Table 3.

Outcome of the first episode of aHUS and at 3 years after onset

AbnormalityCFHCFIC3THBDMCPCFH AntibodiesNone
OverallSporadicFamilialOverallSporadicFamilialOverallSporadicFamilialOverallSporadicFamilialOverallSporadicFamilialOverallSporadicFamilialOverallSporadicFamilial
Outcome of the first episodeTotala623032106412841367171258812810919
Remission50%b67%c34%40%b33%50%42%b37%50%54%67%43%94%92%100%63%62%63%65%47%
Complete remission10%b13%6%30%b33%25%33%b25%50%23%b33%14%88%83%100%25%b25%23%b22%26%
Partial remission40%53%28%10%25%8%13%31%33%29%6%8%38%37%40%43%21%
ESRF-Death50%b33%c66%60%b67%50%58%b63%50%46%33%57%6%8%37%37%37%35%53%
ESRF31%33%28%60%b67%50%58%b63%50%15%33%6%8%37%37%33%34%32%
Death19%d38%31%d57%4%1%21%
Outcome at 3 yearsTotala643034106412841367171258811910118
Remission23%b,d40%e9%40%b33%50%33%f25%50%46%67%29%94%92%100%37%b37%50%b53%28%
Complete remission4%b7%3%30%b33%25%33%b,f25%50%23%b33%14%88%83%100%12%b12%18%b17%22%
Partial remission19%33%6%10%25%23%33%14%6%8%25%25%32%36%6%
ESRF-Death77%b,d60%e91%60%b67%50%67%b75%50%54%33%71%6%8%63%b62%50%b47%72%
ESRF53%b53%53%60%b67%50%67%b75%50%23%33%14%6%8%63%b62%43%b44%39%
Death23%7%38%31%57%7%3%33%
  • Complete remission is defined as normalization of both hematologic parameters (Ht > 30%; Hb > 10 g/dl; LDH < 460 U/L; plts > 150,000/μl) and renal function (s-creatinine < 1.3 mg/dl).

    Partial remission is defined as normalization of hematologic parameters with renal sequelae (chronic renal failure and/or proteinuria >0.2 g/24 h). CFH group includes also patients with CFH-CFHR1 hybrid gene (all familial cases) and two patients with CFH mutations and CFH autoantibodies.

    All P values were computed using Bonferroni's correction for multiple tests.

  • a Number of patients for whom data are available.

  • b P < 0.0024 compared with patients with MCP mutations (overall).

  • c P = 0.011 versus familial, χ2 test.

  • d P < 0.0024 compared with the group without mutations (overall).

  • e P = 0.003 versus familial, χ2 test.

  • f P < 0.0024 compared with patients with CFH mutations (overall).