Abstract
A 10 year old boy is described with a syndrome of adrenal insufficiency due to selective ACTH insensitivity associated with autonomic nervous system disorders and sensory polyneuropathy. Production of glucocorticoids and adrenal androgens were insufficient, but there were no signs of even latent mineralocorticoid insufficiency (after 4 days of low sodium dlet-9mEq/L NaCl/day). In addition to achalasia, defective lacrimation, anisocoria and hyperkeratosis of palmes and soles, we also found defective sweating, permanent cutis anserina (impaired function of smooth hair muscles) and electromyographically proved sensory polyneuropathy, which were not previously reported in this rare syndrome.
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Dumic, M., Radica, A., Jusic, S. et al. ACTH INSENSITIVITY, AUTONOMIC NERVOUS SYSTEM DISORDERS AND SENSORY POLYNEUROPATHY. Pediatr Res 23, 137 (1988). https://doi.org/10.1203/00006450-198801000-00218
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DOI: https://doi.org/10.1203/00006450-198801000-00218