Primary Aldosteronism in a 9-Year-Old Child

Abstract

Primary aldosteronism has provoked a great deal of interest since first described. Many recent reviews of this subject have been published. Consequently the literature is not reviewed extensively here. [See FIG. 8, FIG. 9, FIG. 10 In Source PDF] Hypertension in the pediatric age period is exceedingly important because this diagnosis implies a rather poor prognosis. However, an individual with primary aldosteronism has a form of hypertension which is curable, making the diagnosis of this discase imperative. In this particular case, a 9-year-old boy was seen for a routine school examination at which time hypertension was discovered. The diagnostic procedures were designed for the consideration of disorders of the central nervous system, kidneys, ability for psychologic adjustment, cardiovascular system and, finally, of the endocrine system. In this patient there were coexistent findings of pyelonephritis and primary aldosteronism. From all the evidence presented, it is reasonable to conclude that there was hypersecretion of aldosterone by the adrenal glands of this boy since early infancy. Hypokalemia is associated with increased secretion of aldosterone and has also been shown to predispose the individual to pyelonephritis. Also it is well known that, in the presence of hypokalemia, the kidney is unable to concentrate urine. In view of the facts that the major histopathologic effects of hypokalemia are seen in the collecting tubule and concentration of urine occurs in this part of the nephron, it is possible to assume a direct association. Since surgery, hypokalemia has disappeared and with it so has the polyuria. There has also been improvement in renal concentrating ability, glomerular filtration rate and urea clearance. The renal plasma flow and the ability of the kidney to respond to vasopressin have not yet improved. It is possible that there exists permanent scarring of the kidney and that this is reflected [See FIG. 11. In Source PDF] in depressed renal plasma flow. Similar to the well-documented study of van Buchem and co-workers, who studied an adolescent with primary aldosteronism, there was no evidence of muscular weakness in this case associated with the hypokalemia. Because only a subtotal adrenalectomy was performed, the patient is leading a completely normal life free of medication and the dangers attendant to adrenal insufficiency. Rough measurement of the urinary excretion of other steroid compounds indicates that they are present in amounts expected for the normal child. He has had mild infections since discharge; during none of these has the patient required therapeutic support. Because of the viability of the remaining adrenal tissue it is expected that he will pass through a normal pubescence and adolescence. Finally, it is standard procedure during the investigation of an individual with hypertension to test for the presence of pheochromocytoma. Since 1932, in this hospital there has not been one pheochromocytoma discovered in an individual below the age of 14 years. It is our opinion that it certainly is equally as important to consider seriously the possibility of primary aldosteronism in every child admitted to the hospital with a diagnosis of essential hypertension, because hypertension in children due to primary aldosteronism, as shown in the present report, is a curable disease.