Table 1.

Main clinical and biological features of the three children with anti-FH antibodiesa

Patient 1Patient 2Patient 3
Family historyNoneNoneNone
Age at onset (yr)1039
First symptoms
 fever+
 vomiting++
 diarrhea (bloody)+ (+)+15 days before (−)
 anuria/hypertension/edema+/+/+−/−/−−/+/−
 otherJaundice
Biological evaluation at the acute phase
 hemoglobin (g/dl)5.15.67.1
 schizocytes (%)1353
 platelet counts (×109/L)519319
 creatinine (μmol/L)1218113236
Evolution
 recurrencesYes: 2 in 1 moYes: 4 in 17 moYes: 3 in 3 mo
 extrarenal organs involvementYes: liver, pancreas,Yes: heart, at the first relapseNo
 end stage-renal failureYes: 2 mo after the onsetNoNo
 hypertensionYes: needing binephrectomyYesYes
Actual treatmentChronic hemodialysisPlasma exchanges 1/3 wkAntihypertensive drugs
Antihypertensive drugsLow dose of glucocorticoids
Azathioprine
Etiologic evaluation
 anti-LPS Escherichia coli antibodyNegNegNeg
 stool Stx genes (PCR)NegNegNeg
 ADAMST-13 activityNormalNormalNormal
 plasma homocysteineNormalNormalNormal
 FH gene (23 exons)NormalNormalNormal
Immunologic evaluation
 ANA titer, pattern1:320, speckled1:100, speckled1:200, speckled
 anti-DNA, anti-ENANeg, NegNeg, NegNeg, Neg
 anti-cardiolipin, anti-B2gp1Neg, NegNeg, NegNeg, Neg
 ANCANegNegNeg
  • a FH, factor H; Stx, shigatoxin; ANA, antinuclear antibodies; ANCA, antineutrophil cytoplasmic antibodies; ENA, extractable nuclear antigens.