Immunology: C3 deficiency
Introduction
- RARE autosomal recessive immunodeficiency disease.
- Cause: characterized by deficiency of serum C3 (complement component).
- Signs: increased incidence of sepsis and local bacterial infections in homozygous animals.
- Predominantly gram-negative bacteria involved and Clostridia Clostridium perfringens.
- Reported in single colony of Brittany Spaniel (see Sources - Blumet al).
Presenting signs
- Recurrent sepsis and local bacterial infections.
- Rarely immune complex glomerulonephritis or renal amyloidosis Amyloidosis.
Age predisposition
- Young.
Breed/Species predisposition
- Brittany Spaniel Brittany.
Pathogenesis
Pathophysiology
- C3 deficiency is associated with inability to opsonize and lyse bacteria.
- Animals homozygous for this genetic defect have no detectable C3.
- Heterozygotes have approximately 50% of normal levels.
- Clinical signs are seen in homozygotes.
Diagnosis
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Treatment
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Prevention
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Outcomes
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Further Reading
Publications
Refereed papers
- Recent references from PubMed and VetMedResource.
- Ameratunga, R, Winkelstein J A, Brody L, Binns M, Cork L C, Colombani P & Valle D (1998) Molecular analysis of the third component of canine complement (C3) and identification of the mutation responsible for hereditary canine C3 deficiency. J Immunology 160 (6), 2824-2830 PubMed.
- Guilford W G (1987) Primary immunodeficiency diseases of dogs and cats. Comp Contin Educ 9 (6), 641-650 VetMedResource.
- Blum J R et al (1985) The clinical manifestations of a genetically determined deficiency of the third component of complement in the dog. Clinical Immunology and Immunopathology 34 (4), 304-15 PubMed.
Other sources of information
- Felsberg P J (1992)Primary imunodeficiencies.In:Current Veterinary Therapy XI: Small Animal Practice.Eds: Kirk R W & Bonagura J D. Philadelphia: W B Saunders. pp 448-453.