{"orpha":363999,"name":"Non-immune hydrops fetalis","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Non-immune HF","Non-immune fetal edema","Non-immune fetal hydrops","NIHF"],"definition":"Non-immune hydrops fetalis (NIHF), a form of HF, is a severe fetal condition defined as the excessive accumulation of fetal fluid within the fetal extravascular compartments and body cavities, and is the end-stage of a wide variety of disorders.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-363999/","orphanet_url":"https://www.orpha.net/en/disease/detail/363999","names":{"fr":{"name":"Anasarque foetoplacentaire non immunologique","synonyms":["Anasarque foetale non immunologique","HF non immunologique","Hydrops fetalis non immunologique","Hydrops foetoplacentaire non immunologique"],"definition":"L'anasarque foeto-placentaire non immunologique (NIHF), l'une des formes de l'anasarque foeto-placentaire (HF), est une maladie foetale sévère définie par une accumulation excessive de liquide amniotique dans le compartiment extravasculaire foetal et les cavités. Elle constitue la phase terminale d'une grande variété de maladies."},"de":{"name":"Hydrops fetalis, nicht-immunologischer","synonyms":["NIHF","Nicht-immunologischer fetaler Hydrops","Nicht-immunologisches fetales Ödem"],"definition":"Der Nicht-immunologische Hydrops fetalis (NIHF), eine Form des HF (siehe diesen Terminus), ist eine schwere fetale Störung mit exzessiver Ansammlung fetaler Flüssigkeit im Interstitium und den Körperhöhlen des Feten und kennzeichnet das Endstadium einer großen Zahl von Krankheiten."},"es":{"name":"Hidropesía fetal no inmunológica","synonyms":["Edema fetal no inmunitario","HF no inmunitaria","Hidrops fetal no inmunológico","NIHF"],"definition":"La hidropesía fetal no inmunitaria (HFNI), una forma de HF (ver este término), es una enfermedad fetal grave que se define como la excesiva acumulación de líquido fetal en los compartimentos fetales extravasculares y en las cavidades corporales, y es la consecuencia final de una amplia serie de trastornos."},"it":{"name":"Idrope fetale non immune","synonyms":["Edema fetale non immune","HF non immune","Idropisia fetale non immune"],"definition":"L'idrope fetale non immune (NIHF), una forma di HF (si veda questo termine), è una grave malattia fetale caratterizzata dall'accumulo eccessivo di liquidi nei compartimenti extravascolari e nelle cavità del corpo del feto. Rappresenta lo stadio terminale di un'ampia gamma di malattie."},"nl":{"name":"Niet-immune hydrops foetalis","synonyms":["NIHF","Niet-immune HF","Niet-immune foetale hydrops","Niet-immuun foetaal oedeem"],"definition":"Niet-immune hydrops foetalis (NIHF), een vorm van HF (zie deze term), is een ernstige foetale aandoening die gedefinieerd wordt als de overmatige accumulatie van foetale vloeistof in de foetale extravasculaire compartimenten en lichaamsholten, en is het eindstadium van een grote verscheidenheid aan stoornissen."},"pt":{"name":"Hidropsia fetal não-imune"},"pl":{"name":"Nieimmunologiczny obrzęk płodu","synonyms":["Nieimmunologiczny HF"]},"cs":{"name":"Neimunitní hydrops fetalis","synonyms":["Neimunitní HF","Neimunitní fetální edém","Neimunitní fetální hydrops"]},"uk":{"name":"Неімунна водянка плода","synonyms":["NIHF","Неімунний набряк плода","Неімунна водянка плода","Неімунна ВП"],"definition":"Non-immune hydrops fetalis (NIHF), a form of HF, is a severe fetal condition defined as the excessive accumulation of fetal fluid within the fetal extravascular compartments and body cavities, and is the end-stage of a wide variety of disorders."},"zh":{"name":"非免疫性胎儿水肿","synonyms":["非免疫性胎儿水肿"],"definition":"非免疫性胎儿水肿（NIHF）是胎儿水肿的一种形式，是一种严重的胎儿疾病，其定义为胎儿液体在胎儿血管外腔和体腔内的过度积聚，且是多种疾病的终末期。"},"tr":{"name":"İmmün olmayan hidrops fetalis","synonyms":["NIHF","İmmun olmayan HF","İmmun olmayan fetal ödem","İmmun olmayan fetal hidrops"]}},"xrefs":[{"source":"OMIM","code":"236750","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"OMIM","code":"618773","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"OMIM","code":"619369","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"OMIM","code":"620244","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"P83.2","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"KC41.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1182287637"},{"source":"MONDO","code":"0009369","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0455988","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":89845,"name":"OBSOLETE: Idiopathic hydrops fetalis","status":"inactive"}],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":1041,"name":"Hydrops fetalis","classifications":[147]},{"orpha":183530,"name":"Rare genetic developmental defect during embryogenesis","classifications":[156]}],"children":[],"genes":[{"symbol":"ANGPT2","name":"angiopoietin 2","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) (loss of function) in","status":"Assessed","source":"34876502[PMID]","hgnc":"485","ensembl":"ENSG00000091879","omim":"601922","loci":["8p23.1"]},{"symbol":"CALCRL","name":"calcitonin receptor like receptor","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) in","status":"Assessed","source":"30115739[PMID]","hgnc":"16709","ensembl":"ENSG00000064989","omim":"114190","loci":["2q32.1"]},{"symbol":"THSD1","name":"thrombospondin type 1 domain containing 1","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) in","status":"Assessed","source":"30055085[PMID]","hgnc":"17754","ensembl":"ENSG00000136114","omim":"616821","loci":["13q14.3"]}],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Antenatal","Neonatal"],"prevalence":[{"type":"Prevalence at birth","qualification":"Value and class","class":"1-5 / 10 000","value":42.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Value and class","class":">1 / 1000","value":300.0,"area":"Thailand","status":"Validated","source":"9280003[PMID]"}],"phenotypes":null}