{"orpha":99892,"name":"ACTH-dependent Cushing syndrome","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["ACTH-dependent CS","Adrenocorticotropic hormone-dependent Cushing syndrome","Corticotropin-dependent Cushing syndrome"],"definition":"A group of endogenous Cushing syndrome (CS) caused by abnormal production of ACTH due, in 80% of cases, to adrenocorticotropic hormone (ACTH) oversecretion by a pituitary adenoma (Cushing disease, CD) and in 20% of cases to ectopic ACTH secretion (CS due to EAS) by an extrapituitary tumor or very rarely due to a tumor secreting both ACTH and corticotrophin-releasing hormone (CRH).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99892/","orphanet_url":"https://www.orpha.net/en/disease/detail/99892","names":{"fr":{"name":"Syndrome de Cushing dépendant de l'ACTH","synonyms":["CS dépendant de l'ACTH","Syndrome de Cushing dépendant de l'adrénocorticotropine"],"definition":"Groupe de syndromes de Cushing (SC) endogènes causés par une production anormale d'ACTH due, dans 80 % des cas, à une sécrétion excessive de l'hormone adrénocorticotrope (ACTH) par un adénome hypophysaire (maladie de Cushing, MC) et, dans 20 % des cas, à une sécrétion ectopique de l'ACTH par une tumeur extra-hypophysaire ou très rarement par une tumeur sécrétant à la fois l'ACTH et la corticolibérine (CRH)."},"de":{"name":"Cushing-Syndrom, ACTH-abhängiges","synonyms":["ACTH-abhängiges CS","Adrenocorticotropes Hormon-bedingtes Cushing-Syndrom","Corticotropin-abhängiges Cushing-Syndrom"],"definition":"Eine Form des endogenen Cushing-Syndroms (CS), verursacht durch eine abnormale Produktion von ACTH, die in 80 % der Fälle auf eine Überproduktion des adrenokortikotropen Hormons (ACTH) durch ein Hypophysenadenom (Cushing-Krankheit, CD) und in 20 % der Fälle auf eine ektopische ACTH-Sekretion (CS aufgrund von EAS) durch einen extrapituitären Tumor oder sehr selten durch einen Tumor, der sowohl ACTH als auch Corticotropin-Releasing-Hormon (CRH) sekretiert."},"es":{"name":"Síndrome de Cushing dependiente de ACTH","synonyms":["CS ACTH-dependiente","Síndrome de Cushing dependiente de corticotropina","Síndrome de Cushing dependiente de la hormona adrenocorticotropa"],"definition":"Es un grupo del síndrome de Cushing (SC) endógeno causado por una producción anómala de ACTH debido, en el 80% de los casos, al exceso de secreción de hormona adrenocorticotrópica (ACTH) por un adenoma hipofisario (enfermedad de Cushing, EC) y en el 20% de los casos a la secreción ectópica de ACTH (SC por EAS) por un tumor extrahipofisario o, muy raramente, debido a un tumor que secreta tanto ACTH como hormona liberadora de corticotropina (CRH)."},"it":{"name":"Sindrome di Cushing ACTH-dipendente","synonyms":["CS ACTH-dipendente","Sindrome di Cushing corticotropina-dipendente","Sindrome di Cushing dipendente dall'ormone adrenocorticotropo"],"definition":"La sindrome di Cushing dipendente dall'ormone adrenocorticotropo (CS ACTH-dipendente) è una forma di CS endogena (si veda questo termine) da produzione anomala di ACTH dovuta, nell'80% dei casi, all'ipersecrezione di ACTH da adenoma ipofisario (malattia di Cushing, CD) e, nel 20% dei casi, alla secrezione ectopica di ACTH (CS da EAS; si vedano questi termini) da tumore extraipofisario (che nel 50% dei casi origina nei polmoni e, meno spesso, nel timo, nel pancreas, nei surreni, nella tiroide) o, molto raramente, da tumore secernente sia l'ACTH che l'ormone di rilascio della corticotropina (CRH)."},"nl":{"name":"ACTH-afhankelijk syndroom van Cushing","synonyms":["ACTH-afhankelijk CS","Adrenocorticotroop hormoon-afhankelijke syndroom van Cushing","Corticotropineafhankelijk syndroom van Cushing"],"definition":"Een groep van endogeen syndroom van Cushing (CS), veroorzaakt door abnormale productie van adrenocorticotroop hormoon (ACTH) die in 80% van de gevallen te wijten is aan hypersecretie van ACTH door een hypofyseadenoom (ziekte van Cushing, CD) en in 20% van de gevallen aan ectopische secretie van ACTH (CS door EAS) door een extrahypofysaire tumor, of in zeer zeldzame gevallen aan een tumor die zowel ACTH als corticotropine-releasing hormoon (CRH) afscheidt."},"pt":{"name":"Síndrome Cushing dependente de ACTH","definition":"Grupo da síndrome Cushing (SC) endógena causada pela produção anormal de ACTH devido, em 80% dos casos, à secreção excessiva do hormônio adrenocorticotrófico (ACTH) por um adenoma hipofisário (doença Cushing, DC) e em 20% dos casos à secreção ectópica de ACTH (SC devido a EAS) por um tumor extra-hipofisário ou muito raramente devido a um tumor que secreta ACTH e hormona libertadora de corticotrofina (CRH)."},"pl":{"name":"Zespół Cushinga zależny od ACTH","synonyms":["CS zależny od ACTH","Zespół Cushinga zależny od hormonu adrenokortykotropowego","Zespół Cushinga zależny od kortykotropiny"],"definition":"Grupa endogennego zespołu Cushinga, spowodowanego nieprawidłowym wytwarzaniem ACTH, w 80% przypadków na skutek nadmiernego wydzielania hormonu adrenokortykotropowego (ACTH) przez gruczolaka przysadki (choroba Cushinga), a w 20% przypadków na skutek ektopowego wydzielania ACTH przez guz umiejscowiony poza przysadką lub bardzo rzadko z powodu guza wydzielającego zarówno ACTH, jak i hormon uwalniający kortykotropinę (CRH)."},"cs":{"name":"ACTH dependentní Cushingův syndrom","synonyms":["ACTH-dependentní CS","Adrenokortikotropní hormon-dependentní Cushingův syndrom","Kortikotropin-dependentní Cushingův syndrom"]},"uk":{"name":"АКТГ-залежний синдром Кушинга","synonyms":["АКТГ-залежний КС","Синдром Кушинга, залежний від адренокортикотропного гормону","Кортикотропін-залежний синдром Кушинга"],"definition":"A group of endogenous Cushing syndrome (CS) caused by abnormal production of ACTH due, in 80% of cases, to adrenocorticotropic hormone (ACTH) oversecretion by a pituitary adenoma (Cushing disease, CD) and in 20% of cases to ectopic ACTH secretion (CS due to EAS) by an extrapituitary tumor or very rarely due to a tumor secreting both ACTH and corticotrophin-releasing hormone (CRH)."},"zh":{"name":"促肾上腺皮质激素依赖性库欣综合征","synonyms":["ACTH依赖性CS"],"definition":"ACTH依赖性库欣综合征是一种内源性库欣综合征(CS)，由ACTH的异常产生引起，80%的病例是由于垂体腺瘤(库欣病，CD)过量分泌促肾上腺皮质激素(ACTH)，20%的病例是由于垂体外肿瘤异位分泌ACTH(CS是由EAS引起的)(50%的病例起源于肺部或少见于胸腺、胰腺、肾上腺或甲状腺)，或极少数是由于极少数是由于肿瘤同时分泌促肾上腺皮质激素和促肾上腺皮质激素释放激素(CRH)。"},"tr":{"name":"ACTH-bağımlı Cushing sendromu","synonyms":["ACTH-bağımlı CS","Corticotropin-bağımlı Cushing sendromu","Adrenokortikotropik hormon-bağımlı Cushing sendromu"]}},"xrefs":[{"source":"ICD-10","code":"E24.0","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"MONDO","code":"0020528","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0342442","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":641613,"name":"Endogenous Cushing syndrome","classifications":[193]},{"orpha":399849,"name":"Rare female infertility due to an adrenal disorder","classifications":[201]},{"orpha":399584,"name":"Rare male infertility due to adrenal disorder","classifications":[201]}],"children":[{"orpha":96253,"name":"Cushing disease","type":"Disease"},{"orpha":99889,"name":"Cushing syndrome due to ectopic ACTH secretion","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.55,"area":"Worldwide","status":"Not yet validated","source":"20829610[PMID]_18226430[PMID]_15903005[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}