Ambulatory treatment with intravenous norepinephrine in a patient with end stage renal disease and generalized AA amyloidosis.

A 35-year-old man with juvenile rheumatoid arthritis and generalized AA amyloidosis of 10 years duration developed end stage renal failure. Following appendectomy, the patient experienced progressive circulatory failure which required IV treatment with norepinephrine. All attempts to discontinue IV norepinephrine failed, each leading to recurrent life-threatening hypotension. Finally, a central venous port with a portable mechanical infusion pump system was implanted supplying a continuous norepinephrine infusion. The patient then became independently mobile and could be discharged. For three months, the patient was monitored as an outpatient and treated by ambulatory intermittent hemofiltration. Finally, the patient suffered from a hemorrhagic infarction of the small bowel due to postoperative adhesions and died shortly after surgery. At autopsy, advanced generalized AA amyloidosis was found. Amyloid deposits had almost entirely replaced the cortex and the medulla of the adrenal glands. It can be speculated that the requirement of exogenous norepinephrine may be in part due to an adrenal insufficiency whereas it was initially considered as being only related to cardiac involvement. A continuous ambulatory treatment with catecholamines could be a possible treatment - at least temporarily - in amyloid cases in which all other attempts have failed to prevent chronic life-threatening hypotension.