A Salt Wasting Syndrome in Infancy

Abstract

A continued loss of sodium and chloride is a well-known phenomenon which occurs during the course of uncontrolled adrenal insufficiency.Such losses are of serious consequence and in children lead to a failure of growth and development, to asthenia and infection.It is well recognized that many of the symptoms of adrenal insufficiency are due to the disturbance in mineral metabolism, and it is not surprising, therefore, that other conditions where excessive amounts of salt are lost from the body occasionally give rise to a picture that resembles, in many respects, adrenal insufficiency.In passing, we can mention fibrocystic disease of the pancreas, hydrocephalus with arachno-ureteric drainage, patients with heart disease who are hyper- sensitive to mercurial diuretics, cerebral salt wasting, pink disease and salt losing nephritis where there is advanced destruction of the renal parenchyma and tubular resistance to salt-retaining steroids.This communication considers a case which does not seem to fit into any of the categories in the above list.It is that of an infant, studied over a five-month period, who demonstrated a renal salt wasting condition in the face of otherwise normal kidneys and normal adrenal function and acid-base balance.It will be shown that this salt wasting defect was resistant to desoxycorticosterone acetate -and we suggest that the defect may be due to a refractory state on the part of the tubules to endogenous salt-active steroids or mineralo-corticoids.Methods Sodium and potassium concentrations for serum, sweat and urine were determined on a direct reading flame photometer (Domingo and Klyne, 1949), or by a direct reading E.E.L. flame photometer.Chloride determinations, serum pH, carbon dioxide content, total body water, total body chloride, were all measured by methods set out in a previous communi- cation (Cheek, 1957).To determine total chloride the volume of distribution of bromide was measured and a micro diffusion method was used as described previously (Cheek and West, 1955).Here the