Abstract
A 14 years old boy was observed with constant hypertension (up to 170/100 mm Hg), hyperkalaemia (up to 8.2 mEq/1) and metabolic acidosis (blood HCO-3 16 mEq/1) for over 6 years. The somatic development was normal. CIN and CPAH proximal and distal tubular functions (including response to an acid loading test) were within the normal limits except for a lowered bicarbonate threshold (19,3 mEq/1). Total body potassium (K), plasma renin and aldosterone levels as well as secretion rates of hydrocortisone, aldosterone, corticosterone, DOC and substance S were normal. There was an inverse relationship of serum K and bicarbonate levels (r= -0.672, p<0.001). No correlation could be shown between serum K and urinary K excretion, nor between urinary Na and K excretion. Mean arterial blood ressure was correlated to serum K (r= 0.714; p <0.001) but not to urinary Na excretion. By dietary restriction serum K and hypertension were diminished but not normalised. The clinical findings point to an abnormal mechanism of renal handling of K which has not been previously described.
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Mehls, O., Manz, F. & Schärer, K. A syndrome of hypertension, hyperkalaemia and acidosis with normal renal and adrenal functions. Pediatr Res 8, 894 (1974). https://doi.org/10.1203/00006450-197411000-00012
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DOI: https://doi.org/10.1203/00006450-197411000-00012