Abstract
At the end of conventional ACTH therapy of infantile spasms sudden deteriorations of the infant's condition and even deaths are not rare. We followed 10 infants during and after carboxymethylcellulose ACTH therapy. 80 IU was given at 8 a.m. for 3 weeks and 40 IU for 2 weeks, with tapering during and termination at the end of the 6th week. During therapy 24h urinary cortisol increased 100-fold, morning plasma cortisol did not increase, and plasma aldosterone decreased slightly. After therapy, cortisol and AVP levels fell precipitously, cortisol remained subnormal for >2 weeks. Plasma renin and aldosterone peaked abruptly. Urine flow decreased and body weight increased sharply. Plasma cortisol response to AVP remained decreased for >2 weeks. Plasma cortisol response to ACTH was shortened at 3d and diminished at 1 to 2 weeks, indicating suppression of ACTH secretion. The risk at the end of ACTH therapy appears to be associated with 1) sudden subsidence of cortisol hypersecretion resulting in hypocortisolism and hypomineralocorticoidism 2) abrupt activation of the renin-angiotensin-aldosterone axis subsequent to the hypomineralocorticoidism, and 3) impairment of water excretion subsequent to the hypocortisolism.
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