{"orpha":231580,"name":"Primary unilateral adrenal hyperplasia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PUAH"],"definition":"Primary unilateral adrenal hyperplasia (PUAH) is a surgically-correctable form of primary (hyper) aldosteronism (PA) characterized by renin suppression, unilateral aldosterone hypersecretion, and moderate to severe hypertension secondary to hyperplasia of the adrenal gland.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-231580/","orphanet_url":"https://www.orpha.net/en/disease/detail/231580","names":{"fr":{"name":"Hyperplasie surrénalienne unilatérale primitive","synonyms":["PUAH"],"definition":"L'hyperplasie surrénalienne unilatérale primitive (HSUP) est une forme chirurgicalement curable d'hyperaldostéronisme primitif , caractérisée par une rénine basse, une hypersécrétion unilatérale d'aldostérone et une hypertension artérielle (HTA) modérée à sévère secondaire à l'hyperplasie d'une glande surrénale."},"de":{"name":"Nebennierenhyperplasie, primäre unilaterale","definition":"Die Primäre einseitige Nebennieren-Hyperplasie (PUAH), eine chirurgisch korrigierbare Form des primären Hyper-Aldosteronismus (PA; s. dort), ist gekennzeichnet durch Renin-Suppression, einseitige Aldosteron-Hypersekretion und moderate bis schwere Hypertonie als Folge der Hyperplasie der Nebenniere."},"es":{"name":"Hiperplasia suprarrenal primaria unilateral","synonyms":["PUAH"],"definition":"La hiperplasia suprarrenal primaria unilateral (PUAH; por sus siglas en inglés) es una forma de (hiper)aldosteronismo primario (AP; consulte este término) que puede corregirse quirúrgicamente, caracterizada por la supresión de renina, hipersecreción unilateral de aldosterona e hipertensión de moderada a grave secundaria a una hiperplasia de la glándula suprarrenal."},"it":{"name":"Iperplasia surrenalica unilaterale primitiva","synonyms":["PUAH"],"definition":"L'iperplasia primitiva monolaterale del surrene (PUAH) è una forma di (iper-) aldosteronismo primitivo (PA; si veda questo termine), correggibile chirurgicamente, caratterizzata dalla soppressione della renina, ipersecrezione di aldosterone monolaterale e ipertensione moderata-grave secondaria all'iperplasia delle ghiandole surrenali."},"nl":{"name":"Primaire unilaterale bijnierhyperplasie","synonyms":["PUAH","Primaire unilaterale adrenale hyperplasie"],"definition":"Primaire unilaterale bijnierhyperplasie (PUAH) is een chirurgisch corrigeerbare vorm van primair hyperaldosteronisme (PA; zie deze term), gekarakteriseerd door suppressie van renine, unilaterale hypersecretie van aldosteron, en matige tot ernstige hypertensie secundair aan hyperplasie van bijnier."},"pt":{"name":"Hiperplasia supra-renal unilateral primária","synonyms":["PUAH"],"definition":"A hiperplasia adrenal unilateral primária (PUAH) é uma forma cirurgicamente corrigível de (hiper)aldosteronismo primário (PA; ver este termo) caracterizada por supressão da renina, hipersecreção de aldosterona unilateral e hipertensão moderada a grave secundária a hiperplasia da glândula supra-renal."},"pl":{"name":"Pierwotny jednostronny przerost nadnerczy","synonyms":["PUAH"]},"cs":{"name":"Primární unilaterální adrenální hyperplázie","synonyms":["PUAH"]},"uk":{"name":"Первинна одностороння гіперплазія надниркових залоз","synonyms":["PUAH"],"definition":"Primary unilateral adrenal hyperplasia (PUAH) is a surgically-correctable form of primary (hyper) aldosteronism (PA; see this term) characterized by renin suppression, unilateral aldosterone hypersecretion, and moderate to severe hypertension secondary to hyperplasia of the adrenal gland."},"zh":{"name":"原发性单侧肾上腺过度增生","synonyms":["PUAH"],"definition":"原发性单侧肾上腺增生症（PUAH）是一种可通过外科手术纠正的原发性（高）醛固酮增多症（PA；见该词条），其特征是肾素分泌抑制、单侧醛固酮分泌增多和继发于肾上腺增生的中重度高血压。"},"tr":{"name":"Primer tek taraflı adrenal hiperplazi","synonyms":["PUAH"]}},"xrefs":[{"source":"ICD-10","code":"E26.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"5A72.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1653668765"},{"source":"MONDO","code":"0016504","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4274967","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":231637,"name":"Rare surgically correctable form of primary aldosteronism","classifications":[193]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"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":"2016-08-31","source":"17180480[PMID]_25695882[PMID]_22958674[PMID]_23158191[PMID]_26934393[PMID]_27036860[PMID]","terms":[{"hpo":"HP:0003351","label":"Decreased circulating renin concentration","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0011740","label":"Glucocortocoid-insensitive primary hyperaldosteronism","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0008221","label":"Adrenal hyperplasia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002900","label":"Hypokalemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003081","label":"Increased urinary potassium","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200114","label":"Metabolic alkalosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000421","label":"Epistaxis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003394","label":"Muscle spasm","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001962","label":"Palpitations","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001959","label":"Polydipsia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000360","label":"Tinnitus","frequency":"Occasional (29-5%)","criteria":null}]}}