{"orpha":400,"name":"Cystic echinococcosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Hydatid cyst","Hydatid disease","Hydatidosis"],"definition":"A rare parasitic disease characterized by slowly enlarging cysts of the liver, lungs and other organs which often go unnoticed for years. They are caused principally by the larval form (also called metacestode) of Echinococcus granulosus sensu lato tapeworm. The adult form of this tapeworm parasitizes the intestine of dogs. Cystic echinococcosis (formerly hydatidosis) generally affects large domestic herbivores and humans are dead-end hosts, infected through contact with herding dogs or through ingestion of food contaminated with canine excrement.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-400/","orphanet_url":"https://www.orpha.net/en/disease/detail/400","names":{"fr":{"name":"Echinococcose kystique","synonyms":["Hydatidose","Kyste hydatique","Maladie hydatique"],"definition":"Maladie parasitaire rare, caractérisée des kystes de croissance lente (au niveau du foie, poumons et autres organes), restés souvent non diagnostiqués durant des années. Ces kystes correspondent à la forme larvaire (ou métacestode) du ver Echinococcus granulosus sensu lato. La forme adulte du parasite est présente dans l'intestin des chiens. L'echinococcose kystique (anciennement hydatidose) affecte généralement les grands herbivores domestiques et les humains (qui sont une impasse parasitaire), infectés par contact avec des chiens de troupeau ou par ingestion d'aliments contaminés par des excréments canins."},"de":{"name":"Zystische Echinokokkose","synonyms":["Hydatidenzyste","Hydatidose"],"definition":"Eine seltene parasitäre Erkrankung, die durch langsam wachsende Zysten in Leber, Lunge und anderen Organen gekennzeichnet ist, die oft jahrelang unbemerkt bleiben. Sie werden hauptsächlich durch die Larvenform (auch Metacestode genannt) des kleinen Hundebandwurms <i>Echinococcus granulosus sensu lato</i> verursacht. Die erwachsene Form dieses Bandwurms parasitiert im Darm von Hunden. Die zystische Echinokokkose (früher Hydatidose) befällt in der Regel große pflanzenfressende Haustiere, und der Mensch ist ein Endwirt, der sich durch den Kontakt mit Hütehunden oder durch die Aufnahme von mit Hundekot kontaminierten Lebensmitteln infiziert."},"es":{"name":"Equinococosis quística","synonyms":["Enfermedad hidatídica","Hidatidosis","Quiste hidatídico"],"definition":"Es una enfermedad parasitaria poco frecuente caracterizada por quistes de crecimiento lento en el hígado, los pulmones y otros órganos, y que a menudo pasan desapercibidos durante años. Están causados principalmente por la forma larvaria (también llamada metacestodo) de la tenia <i>Echinococcus granulosus sensu lato</i>. La forma adulta de esta tenia parasita el intestino de los perros. La equinococosis quística (anteriormente hidatidosis) suele afectar a grandes herbívoros domésticos y los humanos son los hospedadores finales, infectados por contacto con perros pastores o por ingestión de alimentos contaminados con excrementos caninos."},"it":{"name":"Echinococcosi cistica","synonyms":["Idatidosi","Malattia idatidea","Cisti idatidea"]},"nl":{"name":"Cystische echinokokkose","synonyms":["Cysteuze echinokokkose","Hydatidencyste","Hydatidenziekte","Hydatidose","Hydatidosis"],"definition":"Een zeldzame parasitaire aandoening, gekenmerkt door traag groeiende cysten van lever, long en andere organen die vaak jarenlang onopgemerkt blijven. Ze worden vooral veroorzaakt door larven (ook gekend als metacestode) van <i>Echinococcus granulosus sensu lato</i> lintwormen. De volwassen vorm van deze lintworm parasiteert de darmen van honden. Cystische echinokokkose (voorheen hydatidose) treft doorgaans grote huiselijke herbivoren; mensen zijn doodlopende gastheren die geïnfecteerd raken door contact met een herdershond of inname van voedsel dat besmet werd door uitwerpselen van honden."},"pt":{"name":"Equinococose quística","synonyms":["Doença hidática","Hidatidose","Quisto hidático"],"definition":"Doença parasitária rara caracterizada por crescimento lento de quistos no fígado, pulmões e outros órgãos, que muitas vezes passam despercebidos durante anos. São causadas principalmente pela forma larvar (também chamada metacestódeo) da ténia Echinococcus granulosus sensu lato. A forma adulta desta ténia parasita o intestino dos cães. A equinococose quística (anteriormente hidatidose) afeta geralmente os grandes herbívoros domésticos e os humanos são hospedeiros finais, infetados pelo contacto com cães pastores ou pela ingestão de alimentos contaminados com excrementos caninos."},"pl":{"name":"Bąblowica","synonyms":["Hydatid cyst","Hydatid disease","Hydatidosis"]},"cs":{"name":"Cystická echinokokóza","synonyms":["Hydatidová nemoc","Hydatidóza"]},"uk":{"name":"Кістозний ехінококоз","synonyms":["Кіста гідатиди","Хвороба Гідатіда","Гідратидоз"],"definition":"A rare parasitic disease characterized by slowly enlarging cysts of the liver, lungs and other organs which often go unnoticed for years. They are caused principally by the larval form (also called metacestode) of Echinococcus granulosus sensu lato tapeworm. The adult form of this tapeworm parasitizes the intestine of dogs. Cystic echinococcosis (formerly hydatidosis) generally affects large domestic herbivores and humans are dead-end hosts, infected through contact with herding dogs or through ingestion of food contaminated with canine excrement."},"zh":{"name":"囊型包虫病","synonyms":["棘球蚴病"]},"tr":{"name":"Kistik ekinokokkoz","synonyms":["Hidatidoz","Hidatik hastalığı"]}},"xrefs":[{"source":"ICD-10","code":"B67.0","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B67.1","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B67.2","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B67.3","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B67.4","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B67.5","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B67.6","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B67.7","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B67.8","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B67.9","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"1F73.Z","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1456802165"},{"source":"MONDO","code":"0018408","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D004443","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4553297","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"2764","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10014096","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":163588,"name":"Rare parasitic disease","classifications":[203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.0,"area":"Europe","status":"Validated","source":"[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2020-04-15","source":"26677245[PMID]_14575976[PMID]","terms":[{"hpo":"HP:0001407","label":"Hepatic cysts","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001880","label":"Increased total eosinophil count","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002611","label":"Cholestatic liver disease","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0410019","label":"Epigastric pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010702","label":"Increased circulating immunoglobulin concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011458","label":"Abdominal symptom","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031983","label":"Abnormal pulmonary thoracic imaging finding","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000775","label":"Abnormality of the diaphragm","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025615","label":"Abscess","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100845","label":"Anaphylactic shock","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005230","label":"Biliary tract obstruction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003155","label":"Elevated circulating alkaline phosphatase concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002910","label":"Elevated circulating hepatic transaminase concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030948","label":"Elevated gamma-glutamyltransferase level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002904","label":"Hyperbilirubinemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005948","label":"Multiple pulmonary cysts","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032445","label":"Pulmonary cyst","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032101","label":"Unusual infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001627","label":"Abnormal heart morphology","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031630","label":"Abnormal subpleural morphology","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000478","label":"Abnormality of the eye","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003011","label":"Abnormality of the musculature","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001732","label":"Abnormality of the pancreas","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002585","label":"Abnormality of the peritoneum","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0045058","label":"Abnormality of the testis size","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000925","label":"Abnormality of the vertebral column","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002099","label":"Asthma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012062","label":"Bone cyst","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0010576","label":"Intracranial cystic lesion","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031700","label":"Invasive parasitic infection","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000952","label":"Jaundice","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0011355","label":"Localized skin lesion","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012578","label":"Membranous nephropathy","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000138","label":"Ovarian cyst","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100592","label":"Peritoneal abscess","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000107","label":"Renal cyst","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0030423","label":"Splenic cyst","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001025","label":"Urticaria","frequency":"Very rare (<4-1%)","criteria":null}]}}