Background: Secondary PHA is a transient aldosterone resistance condition mostly occurring in relation with urinary system infection and/or malformations. Secondary PHA cases and very few case series have been reported in the literature. In this article, we reported a case series of eight patients including different clinic presentations which have not as yet been reported in the literatüre and their long-term follow-ups.
Method: Patients who have secondary PHA reasons in addition to hyponatremia (<130 mEq/l), hyperpotassemia (>6 mEq/l) and high serum aldosteron levels for the age in Erciyes University Faculty of Medicine Pediatrics Department were included in the study.
Results: All the patients in our case series were younger than 3 months old. Among eight patients in our case series, seven patients were diagnosed with PHA secondary to obstructive uropathy (OUP), one patient was diagnosed with PHA secondary to ileostomy. Six patients were diagnosed with OUP together with urinary tract infection (UTI) and in all except one patient, secondary PHA recovered with only UTI treatment before applying surgical correction. All the patients in our case series were younger than 3 months old. Three patients with PUV diagnosis, salt wasting recurred in an UTI attack recurring under 3 months of age. Although they had an UTI attack in later follow-ups, salt wasting did not develop. Salt supplementation was made with IV/oral NaCl of 3 mEq/kg per day at least and 32 mEq/kg per day at most. The salt supplementation lasted between 3 days and 6 months.
Conclusion: Infants known to have UOP should be closely observed for salt wasting in the presence of urinary tract infection, especially in the early infancy period.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology