrhea and fibrocalculous pancreatopathy. It is also commonly associated with the development of pancreatic calculi and pancreatic cancer at a much higher frequency than seen in ordinary chronic pancreatitis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-103918/","orphanet_url":"https://www.orpha.net/en/disease/detail/103918","names":{"fr":{"name":"Pancréatite tropicale","synonyms":["Pancréatite tropicale calcifiante chronique","TCP"],"definition":"Maladie pancréatique juvénile rare qui se retrouve principalement dans les pays tropicaux en voie de développement. Elle est caractérisée par une pancréatite chronique non-alcoolique se manifestant par une douleur abdominale, une stéatorrhée et une pancréatopathie fibro-calculeuse. Elle est aussi souvent associée au développement de calculs pancréatiques et au cancer du pancréas à une fréquence beaucoup plus élevée que celle rapportée pour la pancréatite chronique ordinaire."},"de":{"name":"Pankreatitis, tropische","synonyms":["Pankreatitis, chronische kalzifizierende tropische","TCP"],"definition":"Eine seltene Krankheit der Bauchspeicheldrüse, die vor allem in tropischen Entwicklungsländern auftritt, im Jugendalter beginnt und durch eine chronische nichtalkoholische Pankreatitis gekennzeichnet ist, die sich durch Bauchschmerzen, Steatorrhoe und eine fibrokalzinöse Pankreatopathie äußert. Sie ist auch häufig mit der Entwicklung von Pankreassteinen und Bauchspeicheldrüsenkrebs verbunden, und zwar mit einer viel höheren Inzidenz als bei der gewöhnlichen chronischen Pankreatitis."},"es":{"name":"Pancreatitis tropical","synonyms":["Pancreatitis crónica tropical calcificante","TCP"],"definition":"Es una enfermedad pancreática poco frecuente de aparición juvenil que se da principalmente en países tropicales en desarrollo y se caracteriza por una pancreatitis no alcohólica crónica que se manifiesta con dolor abdominal, esteatorrea y pancreopatía fibrocalculosa (consulte estos términos). Está con asiduidad asociada con el desarrollo de cálculos pancreáticos y cáncer pancreático, con una frecuencia mayor a la observada en la pancreatitis crónica común."},"it":{"name":"Pancreatite tropicale","synonyms":["Pancreatite cronica calcificante tropicale","TCP"],"definition":"La pancreatite tropicale è una malattia rara del pancreas, a esordio giovanile, che si manifesta, in particolare, nei paesi tropicali in via di sviluppo, e si caratterizza per una pancreatite non alcolica cronica, che si manifesta con dolore addominale, steatorrea e una patologia fibrocalcolosa del pancreas (si veda questo termine). Si associa di solito allo sviluppo di calcoli pancreatici e al tumore del pancreas, che hanno una frequenza maggiore rispetto a quella che si osserva nella pancreatite cronica tradizionale."},"nl":{"name":"Tropische pancreatitis","synonyms":["TCP","Tropische kalkvormende chronische pancreatitis"],"definition":"Een zeldzame aandoening van pancreas met juveniele aanvang, vooral aanwezig in tropische ontwikkelingslanden, en gekenmerkt door chronische niet-alcoholische pancreatitis die zich manifesteert met abdominale pijn, steatorroe en fibrocalculeuze pancreatopathie. Het is doorgaans ook geassocieerd met ontwikkeling van calculi en kanker van pancreas met veel hogere frequentie in vergelijking met gewone chronische pancreatitis."},"pt":{"name":"Pancreatite tropical"},"pl":{"name":"Tropikalne zapalenie trzustki","synonyms":["TCP","Tropikalne wapniejące przewlekłe zapalenie trzustki"]},"cs":{"name":"Tropická pankreatitida","synonyms":["TCP","Tropická kalcifikující chronická pankreatitida"]},"uk":{"name":"Тропічний панкреатит","synonyms":["TCP","Тропічний кальцієвий хронічний панкреатит"],"definition":"A rare pancreatic disease of juvenile onset occurring mainly in tropical developing countries and characterized by chronic non-alcoholic pancreatitis manifesting with abdominal pain, steatorrhea and fibrocalculous pancreatopathy. It is also commonly associated with the development of pancreatic calculi and pancreatic cancer at a much higher frequency than seen in ordinary chronic pancreatitis."},"zh":{"name":"热带性胰腺炎","synonyms":["热带钙化性慢性胰腺炎"],"definition":"热带胰腺炎是一种罕见的青少年胰脏疾病，主要发生在热带发展中国家，以慢性非酒精性胰腺炎为特征，表现为腹痛、脂肪溢和纤维结石性胰脏病(见该词条)。与普通慢性胰腺炎相比，它通常与胰脏结石和胰腺癌的发展密切相关。"},"tr":{"name":"Tropikal pankreatit","synonyms":["TCP","Tropikal kalsifik kronik pankreatit"]}},"xrefs":[{"source":"OMIM","code":"608189","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"K86.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DC32.5","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1645607956"},{"source":"MONDO","code":"0011986","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C564276","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1842402","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":99654,"name":"OBSOLETE: Fibrocalculous pancreatopathy","status":"inactive"}],"preferential_parent":{"orpha":97935,"name":"Rare gastroenterologic disease"},"parents":[{"orpha":101937,"name":"Rare pancreatic disease","classifications":[152]}],"children":[],"genes":[{"symbol":"CTRC","name":"chymotrypsin C","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Assessed","source":"22580415[PMID]_19404200[PMID]","hgnc":"2523","ensembl":"ENSG00000162438","omim":"601405","loci":["1p36.21"]},{"symbol":"SPINK1","name":"serine peptidase inhibitor Kazal type 1","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Assessed","source":"12360463[PMID]_12011155[PMID]","hgnc":"11244","ensembl":"ENSG00000164266","omim":"167790","loci":["5q32"]}],"genes_via_subtypes":[],"inheritance":[],"onset":["Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-11-17","source":"25516642[PMID]_30836443[PMID]_12011155[PMID]","terms":[{"hpo":"HP:0005236","label":"Chronic calcifying pancreatitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0410019","label":"Epigastric pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005213","label":"Pancreatic calcification","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030992","label":"Abnormal pancreatic duct morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008205","label":"Insulin-dependent but ketosis-resistant diabetes","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000952","label":"Jaundice","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004395","label":"Malnutrition","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0009800","label":"Maternal diabetes","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006725","label":"Pancreatic adenocarcinoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Occasional (29-5%)","criteria":null}]}}