Hypocomplementemia discloses genetic predisposition to hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: role of factor H abnormalities
M NORIS, P RUGGENENTI, A PERNA… - Journal of the …, 1999 - journals.lww.com
Familial hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP)
carry a very poor outcome and have been reported in association with decreased serum
levels of the third complement component (C3). Uncontrolled consumption in the
microcirculation, possibly related to genetically determined deficiency in factor H—a
modulator of the alternative pathway of complement activation—may account for decreased
C3 serum levels even during disease remission and may predispose to intravascular …
carry a very poor outcome and have been reported in association with decreased serum
levels of the third complement component (C3). Uncontrolled consumption in the
microcirculation, possibly related to genetically determined deficiency in factor H—a
modulator of the alternative pathway of complement activation—may account for decreased
C3 serum levels even during disease remission and may predispose to intravascular …