Cases of complement deficiency have helped defined the role of complement in host defense. (medscape.com)
A North African study of molecular basis of complement factor I deficiency in atypical hemolytic and uremic syndrome patients suggested that the Ile357Met mutation may be a founding effect. (medscape.com)
Patients with a known complement deficiency should be screened for glomerular or immune complex disease. (medscape.com)
Administration of the multivalent meningococcal vaccine is recommended in patients with known complement deficiency, especially those patients deficient in the MAC proteins. (medscape.com)
Deficiency of a MAC component (C5, C6, C7, C8) or of properdin increases the risk for recurrent infections caused by Neisseria organisms. (medscape.com)
Patients with an identified complement deficiency should be counseled regarding possible complications and risks associated with this deficiency. (medscape.com)
Skattum L, van Deuren M, van der Poll T, Truedsson L. Complement deficiency states and associated infections. (medscape.com)
Arnold DF, Roberts AG, Thomas A, Ferry B, Morgan BP, Chapel H. A novel mutation in a patient with a deficiency of the eighth component of complement associated with recurrent meningococcal meningitis. (medscape.com)
C5 Complement Deficiency in a Saudi Family, Molecular Characterization of Mutation and Literature Review. (medscape.com)
Defects in the alpha chain of C8 can result in complement C8 deficiency type I. Furthermore, C8 deficiencies can cause recurring bacterial infections, in particular from Neisseria meningitides. (antibodypedia.com)
Deficiency of the eighth component of complement associated with recurrent meningococcal meningitis--case report and literature review. (antibodypedia.com)
Patients with C9 deficiency suffer from recurrent bacterial infections. (umassmed.edu)
The dysfunction may be due to problems in antibody production, impaired cell-mediated immunity, a combined type of antibody/cellular deficiency, impaired phagocytosis, or complement deficiency. (ssa.gov)
3. Immune deficiency disorders, excluding HIV infection (14.00E) . Immune deficiency disorders are characterized by recurrent or unusual infections that respond poorly to treatment, and are often associated with complications affecting other parts of the body. (ssa.gov)
Deficiencies14
Deficiencies in the complement cascade can lead to overwhelming infection and sepsis. (medscape.com)
Complement deficiencies are said to comprise between 1 and 10% of all primary immunodeficiencies. (medscape.com)
[ 4 ] A registry of complement deficiencies has been established as a means to promote joint projects on treatment and prevention of diseases associated with defective complement function. (medscape.com)
This article outlines some of the disease states associated with complement deficiencies and their clinical implications. (medscape.com)
Complement pathways and deficiencies. (medscape.com)
Deficiencies in complement predispose patients to infection via 2 mechanisms: (1) ineffective opsonization and (2) defects in lytic activity (defects in MAC). (medscape.com)
Specific complement deficiencies are also associated with an increased risk of developing autoimmune disease, such as SLE. (medscape.com)
Complement deficiencies are the result of a lack of any of these proteins. (wikipedia.org)
Inpatient treatment is not necessarily needed to screen for complement deficiencies if the patient is asymptomatic. (medscape.com)
In general, the prognosis for patients with C3 deficiencies is poorer than that of individuals with other complement deficiencies. (medscape.com)
Family members should be screened for complement deficiencies and counseled regarding possible risks. (medscape.com)
Grumach AS, Kirschfink M. Are complement deficiencies really rare? (medscape.com)
Complement deficiencies in systemic lupus erythematosus. (medscape.com)
Complement genetics, deficiencies, and disease associations. (medscape.com)
The complement system consists of more than 35 soluble and cell-bound proteins, 12 of which are directly involved in the complement pathways. (dadamo.com)
Genes that encode the proteins of complement components or their isotypes are distributed throughout different chromosomes, with 19 genes comprising 3 significant complement gene clusters in the human genome. (medscape.com)
The important components of this system are various cell membrane-associated proteins such as complement receptor 1 (CR1), complement receptor 2 (CR2), and decay accelerating factor (DAF). (medscape.com)
Infections1
Immune system disorders may result in recurrent and unusual infections, or inflammation and dysfunction of the body's own tissues. (ssa.gov)
Pathways1
The complement cascade consists of 3 separate pathways that converge in a final common pathway. (medscape.com)
Pathogens2
The complement system helps clear pathogens from an organism. (dadamo.com)
Although the complement system is part of the body's innate, relatively nonspecific defense against pathogens, its role is hardly primitive or easily understood. (medscape.com)
Inflammation2
The complement system is a potent mechanism for initiating and amplifying inflammation. (dadamo.com)
Activation of the complement cascade, with the formation of the effector MAC unit, results in cytotoxic and cytolytic reactions. (dadamo.com)
The main functions of the complement cascade and its role in the acute inflammatory reaction are summarized in Table 1. (dadamo.com)
New studies point to the complex interplay between the complement cascade and adaptive immune response, and complement is also being studied in association with ischemic injury as a target of therapy. (medscape.com)
Innate2
The actions of the complement system affect both innate immunity and acquired immunity. (dadamo.com)
The complement system is part of the innate immune system. (medscape.com)
Antibody2
Lectins activate the lectin pathway in a manner similar to the antibody interaction with complement in the classical pathway. (medscape.com)
This antibody recognizes complement component 8 (C8), a 151 kDa member of the complement C6/C7/C8/C9 family, present in blood serum. (antibodypedia.com)
Clinical1
Some new clinical entities are linked with partial complement defects. (medscape.com)
Episodes1
Patients may have severe, recurrent episodes of pyogenic infection beginning when as young as a few months. (medscape.com)
Pyogenic1
The complement system plays an important part in defense against pyogenic organisms. (medscape.com)
Functional2
From the functional standpoint, complement receptors can be divided into two types: the adherent type and the other receptors. (dadamo.com)
Functional complement C1q abnormality leads to impaired immune complexes and apoptotic cell clearance. (medscape.com)
Immune complexes1
CR1 on erythrocytes may bind circulating immune complexes (that had activated complement) and transport them to the liver where the immune complexes are partially degraded and thus become more soluble. (dadamo.com)
Result1
The result of cytotoxic complement reaction may be beneficial for the body (elimination of the infectious agent or damaged cells) or harmful (damage to autologous normal cells by immunopathological reactions). (dadamo.com)
Infection1
In addition to playing an important role in host defense against infection, the complement system is a mediator in both the pathogenesis and prevention of immune complex diseases, such as systemic lupus erythematosus (SLE). (medscape.com)
Component1
Leiner's disease associated with diminished third component of complement. (medscape.com)
The complement system influence the activity of numerous cells, tissues and physiological mechanism of the body. (dadamo.com)
Anaphylatoxins are proteolytic products of the serine proteases of the complement system: C3a, C4a and C5a. (dadamo.com)
These findings underscore the duality of the complement system. (medscape.com)
Knowledge about the complement system is expanding. (medscape.com)
An intricate system regulates complement activity. (medscape.com)
Specific1
Different fragments, released from individual components during complement activation, operate by a non-cytolytic mechanism through specific receptors present on various cell types. (dadamo.com)
Means1
10. Recurrent means that a condition that previously responded adequately to an appropriate course of treatment returns after a period of remission or regression. (ssa.gov)
Components2
These effects may involve either the whole complement, or only individual components or fragments. (dadamo.com)
This is mediated through fragments of complement components. (dadamo.com)
Cells1
With the evidence that DTN cells receive GABAergic recurrent inhibition, we tested whether the inhibitory effect of CPS was caused by enhanced GABAergic transmission. (epiphanyasd.com)
Website1
This graph shows the total number of publications written about "Complement C9" by people in this website by year, and whether "Complement C9" was a major or minor topic of these publications. (umassmed.edu)