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Table 8 Investigations recommended in patients identified as having atypical hemolytic uremic syndrome, adapted from [90]

From: Atypical hemolytic uremic syndrome

  Investigations
1. STEC infection Stool or rectal swab: culture for STEC (Mac Conkey for 0157:H7); PCR for Stx
Serum: anti-LPS antibodies against the most common serotypes in the local country
2. Disorders of complement regulation C3, C4 (plasma/serum)
Factor H, Factor I, Factor B (plasma/serum)
Anti-factor H autoantibodies
MCP (surface expression on leucocytes (polynuclear or mononuclear leucocytes by FACS)
Gene mutation analysis in factor H, factor I, MCP, C3, factor B
3. ADAMTS13 deficiency
inherited or acquired classification
Plasma ADAMTS13 activity or dosage (Elisa) ± inhibitor
4. Cobalamin metabolism:methyl malonic aciduria Plasma amino-acid chromatography (high homocysteine, low methionine); urine organic acid chromatography (methyl-malonic aciduria)
± mutation analysis in MMACHC gene
5. HIV Serology
6. Pregnancy, HELLP syndrome Pregancy test, liver enzymes. Investigate as in 2 and 3
7. Miscellaneous Antinuclear antibody, lupus anticoagulant, anti-phospholipid antibodies
  1. STEC: Shiga-toxin producing Escherichia coli; Stx: Shiga-like toxin; PCR: polymerase chain reaction; ADAMTS13: A Desintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13;HIV: human immunodeficiency virus; HELLP: Hemolysis, Elevated Liver enzymes, Low Platelet count; MCP: membrane cofactor protein; FACS: fluorescence-activated cell sorter; MMACHC: methylmalonic aciduria and homocystinuria;