[State of the renin-angiotensin system in children with different clinical variants of congenital adrenal cortex hyperplasia caused by 21-hydroxylase deficiency]. / Sostoianie renin-angiotenzinovoi sistemy u detei s razlichnymi klinicheskimi variantami vrozhdennoi giperplazii kory nadpochechnikov, obuslovennoi defitsitom 21-gidroksilazy.

ABSTRACT

A study was made of plasma renin activity (PRA) and serum aldosterone (A) in 52 children with congenital hyperplasia of the adrenal cortex due to 21-hydroxylase deficiency. There were 40 girls and 12 boys aged 1.5 months to 14 years. Of these, 35 had the salt-losing form (group 1), 12 were with a simple virile form (group 2), and 2 with the typical disease form (group 3). By the moment of the first examination the majority of the group 1 patients had received glucocorticoids (GC) combined with mineralocorticoids (MC), group 2 had been given GC alone, and the group 3 children had been untreated. Initially PRA in both the first and second groups was higher than in the controls. However, in group 2 that difference was insignificant. The concentration of A in group 2 noticeably exceeded that in the controls and group 1. In group 3, PRA and A did not differ from normal. After the treatment was potentiated by MC (including MC administration to the group 2 patients) there was a decline of PRA and of the A level. The changes in PRA and A did not often manifest clinically and were not associated with electrolyte disturbances. In the authors' opinion, to choose adequate doses of MC, it is necessary to examine PRA and blood A level.