Table 1.
Patients’ characteristics and clinical outcome
| Patient no. | 1 | 2 | 3 | 4 | 5 | 6 | 7 |
|---|---|---|---|---|---|---|---|
| Year of presentation | 1986 | 2003 | 2003 | 2005 | 2007 | 2009 | 2015 |
| Sex/age (years) | M/39 | F/32 | F/18 | F/38 | M/37 | M/20 | F/28 |
| Precipitant(s) | HE | P | None | HE | HE | None | HE |
| Platelets (g/L) | 179 | 212 | 20 | 228 | 100 | 345 | 228 |
| MAHA | – | + | + | + | + | + | – |
| LDH (U/L) | 680 | 4,106 | 1,000 | 1,800 | 2,125 | 1,251 | 298 |
| Creatinine (mg/dL) | 12.3 | 15.7 | 10.0 | 19.6 | 6.6 | 3.2 | 12.0 |
| Proteinuria (g/day) | Oliguria | >3.5 | 0.4 | Anuria | 6.4 | 4.5 | 5.5 |
| C3 (g/L) | 0.74 | 0.69 | 0.72 | 0.69 | 0.88 | 0.72 | 0.72 |
| CFH-H3/MCPGGAAC | +/– | –/– | +/– | –/– | +/– | –/– | –/– |
| Kidney biopsy | |||||||
| Pattern on light microscopy | CresGN, ischaemia | DC, thrombi | DC, thrombi | DC, thrombi | DC, mes., thrombi | DC, mes. | DC, mes., thrombi |
| Electron lucent material | + | ND | ND | ND | + | + | + |
| FPE (%) | 80 | ND | ND | ND | >50 | 80 | 40 |
| Neurologic disease | + | – | – | – | – | – | – |
| Cardiac disease | + | – | – | – | + | – | – |
| Plasma exchange | – | + | + | + | + | + | + |
| Follow-up (years) | 30.6 | 15.4 | 16.0 | 11.4 | 11.8 | 3.0 | 4.6 |
| Outcome | ESKD, deceased | ESKD | ESKD | ESKD | ESKD | CKD G1 | ESKD |
| Donor kidney(s) | 3 | 1 | 2 | 2 | 1 | 0 | 1 |
| Graft failure (aHUS) | 3 (2) | 1 (1) | 2 (1) | 2 (2) | 1 (1) | N/A | 0 |
CresGN, crescentic glomerulonephritis; DC, double contour formation of the glomerular basement membrane; F, female; HE, hypertensive emergency; M, male; MAHA, microangiopathic haemolytic anaemia; mes., mesangiolysis; N/A, not applicable; ND, not determined; P, pregnancy; +, positive; −, negative.