[Transient hypoaldosteronism. A case report].

Abstract

We observed a 2-week old boy who developed a typical salt-losing syndrome. Urinary 17-ketosteroid excretion of 2.4 mg per day lead us to the diagnosis of congenital adrenal hyperplasia and the usual treatment with hydrocortisone, DOCA and NcCl orally was started. The 6-months old child will tolerate a reduction and subsequent withdrawal of hydrocortisone. Mineralcorticoid and NaCl treatment, however, is to be continued. Further studies clearly showed that in the 8 and 9-month-old child cortisol production could very well be stimulated by synthetic ACTH, but the base line plasma aldosterone was exceedingly low and stimulation by ACTH and salt depletion was impossible. Instant cortisol as well as aldosterone stimulation occurs not until the child is 14 months old. There is valid evidence for a defect in aldosterone biosynthesis, which may be caused by 18-hydroxylation or 18-dehydrogenation deficiency.