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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

2016, Number 3

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Alerg Asma Inmunol Pediatr 2016; 25 (3)

Immunodeficiencies of the complement. Literature review Part I. Generalities and deficiencies of the classical pathway

Fuentes PJM, Jiménez PEN, Espinosa PSE

Language: Spanish
References: 22
Page: 84-88
PDF size: 221.06 Kb.


ABSTRACT

Complement immunodeficiencies are defined as inherited immune system disorders that lead to the absence of the level or function of the protein. We can find deficiencies in complement activation pathways: classical alternative, lectins, or regulatory proteins. Deficiencies of the classical pathway are: C1q deficiency, C1r/s, C4, C2 and C3, which are associated with lupus erythematosus and infections by pyogenic microorganisms. Treatment are measures are support and is reported a case of a Pakistani teenager who at the age of 16 years hematopoietic cell transplantation related live donor (brother) was performed with good results. In this review we demonstrations by inherited disorders of the complement system classical pathway, leading to the total absence of level or function of proteins.


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