{"orpha":2035,"name":"Lymphatic filariasis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Lymphatic filariasis (LF) is a severe form of filariasis, caused by the parasitic worms <i>Wuchereria bancrofti</i>, <i>Brugia malayi</i> and <i>Brugia timori</i>, and the most common cause of acquired lymphedema worldwide. LF is endemic to tropical and subtropical regions. The vast majority of infected patients are asymptomatic but it can also cause a variety of clinical manifestations, including limb lymphedema, genital anomalies (hydrocele, chylocele), elephantiasis in later stages of the disease (frequently in the lower extremities), and tropical pulmonary eosinophilia (nocturnal paroxysmal cough and wheezing, weight loss, low-grade fever, adenopathy, and pronounced blood eosinophilia). Renal involvement (hematuria, proteinuria, nephritic syndrome, glomerulonephritis), and mono-arthritis of the knee or ankle joint have also been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-2035/","orphanet_url":"https://www.orpha.net/en/disease/detail/2035","names":{"fr":{"name":"Filariose lymphatique","definition":"La filariose lymphatique (FL) est une forme sévère de filariose causée par des vers parasitaires : <i>Wuchereria bancrofti</i>, <i>Brugia malayi</i> et <i>Brugia timori</i>, et il s'agit de la cause la plus fréquente au monde de lymphoedème acquis. La FL est endémique des régions tropicales et subtropicales. La grande majorité des personnes infectées sont asymptomatiques mais la maladie peut causer une variété de manifestations cliniques, incluant un lymphoedème des membres, des anomalies génitales (hydrocèle, chylocèle), une éléphantiasis (généralement des extrémités inférieures) et une éosinophilie pulmonaire tropicale (toux et sifflement paroxystiques nocturnes, perte de poids, fébricule, adénopathies et éosinophilie périphérique importante). Une atteinte rénale (hématurie, protéinurie, syndrome néphrotique, glomérulonéphrite), et une mono-arthrite du genou ou de la cheville ont aussi été rapportées."},"de":{"name":"Lymphatische Filariose"},"es":{"name":"Filariasis linfática","definition":"La filariasis linfática (FL) es una forma grave de filariasis (véase este término) causada por los gusanos parásitos <i>Wuchereria bancrofti</i>, <i>Brugia malayi</i> y <i>Brugia timori</i>, constituyendo la causa más común de edema linfático adquirido en todo el mundo. La FL es endémica de las regiones tropicales y subtropicales. La inmensa mayoría de los pacientes infectados son asintomáticos, pero también puede producir diversas manifestaciones clínicas, tales como linfedema en las extremidades, anomalías genitales (hidrocele, quilocele), elefantiasis en estadios tardíos de la enfermedad (con frecuencia en las extremidades inferiores) y eosinofilia pulmonar tropical (tos paroxística nocturna y sibilación, pérdida de peso, fiebre leve, adenopatía y eosinofilia sanguínea acusada). También se ha registrado afectación renal (hematuria, proteinuria, síndrome nefrítico, glomerulonefritis) y monoartritis de la rodilla o del tobillo."},"it":{"name":"Filariosi linfatica","definition":"La filariosi linfatica (LF) è una forma grave di filariosi (si veda questo termine), causata dai vermi parassiti <i>Wuchereria bancrofti</i>, <i>Brugia malayi</i> e <i>Brugia timori</i>, ed è la causa più comune di linfedema acquisito a livello mondiale. La LF è endemica nelle regioni tropicali e subtropicali. La quasi totalità dei pazienti infetti è asintomatica, anche se la malattia può presentarsi con una serie di segni clinici, fra cui il linfedema agli arti, le anomalie genitali (idrocele, chilocele), l'elefantiasi nelle fasi avanzate della malattia (spesso delle estremità inferiori), e l'eosinofilia polmonare tropicale (tosse parossistica notturna e respiro affannoso, perdita di peso, febbricola, adenopatia, marcata eosinofilia ematica). Inoltre, sono stati descritti il coinvolgimento renale (ematuria, proteinuria, sindrome nefrosica, glomerulonefrite) e la monoartrite del ginocchio o della caviglia."},"nl":{"name":"Lymfatische filariasis","definition":"Lymfatische filariasis (LF) is een ernstige vorm van filariasis (zie deze term), veroorzaakt door de parasitaire wormen <i>Wuchereria bancrofti</i>, <i>Brugia malayi</i> en <i>Brugia timori</i>, en is wereldwijd de meest voorkomende oorzaak van verworven lymfoedeem. LF is endemisch in tropische en subtropische regio's. De grote meerderheid van geïnfecteerde patiënten is asymptomatisch, maar er kan ook een verscheidenheid aan klinische manifestaties veroorzaakt worden, waaronder lymfoedeem van de ledematen, genitale afwijkingen (hydrocele, chylocele), elefantiase in latere stadia van de ziekte (regelmatig in de onderste ledematen), en tropische pulmonale eosinofilie (nachtelijke paroxysmale hoest en piepende ademhaling, gewichtsverlies, lichte koorts, adenopathie, en uitgesproken bloedeosinofilie). Betrokkenheid van de nieren (hematurie, proteïnurie, nefritisch syndroom, glomerulonefritis) en monoartritis van het knie- of enkelgewricht werden eveneens gemeld."},"pt":{"name":"Filariose linfática"},"pl":{"name":"Filarioza limfatyczna"},"cs":{"name":"Lymfatická filarióza"},"uk":{"name":"Лімфатичний філяріоз","definition":"Lymphatic filariasis (LF) is a severe form of filariasis (see this term), caused by the parasitic worms <i>Wuchereria bancrofti</i>, <i>Brugia malayi</i> and <i>Brugia timori</i>, and the most common cause of acquired lymphedema worldwide. LF is endemic to tropical and subtropical regions. The vast majority of infected patients are asymptomatic but it can also cause a variety of clinical manifestations, including limb lymphedema, genital anomalies (hydrocele, chylocele), elephantiasis in later stages of the disease (frequently in the lower extremities), and tropical pulmonary eosinophilia (nocturnal paroxysmal cough and wheezing, weight loss, low-grade fever, adenopathy, and pronounced blood eosinophilia). Renal involvement (hematuria, proteinuria, nephritic syndrome, glomerulonephritis), and mono-arthritis of the knee or ankle joint have also been reported."},"zh":{"name":"淋巴性丝虫病"},"tr":{"name":"Lenfatik filaryaz"}},"xrefs":[{"source":"ICD-10","code":"B74.0","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B74.1","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"B74.2","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"1F66.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"247221550"},{"source":"ICD-11","code":"1F66.30","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"1592269710"},{"source":"ICD-11","code":"1F66.31","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"1000592924"},{"source":"ICD-11","code":"1F66.32","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"1326352101"},{"source":"UMLS","code":"C0013884","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"3321","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10016675","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":2034,"name":"Filariasis","classifications":[203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":">1 / 1000","value":0.0,"area":"Specific population","status":"Validated","source":"World Health Organization[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2020-04-09","source":"31027660[PMID]_31312785[PMID]","terms":[{"hpo":"HP:0100763","label":"Abnormality of the lymphatic system","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001004","label":"Lymphedema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003550","label":"Predominantly lower limb lymphedema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012224","label":"Circulating immune complexes","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032061","label":"Hypereosinophilia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000962","label":"Hyperkeratosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000953","label":"Hyperpigmentation of the skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002840","label":"Lymphadenitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012531","label":"Pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002088","label":"Abnormal lung morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000077","label":"Abnormality of the kidney","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000045","label":"Abnormality of the scrotum","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012735","label":"Cough","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000034","label":"Hydrocele testis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031842","label":"Lymphangiectasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032260","label":"Opportunistic bacterial infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031690","label":"Opportunistic infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100796","label":"Orchitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002091","label":"Restrictive ventilatory defect","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100673","label":"Vaginal hydrocele","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030828","label":"Wheezing","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001785","label":"Ankle swelling","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000031","label":"Epididymitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000099","label":"Glomerulonephritis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000790","label":"Hematuria","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0005086","label":"Knee osteoarthritis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000100","label":"Nephrotic syndrome","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0008763","label":"No social interaction","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000093","label":"Proteinuria","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000796","label":"Urethral obstruction","frequency":"Very rare (<4-1%)","criteria":null}]}}