Familial and nonfamilial benign recurrent cholestiasis distinguished by plasma disappearance of indocyanine green but not cholylglycine.

To determine whether a defect in uptake of organic anions is present in patients with benign recurrent cholestasis, the plasma disappearance of simultaneously injected indocyanine green and cholyglycine-1-14C was measured in eight patients with asymptomatic benign recurrent cholestasis, four with the familial type, as well as 22 healthy control subjects. Evans blue was also simultaneously injected to permit correction for variation in blood volume and speed of mixing. Uptake of indocyanine green was decreased in all three patients with nonfamilial benign recurrent cholestatsis, but normal or increased in the four patients uith the familial form of the disorder. By contrast, cholyglycine-1-14C uptake was abnormal in one patient with each type of the disorder. Thus, patients with nonfamilial benign recurrent cholestasis have a defect in indocyanine green uptake, and patients with benign recurrent cholestasis are heterogeneous with respect to organic anion uptake.