{"orpha":141184,"name":"Rapidly involuting congenital hemangioma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["RICH","Rapidly involuting congenital haemangioma"],"definition":"A rare congenital hemangioma characterized by benign round or oval-shaped, solitary and raised or infiltrating vascular tumors that are fully formed in utero and differ from non-involuting congenital hemangiomas (NICH) mainly because they undergo rapid postnatal involution.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-141184/","orphanet_url":"https://www.orpha.net/en/disease/detail/141184","names":{"fr":{"name":"Hémangiome congénital rapidement involutif","synonyms":["RICH"],"definition":"Hémangiome congénital rare caractérisé par des tumeurs vasculaires bénignes, de forme ronde ou ovale, solitaires, surélevées ou infiltrantes, pleinement formées <i>in utero</i>, qui diffèrent d'un hémangiome non-involutif congénital (NICH) principalement en raison de leur involution post-natale rapide."},"de":{"name":"Rasch involutierendes kongenitales Hämangiom","synonyms":["RICH"],"definition":"Ein seltenes kongenitales Hämangiom, das durch gutartige runde oder ovale, solitäre und erhabene oder infiltrierende vaskuläre Tumore gekennzeichnet ist, die sich <i>in utero</i> vollständig ausbilden und sich von nicht-involutierenden kongenitalen Hämangiomen (NICH) hauptsächlich dadurch unterscheiden, dass sie sich nach der Geburt rasch zurückbilden."},"es":{"name":"Hemangioma congénito rápidamente involutivo","synonyms":["RICH"],"definition":"Es un hemangioma congénito poco frecuente caracterizado por tumores vasculares benignos, solitarios, de forma redondeada u ovalada, sobreelevados o infiltrantes, que se forman completamente en el útero y que se diferencian de los hemangiomas congénitos no involutivos (NICH; por sus siglas en inglés) principalmente porque experimentan una rápida involución posnatal."},"it":{"name":"Emangioma congenito a rapida involuzione","synonyms":["RICH"]},"nl":{"name":"Snel involuerend congenitaal hemangioom","synonyms":["RICH"],"definition":"Een zeldzaam congenitaal hemangioom, gekenmerkt door goedaardige, ronde of ovale, solitaire en verheven of infiltrerende vasculaire tumoren die volledig gevormd worden <i>in utero</i> en die verschillen van niet-involuerende congenitale hemangiomen (NICH), vooral vanwege de snelle postnatale involutie die ze ondergaan."},"pt":{"name":"Hemangioma rapidamente evolutivo, congénito","definition":"Os hemangiomas, congénitos, rapidamente evolutivos (RICH) são um distinto tipo de hemangiomas congénitos que se formam completamente <i>in utero</i> e diferem dos hemangiomas congénitos não evolutivos (NICH; ver este termo) principalmente porque passam por uma rápida evolução pós-natal."},"pl":{"name":"Naczyniak wrodzony szybko zanikający","synonyms":["RICH"],"definition":"Rzadki wrodzony naczyniak krwionośny, charakteryzujący się obecnością łagodnych, okrągłych lub owalnych pojedynczych i wypukłych lub naciekających guzów naczyniowych, które rozwijają się w pełni w życiu płodowym i różnią się od wrodzonych naczyniaków krwionośnych nieinwolucyjnych głównie tym, że szybko zanikają po urodzeniu."},"cs":{"name":"Rychle involující vrozený hemangiom","synonyms":["RICH"]},"uk":{"name":"Вроджена гемангіома, що швидко поширюється","synonyms":["RICH"],"definition":"Rapidly involuting congenital hemangiomas (RICH) are a distinctive type of congenital hemangioma that are fully formed <i>in utero</i> and differ from non-involuting congenital haemangiomas (NICH; see this term) mainly because they undergo rapid postnatal involution."},"zh":{"name":"快速消退性先天性血管瘤","synonyms":["RICH"],"definition":"快速消退型先天性血管瘤（RICH）是先天性血管瘤中的一种独特类型，在子宫内完全形成，不同于非消退型先天性血管瘤（NICH；见该词条），主要原因是本病出生后快速消退。"},"tr":{"name":"Hızla gerileyen doğumsal hemanjiyom","synonyms":["RICH"]}},"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":"7534765"},{"source":"ICD-11","code":"XH6RC4","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"2140874384"},{"source":"MONDO","code":"0015404","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1275421","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19958","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]_7911127[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: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:0010885","label":"Avascular necrosis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031207","label":"Hepatic hemangioma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100578","label":"Lipoatrophy","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012329","label":"Tufted angioma","frequency":"Excluded (0%)","criteria":null}]}}