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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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
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author Shimakawa, Urara
Shigehara, Keiichi
Kawabe, Yasuhiro
Ouchi, Kazutaka
Mori, Jun
author_facet Shimakawa, Urara
Shigehara, Keiichi
Kawabe, Yasuhiro
Ouchi, Kazutaka
Mori, Jun
author_sort Shimakawa, Urara
collection PubMed
description 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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spelling pubmed-77791372021-01-05 A Case of Salt-Wasting 21-Hydroxylase Deficiency With Resistance to Aldosterone due to Urinary Tract Infection Shimakawa, Urara Shigehara, Keiichi Kawabe, Yasuhiro Ouchi, Kazutaka Mori, Jun Cureus Endocrinology/Diabetes/Metabolism 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. Cureus 2020-11-29 /pmc/articles/PMC7779137/ /pubmed/33409011 http://dx.doi.org/10.7759/cureus.11763 Text en Copyright © 2020, Shimakawa et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Endocrinology/Diabetes/Metabolism
Shimakawa, Urara
Shigehara, Keiichi
Kawabe, Yasuhiro
Ouchi, Kazutaka
Mori, Jun
A Case of Salt-Wasting 21-Hydroxylase Deficiency With Resistance to Aldosterone due to Urinary Tract Infection
title A Case of Salt-Wasting 21-Hydroxylase Deficiency With Resistance to Aldosterone due to Urinary Tract Infection
title_full A Case of Salt-Wasting 21-Hydroxylase Deficiency With Resistance to Aldosterone due to Urinary Tract Infection
title_fullStr A Case of Salt-Wasting 21-Hydroxylase Deficiency With Resistance to Aldosterone due to Urinary Tract Infection
title_full_unstemmed A Case of Salt-Wasting 21-Hydroxylase Deficiency With Resistance to Aldosterone due to Urinary Tract Infection
title_short A Case of Salt-Wasting 21-Hydroxylase Deficiency With Resistance to Aldosterone due to Urinary Tract Infection
title_sort case of salt-wasting 21-hydroxylase deficiency with resistance to aldosterone due to urinary tract infection
topic Endocrinology/Diabetes/Metabolism
url 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
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