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Assessment of thyroid and adrenal function in patients with familial amyloidotic polyneuropathy

Abstract. This study was performed to evaluate thyroid and adrenal function in patients with familial amyloidotic polyneuropathy. Twenty‐four patients without any clinical overt endocrinological dysfunction were studied. None of the patients showed laboratory evidences of hypo‐ or hyperthyroidism. A short ACTH‐stimulation test was performed in 17 of the patients. A low cortisol response to ACTH stimulation, suggesting adrenocortical insufficiency, was found in four (24%) of the patients, and an intermediate response interpreted as suspected hypofunction was found in three (18%) patients. Low serum dehydroepiandrosterone sulphate levels, suggesting adrenal hypofunction, were found in six (25%) of the patients. We believe that the possibility of glucocorticoid insufficiency should always be considered in patients with familial amyloidotic polyneuropathy.