{"orpha":101959,"name":"Chronic primary adrenal insufficiency","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":["CPAI","Chronic adrenocorticoid insufficiency"],"definition":"Chronic primary adrenal insufficiency (CPAI) is a chronic disorder of the adrenal cortex resulting in the inadequate production of glucocorticoid and mineralocorticoid hormones.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-101959/","orphanet_url":"https://www.orpha.net/en/disease/detail/101959","names":{"fr":{"name":"Insuffisance surrénale primaire chronique","synonyms":["CPAI"],"definition":"Maladie chronique du cortex surrénalien se traduisant par une production insuffisante d'hormones glucocorticoïdes et minéralocorticoïdes."},"de":{"name":"Nebenniereninsuffizienz, chronische primäre","synonyms":["Adrenokortikale Insuffizienz, chronische","CPAI"],"definition":"Die chronische primäre Nebenniereninsuffizienz (CPAI) ist eine chronische Störung der Nebennierenrinde mit unzureichender Produktion von Glukokortikoid- und Mineralokortikoid-Hormonen."},"es":{"name":"Insuficiencia suprarrenal crónica primaria","definition":"La insuficiencia suprarrenal crónica primaria (CPAI) es un trastorno crónico de la corteza suprarrenal que resulta en una producción insuficiente de glucocorticoides y mineralocortocoides."},"it":{"name":"Insufficienza surrenalica primitiva cronica","synonyms":["CPAI","Insufficienza adrenocorticale cronica"],"definition":"L'insufficienza surrenalica cronica primitiva (CPAI) è una malattia cronica della corteccia surrenale che esita in un'insufficiente produzione di ormoni glucocorticoidi e mineralcorticoidi."},"nl":{"name":"Chronische primaire adrenale insufficiëntie","synonyms":["CPAI","Chronische bijnierschorsinsufficiëntie","Chronische primaire bijnierinsufficiëntie"],"definition":"Chronische primaire adrenale insufficiëntie (CPAI) is een chronische aandoening van de bijnierschors die resulteert in een ontoereikende productie van glucocorticoïd en mineralocorticoïd hormonen."},"pt":{"name":"Insuficiência supra-renal primária crónica","synonyms":["CPAI","Insuficiência adrenocortical crónica"],"definition":"A insuficiência supra-renal primária crónica (CPAI) é uma doença crónica do córtex da supra-renal, que resulta na produção insuficiente de hormonas glico e mineralocorticóides."},"pl":{"name":"Przewlekła pierwotna niedoczynność nadnerczy","synonyms":["CPAI","Przewlekła niedoczynność kory nadnerczy"]},"cs":{"name":"Chronická primární adrenální insuficience","synonyms":["CPAI","Chronická adrenokortikoidní insuficience"]},"uk":{"name":"Хронічна первинна надниркова недостатність","synonyms":["Хронічна адренокортикоїдна недостатність","CPAI"],"definition":"Chronic primary adrenal insufficiency (CPAI) is a chronic disorder of the adrenal cortex resulting in the inadequate production of glucocorticoid and mineralocorticoid hormones."},"zh":{"name":"慢性原发性肾上腺功能不全","synonyms":["慢性肾上腺功能不全"],"definition":"慢性原发性肾上腺功能不全(CPAI)是一种慢性肾上腺皮质疾病，导致糖皮质激素和盐皮质激素分泌不足。"},"tr":{"name":"Kronik primer adrenal yetmezlik","synonyms":["CPAI","Kronik adrenokortikoid yetmezliği"]}},"xrefs":[{"source":"MONDO","code":"0015129","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"15129","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5848257","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19803","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":101958,"name":"Primary adrenal insufficiency","classifications":[193]}],"children":[{"orpha":101963,"name":"Acquired chronic primary adrenal insufficiency","type":"Category"},{"orpha":101960,"name":"Genetic chronic primary adrenal insufficiency","type":"Category"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.4,"area":"Europe","status":"Validated","source":"[EXPERT]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":14.0,"area":"Europe","status":"Validated","source":"12788587[PMID]_[EXPERT]"}],"phenotypes":null}