A Reversible Salt-wasting Syndrome of the Newborn and Infant: Possible Infantile Hypoaldosteronism.

The suggestion that transient states of primary adrenal hypofunction confined to salt conservation can occur in infancy has been most convincingly supported by Jaudon (1946, 1948). Many of his cases, however, showed varying combinations of hyperpigmentation, hypoglycaemia and acidosis, with some having diminished, and others increased, steroid excretion. On the other hand, in recent reports of infants with transient salt-losing syndrome (Cheek and Perry, 1958; Donnell, Litman and Roldan, 1959; Lelong, Alagille, Phillipe, Gentil and Gabilan, 1960), this has been ascribed to a renal tubular insensitivity to salt-retaining hormones. Raine and Roy (1962), in their discussion of a further example of this condition, consider this explanation unlikely, pointing out that a similar insensitivity to D.C.A. exists in other conditions where a primary renal tubular dysfunction is not likely to be present. We present a study of two children with manifestations of a salt-losing syndrome within the first month of life. Anorexia and frequent vomiting resulted in a large initial loss of weight and subsequent failure to gain. Constipation and hyperirritability were also present and there was weakness, hypotonia and dehydration with episodes of tachycardia, pallor and collapse, culminating in peripheral circulatory failure. The dependence of these two infants upon a combination of D.C.A. and salt for clinical recovery and electrolyte homeostasis appeared to be specific, but was transient and ended between the first and third years of life. Although many of the features described by Jaudon were not seen in our infants, several of his cases were very similar to ours. In view of the evidence of renal tubular response to D.C.A. or A.C.T.H., as distinct from salt replacement, it appears unlikely that a primary renal defect could underlie the syndrome we describe. The investigations indicate a fractional adrenocortical insufficiency, possibly confined to aldosterone secretion. The occurrence in infancy of a reversible hyponatraemic state resulting from adrenocortical dysfunction, whether primary or secondary, has been questioned (Wilkins, 1957). We hope that the investigations undertaken on these two cases will help to substantiate the existence of this syndrome.