Table 1.
Multi-organ involvement
| Test | At admission | Most abnormal value | At discharge | Normal value or range | |
|---|---|---|---|---|---|
| Differential diagnosis | |||||
| Blood culture | Negative | Negative | |||
| TTP | ADAMTS13 activity (%) | 91% | >65% | ||
| STEC-HUS | Serology | ||||
| O157 IgM/G/A | Negative | ||||
| O26 IgM/G/A | Negative | ||||
| PCR Stx1 gene | Negative | Negative | |||
| Stx2 gene | Positive | Negative | |||
| eae gene | Positive | Negative | |||
| WGS Serotype | O80:H2 | ||||
| Isolate | Stx2d, eae ξ variant | ||||
| Sequence type | ST301 | ||||
| Virulence factors | |||||
| Stx1gene | Negative | ||||
| Stx2gene | Positive | ||||
| eae | Positive | ||||
| aggR | Negative | ||||
| aatA | Negative | ||||
| aHUS | Complement | ||||
| C3 (mg/l) | 874 | NA | NA | 900–1800 | |
| C4 (mg/l) | 71 | NA | NA | 150–400 | |
| Anti factor H autoantibodies | Negative | ||||
| DNA analysis | |||||
| Factor H | No pathogenic variation | ||||
| Factor I | No pathogenic variation | ||||
| Factor B | No pathogenic variation | ||||
| C3 | No pathogenic variation | ||||
| MCP | No pathogenic variation | ||||
| CFHR 1–5 | No pathogenic variation | ||||
| DGKE | No pathogenic variation | ||||
| THBD | No pathogenic variation | ||||
| MLPA Factor H operon | No aberrations | ||||
| Hematological | Hemoglobin (mmol/l) | 4.5 | 3.3 | 7.3 | 6.8–8.6 |
| Platelet count (x109/l) | 22 | 18 | 872 | 210–430 | |
| WBC count (x109/l) | 15.5 | 29.0 | 12.9 | 5.0–17.0 | |
| Schizocytes (%) | >5 | >5 | NA | <0,5 | |
| Haptoglobin (g/l) | Hemolytic | Hemolytic | 1.85a | 0.3–1.6 | |
| LDH (U/l) | 2285 | 6521 | 908 | <250 | |
| Kidney | Creatinine (μmol/l) | 167 | 444 (PD) | 470 (PD) | 15–45 |
| eGFR(ml/min/1.73 m2) | 17 | anuria | anuria | 80–120 | |
| BUN (mmol/l) | 43.9 | 44.2 | 22.4 | 2.5–7.0 | |
| Brain | MRI | Diffusion restriction of the deep white matter consistent with metabolic encephalopathy | NA | NA | |
| EEG | No epileptic activity | NA | NA | ||
| Heart | CK (U/l) | 2743 | 8390 | 139a | <170 |
| Ntpro-BNP (pg/ml) | NA | >180,000 | NA | <320 | |
| Troponin T levels (ng/l) | 557 | 23,444 | 508a | <14 | |
| Echo | NA | Left ventricular dysfunction | Normal left ventricular functiona | ||
| Pancreas | Amylase (U/l) | NA | 1933 | 42a | <105 |
| Glucose (mmol/l) | 9.9 | 30.7 | 6.2 | 4.0–5.6 | |
| Triglycerides (mmol/l) | NA | 14.17 | 4.92 | 0.8–2.0 | |
| Liver | AST (U/l) | 163 | 1020 | 114 | <35 |
| ALT (U/l) | 62 | 480 | 144 | <45 | |
| Gamma-GT (U/l) | 6 | 1810 | 824 | <55 | |
| Alkaline phosphatase (U/l) | 205 | 1529 | 401 | <115 | |
| Direct bilirubin (μmol/l) | 4 | 326 | 35 | <5 | |
| Ultrasound | Edema around and sludge inside gallbladder | ||||
| Biopsy | Cholestasis | ||||
aatA necessary for translocation of dispersin (Aap), ADAMTS13 a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13, aggR transcriptional regulator aggR, aHUS atypical hemolytic uremic syndrome, ALT alanine transaminase, AST aspartate transaminase, BUN blood urea nitrogen, CFHR complement factor H-related proteins, CK creatine kinase, DGKE diacylglycerol kinase epsilon, eae E. coli attachment effacement gene (intimin), EEG electroencephalogram, eGFR estimated glomerular filtration rate based on Schwartz estimation with k-value of 36.5, gammaGT gamma-glutamyl transferase, IgA immunoglobulin A, IgG immunoglobulin G, IgM immunoglobulin M, LDH lactate dehydrogenase, MCP membrane cofactor protein, MLPA multiplex ligation-dependent probe amplification, MRI magnetic resonance imaging, NA not available, Ntpro-BNP N-terminal of the prohormone brain natriuretic peptide, PCR polymerase chain reaction, PD peritoneal dialysis, STEC Shiga toxin-producing Escherichia coli, Stx1 Shiga toxin 1, Stx2 Shiga toxin 2, THBD thrombomodulin, TTP thrombotic thrombocytopenic purpura, WBC white blood cell count, WGS whole genome sequencing
aTwo months after presentation