A Case of Salt-Wasting 21-Hydroxylase Deficiency With Resistance to Aldosterone due to Urinary Tract Infection

Classic salt-wasting 21-hydroxylase deficiency (21-OHD) often requires fludrocortisone (FC) replacement. However, the optimal dose of FC varies between patients and the dose needs to be adjusted depending on the degree of symptoms. Further, the aldosterone resistance due to urinary tract infections...

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Detalles Bibliográficos
Autores principales: Shimakawa, Urara, Shigehara, Keiichi, Kawabe, Yasuhiro, Ouchi, Kazutaka, Mori, Jun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2020
Materias:
Endocrinology/Diabetes/Metabolism
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7779137/
https://www.ncbi.nlm.nih.gov/pubmed/33409011
http://dx.doi.org/10.7759/cureus.11763
Descripción
Sumario:Classic salt-wasting 21-hydroxylase deficiency (21-OHD) often requires fludrocortisone (FC) replacement. However, the optimal dose of FC varies between patients and the dose needs to be adjusted depending on the degree of symptoms. Further, the aldosterone resistance due to urinary tract infections causes salt-wasting symptoms. We recently encountered a patient with 21-OHD who required up to 0.36 mg/day of FC in order to control hyperkalemia despite adequate hydrocortisone (HC) administration. This condition was presumed to be due to aldosterone resistance complications associated with urinary tract infections. Thus, if the initial treatment of 21-OHD with HC and FC is resistant, then one should consider complications that may cause aldosterone resistance, such as urinary tract infections.

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