{"orpha":90363,"name":"Secondary intestinal lymphangiectasia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Secondary intestinal lymphangiectasia is an acquired from of intestinal lymphangiectasia manifesting as a protein-losing enteropathy due to another disorder such as Crohn’s disease, congestive heart failure, sarcoidosis, Turner syndrome and often in patients who have undergone a Fontan operation. It is characterized by malabsorption, diarrhea, edema due hypoproteinemia, steatorrhea and serosal effusions.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90363/","orphanet_url":"https://www.orpha.net/en/disease/detail/90363","names":{"fr":{"name":"Lymphangiectasie intestinale secondaire","definition":"La Lymphangiectasie intestinale secondaire est une forme acquise de lymphangiectasie intestinale se manifestant par une entéropathie avec perte protéique causée par une autre maladie comme, une insuffisance cardiaque congestive, la maladie de Crohn, une sarcoïdose, le syndrome de Turner et souvent chez des patients qui ont subi une intervention de type Fontan. Elle est caractérisée par une malabsorption, une diarrhée, un oedème secondaire à une hypoprotéinémie, une stéatorrhée et des effusions séreuses."},"de":{"name":"Lymphangiektasie, intestinale sekundäre","definition":"Die Sekundäre intestinale Lymphangiektasie ist eine erworbene Form der Intestinalen Lymphangiektasie (s. dort). Sie äußert sich als Proteinverlust-Enteropathie im Gefolge anderer Krankheiten, darunter Morbus Crohn, Stauungs-Herzinsuffizienz, Sarkoidose und Turner-Syndrom (s. diese Termini). Oft tritt sie nach Fontan-Operation auf. Symptome sind Malabsorption, Diarrhoe, Ödeme durch Hypoproteinämie, Steatorrhoe und seröser Ausfluss."},"es":{"name":"Linfangiectasia intestinal secundaria","definition":"La linfangiectasia intestinal secundaria es una forma adquirida de la linfangiectasia intestinal (consulte este término), que se manifiesta como una enteropatía perdedora de proteínas debida a otro trastorno como la enfermedad de Crohn, una insuficiencia cardíaca congestiva, una sarcoidosis, el síndrome de Turner (consulte estos términos) y, con frecuencia, en pacientes que se han sometido a una operación de Fontan. Se caracteriza por malabsorción, diarrea, edema por hipoproteinemia, esteatorrea y efusiones serosas."},"it":{"name":"Linfangiectasia intestinale secondaria"},"nl":{"name":"Secundaire intestinale lymfangiëctasie","definition":"Een verworven vorm van intestinale lymfangiëctasie, zich manifesterend als een enteropathie met eiwitverlies te wijten aan een andere aandoening zoals ziekte van Crohn, congestief hartfalen, sarcoïdose, syndroom van Turner, en vaak bij patiënten die een Fontan-operatie ondergingen. Het wordt gekenmerkt door malabsorptie, diarree, oedeem door hypoproteïnemie, steatorroe, en sereuze effusies."},"pt":{"name":"Linfangiectasia intestinal secundária"},"pl":{"name":"Wtórna limfangiektazja jelitowa"},"cs":{"name":"Sekundární střevní lymfangiektázie"},"uk":{"name":"Вторинна кишкова лімфангіектазія","definition":"Secondary intestinal lymphangiectasia is an acquired from of intestinal lymphangiectasia (see this term) manifesting as a protein-losing enteropathy due to another disorder such as Crohn’s disease, congestive heart failure, sarcoidosis, Turner syndrome (see these terms) and often in patients who have undergone a Fontan operation. It is characterized by malabsorption, diarrhea, edema due hypoproteinemia, steatorrhea and serosal effusions."},"zh":{"name":"继发性小肠淋巴管扩张"},"tr":{"name":"Sekonder bağırsak lenfanjiyektazi"}},"xrefs":[{"source":"ICD-10","code":"I89.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"BD92.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1730894033"},{"source":"MONDO","code":"0019574","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4273969","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97935,"name":"Rare gastroenterologic disease"},"parents":[{"orpha":36204,"name":"Intestinal lymphangiectasia","classifications":[152]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult","Elderly"],"prevalence":[],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-11-19","source":"25618488[PMID]_33431540[PMID]_26837404[PMID]_30322285[PMID]","terms":[{"hpo":"HP:0031686","label":"Increased stool alpha1-antitrypsin concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001004","label":"Lymphedema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002028","label":"Chronic diarrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000969","label":"Edema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003146","label":"Hypocholesterolemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004788","label":"Intestinal lymphedema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001888","label":"Lymphopenia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002024","label":"Malabsorption","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011741","label":"Secondary hyperaldosteronism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011848","label":"Abdominal colic","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012050","label":"Anasarca","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002608","label":"Celiac disease","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012281","label":"Chylous ascites","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001394","label":"Cirrhosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002563","label":"Constrictive pericarditis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002720","label":"Decreased circulating IgA level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032136","label":"Decreased circulating IgG1 level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004313","label":"Decreased circulating antibody level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031085","label":"Decreased circulating prealbumin concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002850","label":"Decreased circulating total IgM","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012537","label":"Food intolerance","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032064","label":"Gastrointestinal eosinophilia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003073","label":"Hypoalbuminemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011900","label":"Hypofibrinogenemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002665","label":"Lymphoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002202","label":"Pleural effusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032387","label":"Reduced transferrin level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100324","label":"Scleroderma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012191","label":"B-cell lymphoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002584","label":"Intestinal bleeding","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0005214","label":"Intestinal obstruction","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002576","label":"Intussusception","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002719","label":"Recurrent infections","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001708","label":"Right ventricular failure","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001907","label":"Thromboembolism","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002580","label":"Volvulus","frequency":"Very rare (<4-1%)","criteria":null}]}}