{"orpha":141209,"name":"Generalized lymphatic anomaly","type":"Malformation syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["Diffuse lymphangioma","Diffuse lymphangiomatosis","Disseminated lymphangioma","Disseminated lymphangiomatosis","Disseminated lymphatic malformation","GLA","Diffuse lymphatic malformation"],"definition":"A rare developmental defect during embryogenesis characterized by multifocal dilated lymphatic vessels involving multiple organs and tissues. Patients mostly present in infancy and childhood. Clinical course and prognosis depend on the affected sites and extent of the condition, deterioration of lung function being a major cause of morbidity and mortality.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-141209/","orphanet_url":"https://www.orpha.net/en/disease/detail/141209","names":{"fr":{"name":"Malformation lymphatique diffuse","synonyms":["Malformation lymphatique disséminée","GLA","Lymphangiomatose diffuse","Lymphangiomatose disséminée","Lymphangiome diffus","Lymphangiome disséminé"],"definition":"Anomalie rare du développement embryonnaire, caractérisée par une dilatation multifocale des vaisseaux lymphatiques affectant plusieurs organes et tissus. La maladie se manifeste la plupart du temps dans la petite enfance et l'enfance. L'évolution clinique et le pronostic dépendent des zones touchées et de l'étendue de l'atteinte, la détérioration de la fonction pulmonaire étant la cause majeure de morbidité et de mortalité."},"de":{"name":"Generalisierte lymphatische Anomalie","synonyms":["GLA","Lymphatische Fehlbildung, diffuse","Lymphangiom, diffuses","Lymphangiom, disseminiertes","Lymphangiomatose, diffuse","Lymphangiomatose, disseminierte","Lymphatische Fehlbildung, disseminierte"],"definition":"Eine seltene Entwicklungsstörung während der Embryogenese, die durch multifokal erweiterte Lymphgefäße gekennzeichnet ist, die mehrere Organe und Gewebe betreffen. Die Patienten werden meist im Säuglings- und Kindesalter vorgestellt. Klinischer Verlauf und Prognose hängen von den betroffenen Stellen und dem Ausmaß der Erkrankung ab, wobei die Verschlechterung der Lungenfunktion eine der Hauptursachen für Morbidität und Mortalität ist."},"es":{"name":"Anomalía linfática generalizada","synonyms":["ALG","Linfangiomatosis diseminada","Malformación linfática difusa","Linfangioma difuso","Linfangioma diseminado","Linfangiomatosis difusa","Malformación linfática diseminada"],"definition":"Es un defecto del desarrollo embrionario poco frecuente caracterizado por unos vasos linfáticos multifocales dilatados afectando a múltiples órganos y tejidos. Los pacientes se presentan principalmente en la lactancia y en la infancia. El curso clínico y el pronóstico de la enfermedad dependen de los sitios afectados y de la extensión del trastorno, siendo el deterioro de la función pulmonar una de las principales causas de morbimortalidad."},"it":{"name":"Anomalia linfatica generalizzata","synonyms":["Malformazione linfatica diffusa","GLA","Linfagiomatosi diffusa","Linfangioma diffuso","Linfangioma disseminato","Linfangiomatosi disseminata","Malformazione linfatica disseminata"],"definition":"Si tratta di un difetto raro dello sviluppo dell'embrione, caratterizzato da una dilatazione multifocale dei vasi linfatici, che interessa diversi organi e tessuti. La malattia di solito esordisce nella prima infanzia e nell'infanzia. Il decorso clinico e la prognosi dipendono dalle zone interessate e dall'estensione della malattia. Il deterioramento della funzione polmonare è la principale causa di morbilità e mortalità."},"nl":{"name":"Gegeneraliseerde lymfatische anomalie","synonyms":["Diffuus lymfangioom","Diffuse lymfangiomatose","Gedissemineerd lymfangioom","Gedissemineerde lymfangiomatose","GLA","Gedissemineerde lymfatische malformatie","Gedissemineerde malformatie van lymfestelsel","Diffuse lymfatische malformatie","Diffuse malformatie van lymfestelsel"],"definition":"Een zeldzaam ontwikkelingsdefect tijdens embryogenese, gekarakteriseerd door multifocale dilatatie van lymfevaten, met betrokkenheid van meerdere organen en weefsels. Patiënten presenteren zich meestal in de zuigelingentijd en kindertijd. Klinisch verloop en prognose hangen af van de aangetaste gebieden en omvang van de aandoening, en aftakeling van longfunctie is een voorname oorzaak van morbiditeit en mortaliteit."},"pt":{"name":"Linfangiomatose difusa"},"pl":{"name":"Rozlana malformacja limfatyczna","synonyms":["GLA","Limfangiomatoza rozlana","Limfangiomatoza rozsiana","Rozlany obrzęk limfatyczny","Rozsiana malformacja limfatyczna","Rozsiany obrzęk limfatyczny","Uogólniona anomalia limfatyczna"]},"cs":{"name":"Difuzní lymfatická malformace","synonyms":["Difuzní lymfangiom","Difuzní lymfangiomatóza","Diseminovaný lymfangiom","Diseminovaná lymfangiomatóza","Diseminovaná lymfatická malformace","GLA","Generalizovaná lymfatická anomálie"]},"uk":{"name":"Дифузна лімфатична мальформація","synonyms":["Дифузна лімфангіома","Дифузний лімфангіоматоз","Дисемінована лімфангіома","Дисемінований лімфангіоматоз","Дисемінована лімфома","Генералізована лімфатична аномалія","GLA"],"definition":"A rare developmental defect during embryogenesis characterized by multifocal dilated lymphatic vessels involving multiple organs and tissues. Patients mostly present in infancy and childhood. Clinical course and prognosis depend on the affected sites and extent of the condition, deterioration of lung function being a major cause of morbidity and mortality."},"zh":{"name":"弥漫性淋巴管畸形","synonyms":["播散性淋巴管畸形"],"definition":"一种罕见的胚胎发育缺陷，其特征是累及多个器官和组织的多病灶扩张性淋巴管。患者多见于婴儿期和儿童期。临床病程和预后取决于病变部位和程度，肺功能恶化是发病和死亡的主要原因。"},"tr":{"name":"Yaygın lenfatik malformasyon","synonyms":["GLA","Yaygın lenfanjiyom","Yaygın lenfanjiyomatoz","Yaygın lenfanjiyom","Jeneralize lenfatik anomali","Yaygın lenfanjiyomatoz","Yaygın lenfatik malformasyon"]}},"xrefs":[{"source":"ICD-10","code":"I89.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2E81.10","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"913891613"},{"source":"MONDO","code":"0015408","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3839921","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19961","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":719950,"name":"Complex lymphatic anomaly","classifications":[147,198]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Infancy","Neonatal"],"prevalence":[],"phenotypes":null}