EC Paediatrics

Recurrent moderate or major bacterial infections, autoimmune diseases, or episodes of angioedema-a painless but frequently spectacular swelling beneath the skin or in the bowels that can be excruciatingly painful-are clinical indicators of potential complement deficits. The clinical issues that patients with complement deficits face are dependent on how the particular complement protein functions normally. The study was conducted to determine the complement C3 deficiency among children suffering from recurrent infections. The study includes both sexes: 49 (54%) male and 41 (46%) female. The level of complement C3 deficiency in the age group (> 2.5, 2.6 - 5, 5.1 - 7.5, 7.6 - 10) years showed 17%, 17%, 42%, and 25%, respectively. The study found that both deficient and low levels were (7%), the normal range was (36%), the high level was (37%), and the very high level was (14%). The relationship between the age group and the level of complement C3 was insignificant. According to residents, 67% of Khartoum North yields the highest rate of complement C3 deficiency than 33% of Khartoum. The study concluded that there is a relationship between complement C3 deficiency and recurrent infection, and early diagnosis is important to decrease the risk of acquiring severe infections during childhood.

 Keywords: Complement Protein C3; Children; Recurrent Infection

1. Ricklin D and Lambris JD. “Preformed mediators of defense-Gatekeepers enter the spotlight”. Immunological Reviews 1 (2016): 5-8.
2. de Bruijn MH and Fey GH. “Human complement component C3: cDNA coding sequence and derived primary structure”. Proceedings of the National Academy of Sciences of the United States of America 3 (1985): 708-712.
3. Advanced search results for UniProt accession P01024. Protein Data Bank in Europe (PDBe). European Bioinformatics Institute (2010).
4. Janssen BJ., et al. “Structures of complement component C3 provide insights into the function and evolution of immunity”. Nature7058 (2005): 505-511.
5. Janssen BJ., et al. “Structure of C3b reveals conformational changes that underlie complement activity”. Nature 7116 (2006): 213-216.
6. Wiesmann C., et al. “Structure of C3b in complex with CRIg gives insights into regulation of complement activation”. Nature7116 (2006): 217-220.
7. Fredslund F., et al. “The structure of bovine complement component 3 reveals the basis for the thioester function”. Journal of Molecular Biology1 (2006): 115-127.
8. Pasch MC., et al. “Synthesis of complement components C3 and factor B in human keratinocytes is differentially regulated by cytokines”. Journal of Investigative Dermatology1 (2000): 78-82.
9. Soames CJ and Sim RB. “Interactions between human complement components factor H, factor I, and C3b”. Biochemical Journal2 (1997): 553-561.
10. Jokiranta TS., et al. “Complement C3b interactions studied with surface plasmon resonance technique”. International Immunopharmacology3 (2001): 495-506.
11. Abul K Abbas., et al. Cellular and Molecular Immunology.
12. Sahu A and Lambris JD. “Structure and biology of complement protein C3, a connecting link between innate and acquired immunity”. Immunological Reviews1 (2001): 35-48.
13. Lachmann PE. “Genetics of the complement system”. Journal of Medical Genetics4 (1975): 372-377.
14. Matsuyama W., et al. “Molecular analysis of hereditary deficiency of the third component of complement (C3) in two sisters”. Internal Medicine 12 (2001): 1254-1258.
15. Walport MJ. “Complement-part I”. New England Journal of Medicine 344 (2001): 1058-1066.
16. Wolach B., et al. “The development of the complement system after 28 weeks' gestation”. Acta Paediatrica5 (1997): 523-527.
17. Janzi M., et al. “Screening for C3 deficiency in newborns using microarrays”. PLoS One4 (2009): e5321.
18. Murphy Ea and Chase Ga. “Principles of genetic counseling”. Chicago, Yearbook Medical Publishers (1975): 70-74.
19. S Reis E., et al. “Clinical aspects and molecular basis of primary deficiencies of complement component C3 and its regulatory proteins factor I and factor H”. Scandinavian Journal of Immunology3 (2006): 155-168.