Improvement of refractory pruritus after lipoprotein-apheresis in arthrogryposis-renal failure-cholestasis syndrome.

ABSTRACT

Accumulation of bile acids can lead to invalidating pruritus in cholestatic patients. Few reports exist on the influence of lipoprotein-apheresis (LA) on plasma level of total bile acids (tBA). We report of significant decrease in tBA levels and drastic improvement of pruritus in a 5-year-old girl with arthrogryposis-renal failure-cholestasis syndrome. We present LA as a suitable rescue treatment option in therapy-refractory cholestasis-associated pruritus, at least as bridge until a long-term solution such as entero-biliary anastomosis or transplantation is possible.