Isolated adrenal mineralocorticoid deficiency due to amyloidosis associated with familial Mediterranean fever.

A patient with familial Mediterranean fever (FMF) associated with renal amyloidosis, presented with hyperkalemia and acidosis which were excessive to his moderate degree of azotemia. The cause of this abnormality was isolated hypoaldosteronism with otherwise normal adrenal function and tubular capacity to transport potassium. This selective involvement of the zona glomerulosa stands in marked contrast to the usual sparing of the glomerulosa seen in post mortem studies of patients with FMF and amyloidosis reported from this country.