Work

Hyperkalaemic acidosis: blood pressure is the diagnostic clue

Year: 2024

Type: article

Abstract: Pseudohypoaldosteronism type 2 (PHA2) is a rare inherited condition of altered tubular salt handling. It is characterized by the specific constellation of hyperkalaemic hyporeninemic hypertension, hyperchloremic metabolic acidosis and hypercalciuria. Molecular genetic testing confirms the diagnosis in the majority of cases. Thiazides constitute effective treatment. Due to its rarity, the diagnosis is often delayed. We here present two children with PHA2, who were initially treated with fludrocortisone and bicarbonate complicated mainly by exacerbation of their hypertension. Discontinuation of their previous therapy and commencement of thiazide diuretics led to normalisation of their blood pressure and electrolyte and acid-base status.

Source: Pediatric Nephrology

DOI: 10.1007/s00467-024-06590-4

Authors: Jakub Zieg, Dana Thomasová, Malgorzata Libik, Zdenek Sumnik, Detlef Bockenhauer

Institutions: Charles University, University Hospital in Motol, Great Ormond Street Hospital, University College London, KU Leuven

Language: English

Topic: Potassium and Related Disorders

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $4,190