Gonadotrophin deficiency and adrenocortical insufficiency in children.
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Visual average evoked responses (AERs) to 4 intensities of light were studied in 5 patients with adrenal cortical insufficiency. Patients were tested off all medications, on ACTH while off all medications, and during replacement with carbohydrate-active steroids. Off treatment, when steroid levels were low, AERs were of small amplitude and short latency. Treatment with ACTH alone decreased the amplitude-intensity response, suggesting extra-adrenal effects. Both with amplitude and latency effects, stimulus intensity was an important parameter in determining steroid and ACTH effects.
A 36-year-old woman with central diabetes insipidus (DI), diagnosed when she was 7, was referred to our Endocrine Unit in January 1993 for further hormonal investigations. Clinical and laboratory findings confirmed the diagnosis of central DI. Cranial computed tomography and magnetic resonance imaging showed only an empty sella. Moreover, we noted impaired glucose tolerance and unusual findings of subclinical adrenocortical failure, i.e. high plasma renin activity with normal aldosterone levels, high ACTH despite normal basal and ACTH-stimulated cortisol levels. Immunological study of the patient's serum showed the presence of arginine vasopressin (AVP)-secreting cell antibodies (Abs), steroid-producing cell Abs, adrenal and islet cell Abs. The following aspects of our case are stressed and discussed: (1) the presence of AVP-secreting cell Abs 29 years after the diagnosis of DI; (2) the association between DI, empty sella and subclinical autoimmune adrenocortical failure with unusual hormonal findings, and (3) impaired glucose tolerance with islet cell antibody positivity.
In order to investigate the functional significance of possible adrenal amyloid infiltration in patients with renal amyloidosis, we performed corticotrophin stimulation tests in 15 patients having renal amyloidosis with no clinical evidence of adrenocortical insufficiency and 12 controls. In 7 of the patients, the cortisol increments obtained during the test were found to be consistent with primary adrenal insufficiency due to possible amyloid infiltration of the adrenal cortex; in contrast, in all control subjects, adrenal responses to the test were regarded as sufficient for proper adrenal function.
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