Abstract
As postulated by Nelson and Sprunt and confirmed by Besser and Landon, Cushing's disease can be subdivided into stages based on plasma-ACTH. All patients supposedly fail to show a normal circadian ACTH-rhythm. Hypothalamo-pituitary function was studied in a girl adrenalectomized at age 12 for Gushing's syndrome with bil. adrenal hyperplasia, in whom Nelson's syndrome developed 2 years later. There was marked sellar enlargement and moderate hyperpigmentation. Plasma-ACTH values at 9:30, 12:30, 15:30, 18:30, 21:30, 0:30, 3:3o and 6:30 were: 3575, 725, 2560, 2750, 2880, 3700, 5900 and 6310 pg/ml (Amersham-Kit). There was subnormal response to consecutive arginine/insulin stimulation for HGH with a peak of 3.6 ng/ml. TSH-values during a TRF-test at 0, 10, 15, 20, 30, 45, 60 and 90 minutes were: 9.3, 11.5, 10.A, 14.2, 14.5, 11.9, 14.6 and 12.3 uU/ml.
Menstrual periods were normal as were LH- and FSH-responses during LHRF-test. Without aggressive therapy the course was benign so far. It is concluded that ACTH circadian rhythm may be preserved even at an extreme level in children with the Nelson syndrome.
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Petrykowski, W. MAINTAINED CIRCADIAN RHYTHM OF PLASMA-ACTH IN A GIRL WITH NELSON SYNDROME. Pediatr Res 9, 673 (1975). https://doi.org/10.1203/00006450-197508000-00050
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DOI: https://doi.org/10.1203/00006450-197508000-00050