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Transient pseudohypoaldosteronism

MedGen UID:
900190
•Concept ID:
C4273962
•
Disease or Syndrome
Synonyms: Secondary pseudohypoaldosteronism; TPHA; transient pseudohypoaldosteronism
SNOMED CT: Transient pseudohypoaldosteronism (717263009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019643
Orphanet: ORPHA93164

Definition

A renal tubulopathy characterized by renal tubular resistance to aldosterone, characterized by hyponatremia, metabolic acidosis and hyperkalemia and manifesting as dehydration, secondary to urinary tract malformation and infections in infants. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTransient pseudohypoaldosteronism

Recent clinical studies

Etiology

Transient pseudohypoaldosteronism: a potentially severe condition affecting infants with urinary tract malformation.
Delforge X, Kongolo G, Cauliez A, Braun K, Haraux E, Buisson P
J Pediatr Urol 2019 May;15(3):265.e1-265.e7. Epub 2019 Mar 9 doi: 10.1016/j.jpurol.2019.03.002. PMID: 30962012
Transient pseudohypoaldosteronism in infancy secondary to urinary tract infection.
Abraham MB, Larkins N, Choong CS, Shetty VB
J Paediatr Child Health 2017 May;53(5):458-463. Epub 2017 Feb 24 doi: 10.1111/jpc.13481. PMID: 28233358
Congenital primary adrenal insufficiency and selective aldosterone defects presenting as salt-wasting in infancy: a single center 10-year experience.
Bizzarri C, Olivini N, Pedicelli S, Marini R, Giannone G, Cambiaso P, Cappa M
Ital J Pediatr 2016 Aug 2;42(1):73. doi: 10.1186/s13052-016-0282-3. PMID: 27485500Free PMC Article
Transient pseudohypoaldosteronism with hyponatremia-hyperkalemia in infant urinary tract infection.
Schoen EJ, Bhatia S, Ray GT, Clapp W, To TT
J Urol 2002 Feb;167(2 Pt 1):680-2. doi: 10.1016/S0022-5347(01)69124-9. PMID: 11792953
Transient pseudohypoaldosteronism in obstructive renal disease.
Pumberger W, Frigo E, Geissler W
Eur J Pediatr Surg 1998 Jun;8(3):174-7. doi: 10.1055/s-2008-1071148. PMID: 9676402

Diagnosis

Transient pseudohypoaldosteronism: a potentially severe condition affecting infants with urinary tract malformation.
Delforge X, Kongolo G, Cauliez A, Braun K, Haraux E, Buisson P
J Pediatr Urol 2019 May;15(3):265.e1-265.e7. Epub 2019 Mar 9 doi: 10.1016/j.jpurol.2019.03.002. PMID: 30962012
Transient Pseudohypoaldosteronism Caused by Intestinal Abnormalities.
Watanabe T
Klin Padiatr 2018 Mar;230(2):104. Epub 2017 Dec 13 doi: 10.1055/s-0043-120068. PMID: 29237184
An infant presenting with failure to thrive and hyperkalaemia owing to transient pseudohypoaldosteronism: case report.
De Clerck M, Vande Walle J, Dhont E, Dehoorne J, Keenswijk W
Paediatr Int Child Health 2018 Nov;38(4):277-280. Epub 2017 May 30 doi: 10.1080/20469047.2017.1329889. PMID: 28557682
Transient pseudohypoaldosteronism in infancy secondary to urinary tract infection.
Abraham MB, Larkins N, Choong CS, Shetty VB
J Paediatr Child Health 2017 May;53(5):458-463. Epub 2017 Feb 24 doi: 10.1111/jpc.13481. PMID: 28233358
Transient pseudohypoaldosteronism secondary to posterior urethral valves--a case report and review of the literature.
Bülchmann G, Schuster T, Heger A, Kuhnle U, Joppich I, Schmidt H
Eur J Pediatr Surg 2001 Aug;11(4):277-9. doi: 10.1055/s-2001-17151. PMID: 11558022

Therapy

An infant presenting with failure to thrive and hyperkalaemia owing to transient pseudohypoaldosteronism: case report.
De Clerck M, Vande Walle J, Dhont E, Dehoorne J, Keenswijk W
Paediatr Int Child Health 2018 Nov;38(4):277-280. Epub 2017 May 30 doi: 10.1080/20469047.2017.1329889. PMID: 28557682

Prognosis

Transient pseudohypoaldosteronism in infancy secondary to urinary tract infection.
Abraham MB, Larkins N, Choong CS, Shetty VB
J Paediatr Child Health 2017 May;53(5):458-463. Epub 2017 Feb 24 doi: 10.1111/jpc.13481. PMID: 28233358
Transient pseudohypoaldosteronism in obstructive renal disease.
Pumberger W, Frigo E, Geissler W
Eur J Pediatr Surg 1998 Jun;8(3):174-7. doi: 10.1055/s-2008-1071148. PMID: 9676402

Clinical prediction guides

Transient pseudohypoaldosteronism: a potentially severe condition affecting infants with urinary tract malformation.
Delforge X, Kongolo G, Cauliez A, Braun K, Haraux E, Buisson P
J Pediatr Urol 2019 May;15(3):265.e1-265.e7. Epub 2019 Mar 9 doi: 10.1016/j.jpurol.2019.03.002. PMID: 30962012

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