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ISSN 1405-1699 (Print)
Órgano Oficial del Colegio Mexicano de Alergia, Asma e Inmunología Pediátrica y de la Asociación Latinoamericana de Pediatría

2018, Number 2

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Alerg Asma Inmunol Pediatr 2018; 27 (2)

Immunodeficiencies of the complement. Review of the literature Part II. Deficiencies of the alternate route, lectins, terminal pathway, control, regulation and complement receptors Diagnosis and treatment of complement immunodeficiencies

Fuentes PJM, Jiménez PEN, Espinosa PSE

Language: Spanish
References: 12
Page: 44-48
PDF size: 148.43 Kb.


ABSTRACT

The deficiency of lectin pathways is the most common complement deficiency. ACL deficiency, terminal, control, regulation, and complement receptors are less common, with the exception of hereditary angioedema. The deficiencies of the terminal route are related to severe infections by Neisseria. Laboratory studies include tests to assess the functional activity of the classical pathway (CH50), the alternate pathway (AH50), and the lectin pathway. The management depends on the specific component that is deficient. Replacement of the specific component is usually not possible, with the exception of C1 esterase inhibitor deficiency. Therefore the management of complement deficiencies is usually limited to supportive care and relates to the clinical manifestations that are secondary to the specific deficiency.


REFERENCES

  1. Sullivan KE, Stiehm ER. Stiehm’s immune deficiencies. Deficiencies of the Complement System. Chapter 38. Elsevier Inc. 2014; 731-756.

  2. Palarasah Y, Nielsen C, Sprogøe U, Christensen ML, Lillevang S, Madsen HO et al. Novel assays to assess the functional capacity of the classical, the alternative and the lectin pathways of the complement system. Clin Exp Immunol. 2011; 164 (3): 388-395.

  3. Bryan AR, Wu EY. Complement deficiencies in systemic lupus erythematosus. Curr Allergy Asthma Rep. 2014; 14 (7): 448.

  4. Al-Herz W, Bousfiha A, Casanova JL, Chatila T, Conley ME, Cunningham-Rundles C et al. Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency. Front Immunol. 2014; 5: 162.

  5. Botto M, Kirschfink M, Macor P, Pickering MC, Würzner R, Tedesco F. Complement in human diseases: lessons from complement deficiencies. Mol Immunol. 2009; 46 (14): 2774-2783.

  6. Available in: http://www.jmfworld.com

  7. Wen L, Atkinson JP, Giclas PC. Clinical and laboratory evaluation of complement deficiency. J Allergy Clin Immunol. 2004; 113: 585-593.

  8. Arkwright PD, Riley P, Hughes SM, Alachkar H, Wynn RF. Successful cure of C1q deficiency in human subjects treated with hematopoietic stem cell transplantation. J Allergy Clin Immunol. 2014; 133 (1): 265-267.

  9. Grumach AS, Kirschfink M. Are complement deficiencies really rare? Overview on prevalence, clinical importance and modern diagnostic approach. Mol Immunol. 2014; 61 (2): 110-117.

  10. Hudson-Peacock MJ, Joseph SA, Cox J, Munro CS, Simpson NB. Systemic lupus erythematosus complicating complement type 2deficiency: successful treatment with fresh frozen plasma. Br J Dermatol. 1997; 136 (3): 388-392.

  11. Sprong T, Roos D, Weemaes C, Neeleman C, Geesing CL, Mollnes TE et al. Deficient alternative complement pathway activation due to factor D deficiency by 2 novelmutations in the complement factor D gene in a family with meningococcal infections. Blood. 2006; 107 (12): 4865-4870.

  12. Biesma DH, Hannema AJ, van Velzen-Blad H, Mulder L, van Zwieten R, Kluijt I et al. A family with complement factor D deficiency. J Clin Invest. 2001; 108 (2): 233-240.




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