Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy

Litzman, Jiří; Freiberger, Tomáš; Bartoňková, Dana; Vlková, Marcela; Thon, Vojtěch; Lokaj, J

doi:10.1046/j.1440-1754.2003.00128.x

Cited by 11 publications

(8 citation statements)

References 21 publications

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“…11e15, 24,25 In the study by Lorraine et al, 11 only one patient had low complement levels, which was secondary to a mild disseminated intravascular coagulation. They believed that complement levels may increase or decrease in meningitis depending on the phase of disease and the balance between complement production and consumption.…”

Section: Discussionmentioning

confidence: 97%

Lymphocyte subsets, immunoglobulin levels, complement activity CH50, and phagocytic peroxide production in 19 Iranian patients with first episode of bacterial meningitis

Ahmadinejad¹,

Bagherian²,

Atarord³

et al. 2011

Journal of Microbiology, Immunology and Infection

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Section: Discussionmentioning

confidence: 97%

Lymphocyte subsets, immunoglobulin levels, complement activity CH50, and phagocytic peroxide production in 19 Iranian patients with first episode of bacterial meningitis

Ahmadinejad¹,

Bagherian²,

Atarord³

et al. 2011

Journal of Microbiology, Immunology and Infection

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“…Of the 13 homozygous C2-deficient individuals with infections, 85% had IgG4 deficiency, compared with 64% of 25 individuals without infections. In another report, the clinical courses of three C2-deficient patients (two were siblings) were recorded (256). All three children suffered infectious complications early in life.…”

Section: Inherited Deficiencies Of Complement Activationmentioning

confidence: 99%

Infections of People with Complement Deficiencies and Patients Who Have Undergone Splenectomy

2010

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SUMMARY The complement system comprises several fluid-phase and membrane-associated proteins. Under physiological conditions, activation of the fluid-phase components of complement is maintained under tight control and complement activation occurs primarily on surfaces recognized as “nonself” in an attempt to minimize damage to bystander host cells. Membrane complement components act to limit complement activation on host cells or to facilitate uptake of antigens or microbes “tagged” with complement fragments. While this review focuses on the role of complement in infectious diseases, work over the past couple of decades has defined several important functions of complement distinct from that of combating infections. Activation of complement in the fluid phase can occur through the classical, lectin, or alternative pathway. Deficiencies of components of the classical pathway lead to the development of autoimmune disorders and predispose individuals to recurrent respiratory infections and infections caused by encapsulated organisms, including Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae. While no individual with complete mannan-binding lectin (MBL) deficiency has been identified, low MBL levels have been linked to predisposition to, or severity of, several diseases. It appears that MBL may play an important role in children, who have a relatively immature adaptive immune response. C3 is the point at which all complement pathways converge, and complete deficiency of C3 invariably leads to severe infections, including those caused by meningococci and pneumococci. Deficiencies of the alternative and terminal complement pathways result in an almost exclusive predisposition to invasive meningococcal disease. The spleen plays an important role in antigen processing and the production of antibodies. Splenic macrophages are critical in clearing opsonized encapsulated bacteria (such as pneumococci, meningococci, and Escherichia coli) and intraerythrocytic parasites such as those causing malaria and babesiosis, which explains the fulminant nature of these infections in persons with anatomic or functional asplenia. Paramount to the management of patients with complement deficiencies and asplenia is educating patients about their predisposition to infection and the importance of preventive immunizations and seeking prompt medical attention.

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“…Immunoglobulin deficiencies are more associated to ACNS infections than primary complement deficiencies [ 19 – 21 ]. Usually, a combined IgG and IgA deficiency or IgG deficiency and neutrophil chemotaxis abnormality have been documented [ 16 ] In our study, all patients had normal IgA values while IgM and IgG levels were lower than the age-related standard.…”

Section: Discussionmentioning

confidence: 99%

Follow-up evaluation of the immunological status of children admitted for acute cerebral nervous system infections: a retrospective study

et al. 2021

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Background Acute Cerebral Nervous System Infections (ACNS) may cause death or severe complications even to promptly treated children. The role of the immune system in influencing the course and the outcome of meningitis has been studied but it is not yet completely understood. The aim of the research is to ascertain whether children who experienced ACNS infection had a normal immune system. Methods Patients under 18 years of age admitted at Bambino Gesù Children from January 2006 till June 2016 for meningitis were asked to participate to the follow-up study. The immune status was evaluated both clinically and by laboratory investigations. Results Most of the patients over 3 years at follow up had at least one immunological alteration at follow-up evaluation (74%). Considering ACNS infection etiology, certain pathogens were almost exclusive of patients affected by some immunological alteration, regardless of their age. Discussion Our preliminary results indicate that sub-clinical immunological defects may be associated to ACNS pediatric infections. Moreover, to the best of our knowledges, this is the first study correlating pathogens to immune evaluation in ACNS infections. It is, however, important to underline the high frequency of persistent immunological alterations in the analyzed patients. Further studies are needed to confirm our conclusions. Conclusions We recommend an immunological assessment at follow up evaluation in children who experienced an ACNS infection.

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Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy

Abstract: Our observations point to an early manifestation of C2 deficiency within the first year of life, with meningitis as the most severe complication. The severity of immunodeficiency may be influenced by concomitant deficiencies of immunoglobulin isotypes.

Cited by 11 publications

References 21 publications

Lymphocyte subsets, immunoglobulin levels, complement activity CH50, and phagocytic peroxide production in 19 Iranian patients with first episode of bacterial meningitis

Lymphocyte subsets, immunoglobulin levels, complement activity CH50, and phagocytic peroxide production in 19 Iranian patients with first episode of bacterial meningitis

Infections of People with Complement Deficiencies and Patients Who Have Undergone Splenectomy

Follow-up evaluation of the immunological status of children admitted for acute cerebral nervous system infections: a retrospective study

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