Transient early-childhood hyperkalaemia without salt wasting, pathophysiological approach of three cases

Two types of early childhood hyperkalemia had been recognized, according to the presence or absence of urinary salt wasting. This condition was attributed to a maturation disorder of aldosterone receptors and is characterized by sustained hyperkalemia, hyperchloremic metabolic acidosis (MA) due to reduced ammonium urinary excretion and bicarbonate loss, and normal creatinine with growth delay. We present 3 patients of the type without salt wasting, which we will call transient early-childhood hyperkalemia (TECHH) without salt wasting, and discuss its physiopathology according to new insights into sodium and potassium handling by the aldosterone in distal nephron. In 3 children from 30 to 120-day-old admitted with bronchiolitis and growth delay hyperkalemia was found in routine laboratory. Further studies revealed a normal creatinine with inappropriately normal or low fractional excretion (FE) of potassium, accompanied by inadequately normal serum aldosterone and plasma renin activity for their higher plasma potassium levels, but without urine salt wasting. They also presented hyperchloremic MA with FE of bicarbonate 0.58%-2.2%, positive urinary anion gap during MA and normal ability to acidify the urine. Based on these findings a diagnosis of TECHH without salt wasting was made and they were treated sodium bicarbonate and hydrochlorothiazide with favorable response. The condition was transient in all cases leading to treatment discontinuation. Given that TECCH without salt wasting is a tubular disorder of transient nature with mild symptoms; it must be keep in mind in the differential diagnosis of hyperkalemia in young children.