{"orpha":97282,"name":"VIPoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Diarrheogenic islet cell tumor","Pancreatic cholera","VIP-secreting tumor","Verner-Morrison syndrome","WDHA syndrome","Watery diarrhea-hypokalemia-achlorhydria syndrome"],"definition":"VIPoma is an extremely rare type of pancreatic neuroendocrine tumor that secretes vasoactive intestinal polypeptide (VIP) leading to the manifestations of watery diarrhea, hypokalemia and achlorhydia or hypochhlorhydia (known as WDHA syndrome).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-97282/","orphanet_url":"https://www.orpha.net/en/disease/detail/97282","names":{"fr":{"name":"VIPome","synonyms":["Choléra endocrine","Choléra endocrinien","Choléra pancréatique","Syndrome WDHA","Syndrome de Verner-Morrison","Syndrome de diarrhée aqueuse avec hypokaliémie et achlorhydrie","Tumeur sécrétant du VIP"],"definition":"Type extrêmement rare de tumeur neuroendocrine pancréatique, sécrétant du peptide vasoactif intestinal (VIP), provoquant des manifestations d'une diarrhée aqueuse, d'une hypokaliémie et d'une achlorhydrie ou d'une hypochlorhydrie (connu sous le nom de syndrome WDHA)."},"de":{"name":"VIPom","synonyms":["Pankreatische Cholera","VIP-produzierender Tumor","Verner-Morrison-Syndrom","WDHA-Syndrom","Wässrige Durchfälle - Hypokaliämie - Achlorhydrie"]},"es":{"name":"VIPoma","synonyms":["Cólera pancreático","Diarrea acuosa-hipopotasemia-aclorhidria","Síndrome WDHA","Síndrome de Verner-Morrison","Tumor diarreogénico de las células de los islotes","Tumor secretor de VIP"],"definition":"El VIPoma es un tumor pancreático neuroendocrino extremadamente raro (consulte este término) que secreta el polipéptido intestinal vasoactivo (VIP) conduciendo a las manifestaciones de diarrea acuosa, hipopotasemia y aclorhidria o hipoclorhidria (conocido como el síndrome WDHA)."},"it":{"name":"VIPoma","synonyms":["Colera pancreatico","Diarrea acquosa - ipokaliemia - acloridria","Sindrome WDHA","Sindrome di Verner-Morrison","Tumore diarreogenico delle cellule degli isolotti","Tumore secernente VIP"],"definition":"Il VIPoma è un tumore neuroendocrino pancreatico (si veda questo termine) molto raro che secerne il polipeptide intestinale vasoattivo (VIP), che causa caratteristicamente diarrea acquosa, ipokaliemia e acloridria o ipocloridria (nota come sindrome WDHA)."},"nl":{"name":"VIPoom","synonyms":["Diarreeveroorzakende tumor van de eilandjes van Langerhans","Pancreatische cholera","Syndroom van Verner-Morrison","VIP-secreterende tumor","WDHA-syndroom","Waterige diarree - hypokaliëmie - achloorhydrie-syndroom"],"definition":"Een extreem zeldzaam type van neuro-endocriene tumor van pancreas die vasoactief intestinaal polypeptide (VIP) afscheidt, wat leidt tot manifestaties van waterige diarree, hypokaliëmie en achloorhydrie of hypochloorhydrie (bekend als WDHA-syndroom)."},"pt":{"name":"VIPoma"},"pl":{"name":"VIPoma","synonyms":["Cholera trzustkowa","Guz wydzielający VIP","Wodnista biegunka - hipokalemia - bezkwaśność","Zespół Vernera i Morrisona","Zespół WDHA"]},"cs":{"name":"VIPom","synonyms":["Pankreatická cholera","Nádor produkující VIP","WDHA syndrom","Diareogenní nádor ostrůvkových buněk","Vernerův-Morrisonův syndrom","Syndrom zahrnující vodnatý průjem, hypokalémii a achlorhydrii","Verner-Morrisonův syndrom"]},"uk":{"name":"VIPoma","synonyms":["Діареогенна острівцево-клітинна пухлина","Панкреатична холера","Синдром Вернера-Моррісона","VIP-секретуюча пухлина","Синдром водянистої діареї-гіпокаліємії-ахлоргідрії","Синдром WDHA"],"definition":"VIPoma is an extremely rare type of pancreatic neuroendocrine tumor (see this term) that secretes vasoactive intestinal polypeptide (VIP) leading to the manifestations of watery diarrhea, hypokalemia and achlorhydia or hypochhlorhydia (known as WDHA syndrome)."},"zh":{"name":"胰腺瘤","synonyms":["水样泻-低钾血症-胃酸缺乏综合征"],"definition":"血管活性肠肽瘤(VIPoma)是一种极为罕见的胰腺神经内分泌肿瘤（见该词条），它分泌血管活性肠肽（VIP），导致水样腹泻、低钾血症和低氯尿症（WDHA综合征）。"},"tr":{"name":"VIPom","synonyms":["WDHA sendromu","Pankreatik kolera","VIP-salgılayan tümör","Verner-Morrison sendromu","Diyareojenik adacık hücre tümörü","Sulu diyare-hipokalemi-aklorhidri sendromu"]}},"xrefs":[{"source":"ICD-10","code":"D37.7","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"E16.8","qualifier":"ND","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C10.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"20634476"},{"source":"ICD-11","code":"XH72E5","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1712002145"},{"source":"MONDO","code":"0019960","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D003969","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0011993","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"3787","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10047430","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":506060,"name":"Functioning neuroendocrine tumor of pancreas","classifications":[152,193,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-02-06","source":"13492596[PMID]_10834901[PMID]_15317894[PMID]_23993192[PMID]_22997445[PMID]_16416161[PMID]_22261872[PMID]_23582916[PMID]_18798544[PMID]_10940685[PMID]_2440390[PMID]_18941127[PMID]_22052063[PMID]","terms":[{"hpo":"HP:0002900","label":"Hypokalemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002894","label":"Neoplasm of the pancreas","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005208","label":"Secretory diarrhea","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002039","label":"Anorexia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012432","label":"Chronic fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001944","label":"Dehydration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000819","label":"Diabetes mellitus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002574","label":"Episodic abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003324","label":"Generalized muscle weakness","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003072","label":"Hypercalcemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002024","label":"Malabsorption","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003394","label":"Muscle spasm","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001895","label":"Normochromic anemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004396","label":"Poor appetite","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030895","label":"Abnormal gastrointestinal motility","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001438","label":"Abnormality of abdomen morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001541","label":"Ascites","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012334","label":"Extrahepatic cholestasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001046","label":"Intermittent jaundice","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001406","label":"Intrahepatic cholestasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000820","label":"Abnormality of the thyroid gland","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0008256","label":"Adrenocortical adenoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0006719","label":"Benign gastrointestinal tract tumors","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003528","label":"Elevated calcitonin","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000845","label":"Elevated circulating growth hormone concentration","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0006731","label":"Follicular thyroid carcinoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003005","label":"Ganglioneuroma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003118","label":"Increased circulating cortisol level","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000837","label":"Increased circulating gonadotropin level","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000870","label":"Increased circulating prolactin concentration","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002896","label":"Neoplasm of the liver","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002897","label":"Parathyroid adenoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002893","label":"Pituitary adenoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0008200","label":"Primary hyperparathyroidism","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002747","label":"Respiratory insufficiency due to muscle weakness","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001031","label":"Subcutaneous lipoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002573","label":"Hematochezia","frequency":"Excluded (0%)","criteria":null}]}}