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Case Reports
. 2020 Nov 29;12(11):e11763.
doi: 10.7759/cureus.11763.

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

Urara Shimakawa  1 , Keiichi Shigehara  1 , Yasuhiro Kawabe  1 , Kazutaka Ouchi  1 , Jun Mori  2
Affiliations
Case Reports

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

Urara Shimakawa et al. Cureus. .

Abstract

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.

Keywords: 21-hydroxylase deficiency; fludrocortisone; hyperkalemia; pseudohypoaldosteronism; urinary tract infection.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. The photograph of pigmentation of the genital area show
Figure 2
Figure 2. Ultrasound imaging of hydronephrosis
(a) Left kidney has renal pelvis dilation, showing grade 1 hydronephrosis. (b) Right kidney without renal pelvis dilation.
Figure 3
Figure 3. Post-admission course
ABPC, ampicillin; CTX, cefotaxime; Glu, blood glucose level; GI, glucose-insulin; HDC, hydrocortisone; FC, fludrocortisone; 17-OHP, 17-hydroxyprogesterone
See this image and copyright information in PMC

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