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Table 4 Methods for assessment of plasma and membrane complement proteins and screening for anti- factor H antibodies

From: Atypical hemolytic uremic syndrome

Plasma or membrane complement

Proteins

Plasma concentration

(mg/L) (- 2 to + 2 SD) or membrane expression

Technique

Laboratory

Interpretation

C3

660-1250

Nephelometry

Basic complement screen

Severe complement consumption through the alternative pathway indicated by very low plasma levels of C3 and CFB. Frequently, there is only an isolated moderate decrease of C3 level with normal CFB level

CFB

93-380

Nephelometry

Specialized diagnostic

 

CFH

330-680

(no international standard)

ELISA

Specialized diagnostic

CFH or CFI less than 60% of normal are compatible with quantitative deficiency

CFI

40-80

(no international standard)

ELISA

(or radial immunodiffusion)

Specialized diagnostic

 

Anti-CFH Ab

Screening

ELISA

Specialized diagnostic

The title is expressed in Arbitrary Units (AU)

MCP

Mean fluorescent intensity (MFI)

FACS (a) with anti- MCP phycoerythrin -conjugated antibodies

Specialized diagnostic

No MCP expression is detected in patients with homozygous MCP deficiency. The MFI in patients of heterozygous MCP deficiency is around 50% of the normal range

  1. (a) Usually performed on peripheral granulocytes or mononuclear cells in EDTA-blood sample.
  2. Elisa, enzyme-linked immunosorbent assay; EDTA, ethylene diamine tetra-acetic acid; FACS, fluorescence-activated cell sorter.