Primary Familial Addison's Disease

Primary familial Addison's Disease is a rare disorder in children and may be manifested by either total adrenal failure with salt losing features, or by partial adrenal failure without salt loss. Knowledge of the pathology is limited and so far aplasia, hypoplasia or degeneration of the adrenal cortex has been described. This communication concerns a remarkable family of 5 children, 3 of whom have distinctive clinical and biochemical findings of partial adrenal failure without salt loss.