RESUMO
Fourteen patients with sickle cell anemia, ages 6 to 20 years, were studied while ingesting high- and low-sodium diets. Although none of the patients had excessive urinary loss of sodium, the majority had elevated plasma renin activities and aldosterone secretion rates. The PRA was higher in patients over 10 years of age; ASR in patients receiving the high-sodium diet increased with age. Patients with sickle cell anemia appeared to compensate for urinary sodium loss between crises. The mechanism of this loss could be a defect in the function of either the distal tubule or the loop of Henle.
Assuntos
Anemia Falciforme/sangue , Hiperaldosteronismo/sangue , Renina/sangue , Adolescente , Adulto , Fatores Etários , Aldosterona/sangue , Criança , Dieta , Dieta Hipossódica , Eletrólitos/sangue , Humanos , Masculino , Sódio/metabolismoRESUMO
Therapy of congenital adrenal hyperplasia tranditionally is monitored by the amount of urinary 17-KS. However, 24-hour urine collections are difficult to obtain and are often unreliable. Measurement of the plasma concentrations of androgens, such as delta or T, would therefore be a more convenient way to determine the efficacy of treatment. Over a period of 2 to 24 months, 23 patients were periodically assessed by clinical examination, bone age, and determinations of plasma delta, plasma T, and 24-hour urinary 17-KS. Plasma T concentration correlated well with clinical control in females and in preadolescent males, but not in infant and pubertal males. By contrast, plasma delta concentration correlated well with clinical control in either sex, regardless of stage of puberty. The present study suggests that monitoring plasma delta concentration is useful in the long-term management of patients with CAH.