{"orpha":93164,"name":"Transient pseudohypoaldosteronism","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Secondary pseudohypoaldosteronism","TPHA"],"definition":"A rare renal tubulopathy secondary to urinary tract infection (UTI) and/or urinary tract malformation (UTM) characterized by renal tubular resistance to aldosterone, characterized by hyponatremia, metabolic acidosis, hyperkalemia and inappropriately high serum aldosterone concentration and clinically manifesting as dehydration, vomiting, and poor oral intake.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-93164/","orphanet_url":"https://www.orpha.net/en/disease/detail/93164","names":{"fr":{"name":"Pseudohypoaldostéronisme transitoire","synonyms":["Pseudohypoaldostéronisme secondaire"],"definition":"Tubulopathie rénale rare secondaire à une infection des voies urinaires (IVU) et/ou à une malformation des voies urinaires (MVU), caractérisée par un état de résistance tubulaire rénale à l'aldostérone, par une hyponatrémie, une hyperkaliémie et une acidose métabolique et une concentration sérique d'aldostérone trop élevée. Sur le plan clinique, la maladie se manifeste par une déshydratation, des vomissements et une mauvaise absorption par voie orale."},"de":{"name":"Pseudohypoaldosteronismus, transienter","definition":"Eine seltene renale Tubulopathie, die sekundär zu einer Harnwegsinfektion (UTI) und/oder Fehlbildung der Harnwege (UTM) auftritt und durch eine renale tubuläre Resistenz gegen Aldosteron gekennzeichnet ist, die sich durch Hyponatriämie, metabolische Azidose, Hyperkaliämie und eine unangemessen hohe Aldosteronkonzentration im Serum auszeichnet und sich klinisch durch Dehydratation, Erbrechen und schlechte orale Aufnahme äußert."},"es":{"name":"Pseudohipoaldosteronismo transitorio","definition":"Es una tubulopatía renal rara, secundaria a la infección del tracto urinario (ITU) y/o malformación del tracto urinario (UTM) caracterizada por una resistencia tubular renal a la aldosterona, que se manifiesta como hiponatremia, acidosis metabólica, hiperpotasemia y una concentración sérica de aldosterona inapropiadamente elevada. Clínicamente se expresa como deshidratación, vómitos y dificultades en la ingesta oral."},"it":{"name":"Pseudoipoaldosteronismo transitorio","definition":"Lo pseudoipoaldosteronismo transitorio (TPHA1) è una tubulopatia renale rara, secondaria a un'infezione delle vie urinarie (UTI) e/o a una loro malformazione (UTM). È caratterizzato dalla resistenza dei tubuli renali all'aldosterone, associata a iponatriemia, acidosi metabolica, iperkaliemia e ad una concentrazione elevata di aldosterone nel siero. Dal punto di vista clinico, si manifesta con disidratazione, vomito e difficoltà di alimentazione."},"nl":{"name":"Transiënt pseudohypoaldosteronisme","synonyms":["Secundair pseudohypoaldosteronisme","TPHA","Voorbijgaand pseudohypoaldosteronisme"],"definition":"Een zeldzame niertubulopathie secundair aan infectie (UTI) en/of malformatie (UTM) van urinewegstelsel, gekarakteriseerd door resistentie van niertubulus voor aldosteron, gekenmerkt door hyponatriëmie, metabole acidose, hyperkaliëmie en ongepast hoge concentratie van aldosteron in serum, met dehydratie, braken, en ondermaatse orale inname als klinische manifestaties."},"pt":{"name":"Pseudohipoaldosteronismo transitório"},"pl":{"name":"Przejściowy pseudohipoaldosteronizm","synonyms":["TPHA"]},"cs":{"name":"Přechodný pseudohypoaldosteronismus","synonyms":["TPHA"]},"uk":{"name":"Транзиторний псевдогіпоальдостеронізм","synonyms":["Вторинний псевдогіпоальдостеронізм","TPHA"],"definition":"A rare renal tubulopathy secondary to urinary tract infection (UTI) and/or urinary tract malformation (UTM) characterized by renal tubular resistance to aldosterone, characterized by hyponatremia, metabolic acidosis, hyperkalemia and inappropriately high serum aldosterone concentration and clinically manifesting as dehydration, vomiting, and poor oral intake."},"zh":{"name":"一过性假性醛固酮减少症","synonyms":["继发性假性低醛固酮血症"],"definition":"一种罕见的继发于尿路感染（UTI）和/或尿路畸形（UTM）的肾小管病变，以肾小管对醛固酮的抵抗为特征，表现为低钠血症、代谢性酸中毒、高钾血症和血清醛固酮浓度异常升高，临床表现为脱水、呕吐和胃纳减退。"},"tr":{"name":"Geçici psödohipoaldosteronizm","synonyms":["TPHA","Sekonder psödohipoaldosteronizm"]}},"xrefs":[{"source":"ICD-10","code":"N15.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"GB90.4Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"225064822"},{"source":"MONDO","code":"0019643","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4273962","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93626,"name":"Rare renal disease"},"parents":[{"orpha":444916,"name":"Pseudohypoaldosteronism","classifications":[188]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Infancy"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":152.0,"area":"Worldwide","status":"Validated","source":"36090572[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"36090572[PMID]_ORPHANET"}],"phenotypes":null}