6359 Population surveillance study in Wales of infants presenting with hyponatraemia and hyperkalaemia; remember CAH and renal causes

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Objectives Infants presenting with life threatening hyponatraemia and hyperkalaemia present a diagnostic conundrum that can reflect abnormally low aldosterone production (e.g. congenital adrenal hyperplasia (CAH), renal resistance to aldosterone associated with infec ted urinary tract malformations or single gene disorders also associated with failure of the kidney to respond to aldosterone (pseudohypoaldosteronism). Although incidence figures for individual conditions exist, no overall data exists for presentation of this characteristic la boratory pattern and underlying causes. We aimed to report the incidence, aetiology and outcome of infants presenting with hyponatraemia and hyperkalaemia. Methods Population-based surveillance study July2021-June2023 (Wales population 3.1 million1 with 28,879 live births/year2). Cases were identified by paediatricians using the monthly electronic Welsh Paediatric Surveillance Unit(WPSU) reporting system.3 4 Hospital biochemists and tertiary specialists were recruited to optimise ascertainment. Inclusion criteria: term infants<12 months with sodium<130 mmol/L(<2.5 SD) AND Potassium>5.5 mmol/L(>2.5 SD). Results 13 notifications were reported with 2 additional cases identified by receiving subspecialists; 10 met the inclusion criteria. The incidence of hyponatraemia and hyperkalaemia was 1.7cases/10,000 infants per year. Eventual diagnoses were: 3 CAH, 4 transient pseudohypoaldosteronism (3 with underlying urinary infection and urinary tract malformation), 1 with maternal hyponatraemia, 2 transient electrolyte abnormalities on NNU. All 10 were white ethnicity infants with birth weights >3kg, (5/10 male). Infants presented at median 14 days old (1 hour to 3 months). 4 were shocked or with >10% weight loss. All received 0.9% normal saline fluid boluses with 1/10 receiving insulin for h yperkalemia. Serum sodium and potassium corrected by median one day after admission (range 0–3 and 0–2 days respectively). Electrolyte abnormalities led to diagnosis of urinary tract malformations in those with urinary infections. Patients were discharged after median 6 days (range 3–17). One infant represented after one week with a seizure with normal neuro -imaging and examination. No other sequelae were reported at one month follow up. No deaths occurred. Conclusion This is the first ever study of infants presenting with severe hyponatraemia and hyperkalaemia across a whole population. Severe hyponatraemia and hyperkalaemia is rare. Normalisation of biochemistry derangement was rapid and short-term outcome favourable. When faced with this unusual clinical picture remember CAH and renal causes; early ultrasound may help to delineate the cause. References www.ons.gov.uk/peoplepopulationandcommunity/populationandmigration/populationestimates/bulletins/populationandhouseholdestimateswales/census2021 www.gov.wales/maternity-and-birth-statistics-2021-html Sibert JR, Morgan RJ, O’Connell HI, Lynn R, Guildea ZE, Palmer SR; Welsh Paediatric Society-WPSU study group. Short report: Is regional paediatric surveillance useful? Experience in Wales. Arch Dis Child. 2001 Jun;84(6):486–7. doi: 10.1136/adc.84.6.486. PMID: 11369563; PMCID: PMC1718781. https://www.welshpaediatrics.org.uk/wpsu.

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