Salt-losers and non salt-losers in congenital adrenal hyperplasia.

نویسنده

  • F C Bartter
چکیده

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Congenital adrenal hyperplasia--a clinical and genetic survey. Are we detecting male salt-losers?

In a genetic and clinical study in Wales of congenital adrenal hyperplasia due to 21-hydroxylase deficiency, 26 cases were found in the period 1966 to 1977. No one was known to have died from the condition in the period. There were 14 female and 5 male salt-losers, and 5 female and 2 male nonsalt-losers. The discrepancy between the sexes suggests that some cases of congenital adrenal hyperplasi...

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Should we screen for congenital adrenal hyperplasia? A review of 117 cases.

A total of 117 patients with congenital adrenal hyperplasia who were under the care of paediatricians at Birmingham Children's Hospital between 1958 and 1985 were reviewed retrospectively. There were 47 boys (40%) and 70 girls (60%); 30 of the 47 boys (64%) and 38 of the girls (58% of the 66 whose salt state was known) were salt losers. In all salt losers the condition was diagnosed before the ...

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Evaluation of deficiency of 21-hydroxylation in patients with congenital adrenal hyperplasia.

Congenital adrenal hyperplasia can manifest itself in a variety of clinical and biochemical abnormalities (Bongiovanni and Root, 1963). The salt-losing tendency in some of these patients can be due to the absence of specific enzymes: dehydrogenases or hydroxylases (Bongiovanni and Root, 1963; Ulick et al., 1964; Visser and Cost, 1964). In addition to the impaired production of certain steroids,...

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Continuing need for mineralocorticoid therapy in salt-losing congenital adrenal hyperplasia.

Four patients with salt-losing congenital adrenal hyperplasia (CAH) who had stopped mineralocorticoid therapy for several years, showed raised plasma concentrations of 17OH-progesterone and plasma renin activity, despite adequate glucoticoid therapy. One patient was able to reduce urinary sodium excretion when the sodium intake was restricted. Another patient who was a salt-loser, developed sig...

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Dissociation of plasma renin activity and plasma aldosterone level during dexamethasone suppression test in non-salt-losers with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

We have studied plasma renin activity, the plasma aldosterone level and urinary metabolites of glucocorticoid precursors before and during a dexamethasone suppression test in three non-salt-losers with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, who had never been treated with glucocorticoid. Plasma renin activity, the plasma aldosterone level and urinary pregnanetriol excr...

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عنوان ژورنال:
  • Archives of disease in childhood

دوره 44 233  شماره 

صفحات  -

تاریخ انتشار 1969