80 G. I. M. SwYER 



to elevation of the plasma potassium, as a result of which 

 early death may occur from dehydration and circulatory 

 collapse, or from cardiac arrest due to hyperkalaemia. Once 

 again, the number of females affected is some three times that 

 of males. 



The mechanism for this sodium loss is not understood. Very 

 likely there is a defect in aldosterone synthesis, but it is also 

 possible that some of the abnormal steroids produced by the 

 hyperplastic adrenals may actually cause sodium loss. It is 

 well known that surprisingly large amounts of sodium 

 chloride and cortexone acetate (DOCA) may be needed to 

 remedy the electrolyte defects in these infants, suggesting 

 that more than mere replacement of deficient hormone is 

 necessary. However, the response to 9a-fluorohydrocortisone, 

 together with cortisone, may be far more satisfactory. In a 

 Ij-year-old patient of the writer's, a female pseudoherma- 

 phrodite with the salt-losing disorder, 10 mg. daily of DOCA 

 intramuscularly, together with large sodium supplements, 

 was necessary to maintain electrolyte balance. With only 

 0-25 mg. of 9a-fluorohydrocortisone daily by mouth, it was 

 possible to maintain balance with no sodium supplement at 

 all. 



A small proportion of patients with adrenal hyperplasia 

 (about 6 per cent) may show hypertension. It is possible that 

 in these there is actually sodium retention. Bongiovanni and 

 Eberlein (1955) have demonstrated in such a patient a defect 

 in the synthesis of Cortisol different from that usually found 

 in adrenal hyperplasia. This patient was producing increased 

 amounts of cortexone and 17-hydroxycortexone; it is thought 

 probable that these steroids were responsible for the hyper- 

 tension. 



Changes in Relation to Adolescence 



Knowledge of endocrine changes in relation to adolescence 

 is rather sketchy. It is ably summarized by Tanner (1955). 

 The impact of these changes on fluid and electrolyte metabol- 

 ism is somewhat obscure. Certain morphological changes of 



