ISSN: 2149-2247 | E-ISSN: 2149-2549

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Clinical Course and Follow-Up o Type 1 Pseudohypoaldosteronism [Erciyes Med J]
Erciyes Med J. 2018; 40(3): 113-119 | DOI: 10.5152/etd.2018.0033

Clinical Course and Follow-Up o Type 1 Pseudohypoaldosteronism

Sabriye Korkut1, Leyla Akın2, Nihal Hatipoğlu2, Ahmet Özdemir1, Levent Korkmaz1, Mustafa Kendirci3, Ruhan Düşünsel4, Tamer Güneş1, Mehmet Adnan Öztürk1, Selim Kurtoğlu5
1Division of Neonatology, Department of Pediatrics, Erciyes University Faculty of Medicine, Kayseri, Turkey
2Division of Pediatric Endocrinology, Erciyes University Faculty of Medicine, Kayseri, Turkey
3Division of Pediatric Metabolism, Erciyes University Faculty of Medicine, Kayseri, Turkey
4Division of Pediatric Nephrology, Erciyes University Faculty of Medicine, Kayseri, Turkey
5Division of Neonatology, Department of Pediatrics, Erciyes University Faculty of Medicine; Division of Pediatric Nephrology, Erciyes University Faculty of Medicine, Kayseri, Turkey

Objective: This study aimed to evaluate the management of primary pseudohypoaldosteronism type 1 (PHA1), which is a rare disease.
Materials and Methods: We retrospectively reviewed the hospital records of patients who were followed up with a diagnosis of primary PHA1.
Results: Of the eight patients diagnosed with primary PHA1, two had renal PHA1 and five had systemic PHA1. Five patients were initially administered steroids until a definite diagnosis was made. One patient was initially misdiagnosed with congenital adrenal hyperplasia due to “the high-dose hook effect”. In patients with systemic PHA1, the highest salt requirement was 32–53 mEq/kg/day, which gradually decreased in all patients. Salt supplementation could not be stopped in patients with systemic PHA1 except one patient. Four of the eight patients died.
Conclusion: After excluding the causes that may lead to secondary PHA in the initial evaluation of patients referred with a salt-depletion crisis, a differential diagnosis of congenital adrenal hyperplasia and PHA1 should be made. The hook effect and interference probabilities should be considered for evaluating hormone levels.

Keywords: Pseudohypoaldosteronism type 1, salt-depletion crisis, aldosterone, treatment

Sabriye Korkut, Leyla Akın, Nihal Hatipoğlu, Ahmet Özdemir, Levent Korkmaz, Mustafa Kendirci, Ruhan Düşünsel, Tamer Güneş, Mehmet Adnan Öztürk, Selim Kurtoğlu. Clinical Course and Follow-Up o Type 1 Pseudohypoaldosteronism. Erciyes Med J. 2018; 40(3): 113-119

Corresponding Author: Sabriye Korkut, Türkiye
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