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Copyright: ©Author(s) 2026. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution-NonCommercial (CC BY-NC 4.0) license. No commercial re-use. See permissions. Published by Baishideng Publishing Group Inc.
World J Clin Pediatr. Jun 9, 2026; 15(2): 113666
Published online Jun 9, 2026. doi: 10.5409/wjcp.v15.i2.113666
Rare coexistence of aldosterone resistance and adrenal insufficiency in asymptomatic infant with persistent hyperkalaemia: A case report
Fazila H Wan-Nik, Hani A Zulkeflee, Nadirah S Ab Rahim, Salwani T Tuan-Ismail
Fazila H Wan-Nik, Department of Chemical Pathology, School of Medical Sciences, Universiti Sains Malaysia, Kota Bharu 16150, Kelantan, Malaysia
Hani A Zulkeflee, Faculty of Medicine and Health Sciences, Universiti Sains Islam Malaysia, Bandar Baru Nilai, Nilai 71800, Negeri Sembilan, Malaysia
Nadirah S Ab Rahim, Pathology Unit, Faculty of Medicine and Defence Health, National Defence University of Malaysia, Kem Perdana Sungai Besi 57000, Kuala Lumpur, Malaysia
Salwani T Tuan-Ismail, Endocrinology Unit, Department of Chemical Pathology, School of Medical Sciences, Universiti Sains Malaysia, Kota Bharu 16150, Kelantan, Malaysia
Author contributions: Wan-Nik FH, Zulkeflee HA, Ab Rahim SN contributed to manuscript writing and editing, Tuan-Ismail ST contributed to conceptualization and supervision; all authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report.
Conflict-of-interest statement: All authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Salwani T Tuan-Ismail, Associate Professor, Endocrinology Unit, Department of Chemical Pathology, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Kota Bharu 16150, Kelantan, Malaysia. tusti@usm.my
Received: September 1, 2025
Revised: November 2, 2025
Accepted: January 9, 2026
Published online: June 9, 2026
Processing time: 255 Days and 7 Hours
Abstract
BACKGROUND

Hyperkalaemia is a potentially life-threatening electrolyte disturbance, most commonly encountered in neonates and infants with renal or endocrine abnormalities. Persistent hyperkalaemia in an otherwise clinically well child is unusual and warrants careful investigation. This case illustrates a rare coexistence of pseudohypoaldosteronism and adrenal insufficiency in an otherwise well infant, presenting solely with persistent hyperkalaemia.

CASE SUMMARY

We report a 6-month-old male infant who was incidentally found to have persistent hyperkalaemia during hospitalisation for acute bronchiolitis. He remained asymptomatic, with no clinical or electrocardiographic features of hyperkalaemia. Laboratory investigations revealed persistent hyperkalaemia with normal serum sodium and chloride levels, low serum bicarbonate, elevated serum aldosterone, and suppressed plasma renin activity which consistent with features of aldosterone resistance. Further testing demonstrated low basal serum cortisol with inadequate response to synacthen stimulation, while serum 17-hydroxyprogesterone levels were normal, excluding congenital adrenal hyperplasia. The child was managed with hydrocortisone, high-dose fludrocortisone, and potassium-binding resins, with subsequent improvement.

CONCLUSION

This case illustrates a rare coexistence of aldosterone resistance and adrenal insufficiency in an otherwise well infant, presenting solely with persistent hyperkalaemia. It emphasises the importance of considering overlapping endocrine pathologies and highlights the need for early hormonal profiling in infants with unexplained electrolyte disturbances.

Keywords: Hyperkalemia; Aldosterone resistance; Adrenal insufficiency; Electrolyte imbalance; Diagnosis; Case report

Core Tip: A 6-month-old male was incidentally found to have persistent hyperkalaemia despite being asymptomatic with normal electrocardiogram documented during hospitalization for bronchiolitis. Laboratory evaluation showed elevated serum aldosterone, suppressed plasma renin activity, normal serum sodium and chloride with low serum bicarbonate, consistent with aldosterone resistance. Additionally, a short synacthen test confirmed adrenal insufficiency, with normal serum 17-hydroxyprogesterone excluding (congenital adrenal hyperplasia). He required escalating fludrocortisone alongside hydrocortisone and potassium binders, indicating mineralocorticoid resistance. Genetic testing was not performed, but clinical and biochemical findings strongly support coexisting aldosterone resistance and adrenal insufficiency; an exceptionally rare dual endocrinopathy.

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