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C3FX<br />

81090<br />

FC3AL<br />

90463<br />

Reference Values:<br />

25-47 U/mL<br />

Clinical References: 1. Gaither TA, Frank MM: Complement. In Clinical Diagnosis and<br />

Management by Laboratory Methods. 17th edition. Edited by JB Henry. Philadelphia, PA, WB Saunders<br />

Company, 1984, pp. 879-892 2. Agnello V: Complement deficiency states. Medicine 1978;57:1-23 3.<br />

Buckley D, Barnes L: Childhood subacute cutaneous lupus erythematosus associated with homozygous<br />

complement 2 deficiency. Pediatr Dermatol 1995;12:327-330<br />

C3 Complement, Functional, Serum<br />

Clinical Information: Complement proteins are components of the innate immune system. There are<br />

3 pathways to complement activation: 1) the classic pathway, 2) the alternative (or properdin) pathway,<br />

and 3) the lectin activation (mannan-binding protein [MBP]) pathway. The classic pathway of the<br />

complement system is composed of a series of proteins that are activated in response to the presence of<br />

immune complexes. The activation process results in the generation of peptides that are chemotactic for<br />

neutrophils and that bind to immune complexes and complement receptors. The end result of the<br />

complement activation cascade is the formation of the lytic membrane attack complex (MAC). The<br />

absence of early components (C1-C4) of the complement cascade results in the inability of immune<br />

complexes to activate the cascade. Patients with deficiencies of the early complement proteins are unable<br />

to clear immune complexes or to generate lytic activity. These patients have increased susceptibility to<br />

infections with encapsulated microorganisms. They may also have symptoms that suggest autoimmune<br />

disease and complement deficiency may be an etiologic factor in the development of autoimmune disease.<br />

C3 is at the entry point for all 3 activation pathways to activate the MAC. C3 deficiency may result in<br />

pneumococcal and neisserial infections as well as autoimmune diseases such as glomerulonephritis.<br />

Complement levels can be detected by antigen assays that quantitate the amount of the protein (C3/8174<br />

Complement C3, Serum). For most of the complement proteins, a small number of cases have been<br />

described in which the protein is present but is non functional. These rare cases require a functional assay<br />

to detect the deficiency.<br />

Useful For: Diagnosis of C3 deficiency Investigation of a patient with undetectable total complement<br />

(CH[50]) level<br />

Interpretation: Low levels of complement may be due to inherited deficiencies, acquired deficiencies,<br />

or due to complement consumption (eg, as a consequence of infectious or autoimmune processes). Absent<br />

C3 levels in the presence of other normal complement values are consistent with a C3 deficiency.<br />

Reference Values:<br />

21-50 U/mL<br />

Clinical References: 1. Davis ML, Austin C, Messmer BL, et al: IFCC-standardization pediatric<br />

reference intervals for 10 serum proteins using the Beckman Array 360 system. Clin Biochem<br />

1996;29(5):489-492 2. Gaither TA, Frank MM: Complement. In Clinical Diagnosis and Management by<br />

Laboratory Methods. 17th edition. Edited by JB Henry. Philadelphia, WB Saunders Company, 1984, pp<br />

879-892 3. O'Neil KM: Complement deficiency. Clin Rev Allergy Immunol 2000;19:83-108 4. Frank<br />

MM: Complement deficiencies. Pediatr Clin North Am 2000;47(6):1339-1354<br />

C3a Level<br />

Reference Values:<br />

0 - 780 ng/mL<br />

<strong>Test</strong> Performed by: National Jewish Health<br />

Advanced Diagnostic <strong>Laboratories</strong><br />

1400 Jackson Street<br />

Denver, CO 80206-2761<br />

Current as of January 4, 2013 7:15 pm CST 800-533-1710 or 507-266-5700 or <strong>Mayo</strong><strong>Medical</strong><strong>Laboratories</strong>.com Page 321

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