Transient pseudohypoaldosteronism
Synonyms: Secondary pseudohypoaldosteronism · TPHA
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Infancy
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Transient pseudohypoaldosteronism | Secondary pseudohypoaldosteronism · TPHA |
| fr | Pseudohypoaldostéronisme transitoire | Pseudohypoaldostéronisme secondaire |
| de | Pseudohypoaldosteronismus, transienter | |
| es | Pseudohipoaldosteronismo transitorio | |
| it | Pseudoipoaldosteronismo transitorio | |
| nl | Transiënt pseudohypoaldosteronisme | Secundair pseudohypoaldosteronisme · TPHA · Voorbijgaand pseudohypoaldosteronisme |
| pt | Pseudohipoaldosteronismo transitório | |
| pl | Przejściowy pseudohipoaldosteronizm | TPHA |
| cs | Přechodný pseudohypoaldosteronismus | TPHA |
| uk | Транзиторний псевдогіпоальдостеронізм | Вторинний псевдогіпоальдостеронізм · TPHA |
| zh | 一过性假性醛固酮减少症 | 继发性假性低醛固酮血症 |
| tr | Geçici psödohipoaldosteronizm | TPHA · Sekonder psödohipoaldosteronizm |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | N15.8 | NTBT | attributed | validated |
| ICD-11 | GB90.4Y | NTBT | index | validated |
| MONDO | 0019643 | E | validated | |
| UMLS | C4273962 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:444916Pseudohypoaldosteronism
rare renal diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 152 cases | Worldwide | Case(s) | Validated | 36090572[PMID] | |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | 36090572[PMID]_ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/93164
- This record as JSON: /data/d/93164.json