{"orpha":141179,"name":"Non-involuting congenital hemangioma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["NICH","Non-involuting congenital haemangioma"],"definition":"A rare congenital hemangioma characterized by flat or slightly bossed, round or oval shape, usually solitary, that are fully formed in utero and differ from rapidly involuting congenital hemangiomas (RICH) mainly because they do not undergo a postnatal involuting phase.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-141179/","orphanet_url":"https://www.orpha.net/en/disease/detail/141179","names":{"fr":{"name":"Hémangiome congénital non involutif","synonyms":["NICH"],"definition":"Hémangiome congénital rare caractérisé par une lésion de forme ronde ou ovale, plate ou légèrement en relief, généralement solitaire, entièrement formée in utero et qui diffère des hémangiomes congénitaux à involution rapide (RICH) principalement en raison de l'absence de la phase d'involution post-natale."},"de":{"name":"Nicht-involutierendes kongenitales Hämangiom","synonyms":["NICH"],"definition":"Es handelt sich um ein seltenes kongenitales Hämangiom, das sich durch eine flache oder leicht gewölbte, runde oder ovale Form auszeichnet. In der Regel ist es solitär und bereits bei der Geburt vollständig entwickelt. Es unterscheidet sich von den rasch zurückbildenden kongenitalen Hämangiomen (RICH; Rapid Involuting Congenital Hemangioma) hauptsächlich dadurch, dass es keine postnatale Involutionsphase durchläuft."},"es":{"name":"Hemangioma congénito no involutivo","synonyms":["NICH"],"definition":"Es un hemangioma congénito poco frecuente caracterizado por una lesión plana o ligeramente sobreelevada, de contorno redondeado u ovalado, generalmente único, que está completamente formada in útero y que se diferencia de los hemangiomas congénitos de involución rápida (RICH; por sus siglas en inglés) principalmente porque no presentan una fase de involución posnatal."},"it":{"name":"Emangioma congenito non involutivo","synonyms":["NICH"]},"nl":{"name":"Niet-involuerend congenitaal hemangioom","synonyms":["NICH"],"definition":"Een zeldzaam congenitaal hemangioom, gekenmerkt door platte of licht gebolde, ronde of ovale, meestal solitaire vasculaire tumoren die volledig gevormd worden <i>in utero</i> en die verschillen van snel involuerende congenitale hemangiomen (RICH), vooral omdat ze geen postnatale involutie ondergaan."},"pt":{"name":"Hemangioma não evolutivo, congénito","definition":"Os hemangiomas não-evolutivos congénitos (NICH) são um tipo distinto de hemangiomas congénitos grandes, totalmente formados <i>in utero</i> e diferem dos hemangiomas congénitos rapidamente evolutivos (RICH; ver este termo) principalmente porque passam por uma fase evolutiva pós-natal."},"pl":{"name":"Naczyniak wrodzony niezanikający","synonyms":["NICH"],"definition":"Rzadki wrodzony naczyniak krwionośny charakteryzujący się płaskim lub lekko wypukłym, okrągłym lub owalnym kształtem, zwykle pojedynczy, który rozwija się w pełni w życiu płodowym i różni się od szybko zanikających wrodzonych naczyniaków krwionośnych głównie tym, że nie przechodzi fazy inwolucji po urodzeniu."},"cs":{"name":"Neinvolující vrozený hemangiom","synonyms":["NICH"]},"uk":{"name":"Неінвазивна вроджена гемангіома","synonyms":["NICH"],"definition":"Non-involuting congenital hemangiomas (NICH) are a distinctive type of large congenital hemangioma that are fully formed <i>in utero</i> and differ from rapidly involuting congenital hemangiomas (RICH; see this term) mainly because they do not undergo a postnatal involuting phase."},"zh":{"name":"非消退性先天性血管瘤","synonyms":["NICH"],"definition":"非消退型先天性血管瘤（NICH）是巨大先天性血管瘤中的一种独特类型，在子宫内完全形成，与快速消退型先天性血管瘤（RICH；见该词条）不同，主要原因是本病出生后无消退期。"},"tr":{"name":"Gerilemeyen doğumsal hemanjiyom","synonyms":["NICH"]}},"xrefs":[{"source":"ICD-10","code":"D18.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2E81.2Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1566404182"},{"source":"ICD-11","code":"XH5427","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"557135587"},{"source":"MONDO","code":"0015403","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1275417","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10890","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":458775,"name":"Congenital hemangioma","classifications":[147,198,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Antenatal"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-05-15","source":"27058448[PMID]_18676554[PMID]_27540637[PMID]","terms":[{"hpo":"HP:0001028","label":"Hemangioma","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0007466","label":"Midfrontal capillary hemangioma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031449","label":"Perineal hemangioma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007618","label":"Subcutaneous calcification","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100585","label":"Telangiectasia of the skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001635","label":"Congestive heart failure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031207","label":"Hepatic hemangioma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100784","label":"Peripheral arteriovenous fistula","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001015","label":"Prominent superficial veins","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0410266","label":"Visceral hemangioma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012329","label":"Tufted angioma","frequency":"Excluded (0%)","criteria":null}]}}