In one of two children suffering from a primary sclerosing cholangitis
, the liver disease was clinically the main problem and only later a c
olitis was diagnosed additionally. In the second case, the colitis ulc
erosa was clinically dominant, and only the elevation of serum aminotr
ansferase led to the diagnosis of a mild early form of primary scleros
ing cholangitis. Reviewing the literature the etiological, immunologic
al, diagnostical, clinical and therapeutical aspects of this disease a
re described.