11:47,23,May,2010 | (1589/0/0) | Original

selective immune deficiency

Medical HEALTH "> Medical>> Pediatrics>> Summary of selective IgA deficiency and comment on recommended online download full collection in this article Zhou Yufeng Fang Fenghua Technology, Tongji Hospital, Tongji Medical College of Clinical Pediatric HIV Research Center, Wuhan 430030," Practical Training of Medicine, "33, 2005 No. 1 fast-food prizes Report Summary: by genetic factors or congenital hypoplasia of the immune system caused by immune dysfunction caused by the disease, called primary immune deficiency disease (primary immu- nodeficiency diseases, PID) or congenital immune deficiency (congenital immunodeficiency diseases), recurrent infections are the most significant features. PID is the most common selective IgA defects, the incidence rate of 1/223-1/3000; Number of pages: 4 pages :1-4 Keywords: immunoglobulin deficiency and immune dysfunction common congenital immune system with recurrent infections of primary immunodeficiency disease hypoplasia subject classification: R725.9 [medicine, HEALTH "Pediatrics> Pediatric within SCIENCES" children with systemic diseases] R554 [medicine, HEALTH "within SCIENCES" blood and lymphatic system diseases> bleeding disorders] Related articles: subject
selective immune deficiency

Children with selective immunoglobulin G subclass deficiency (alias: Children selective IgG subclass deficiency)
Children with selective immunoglobulin G subclass deficiency is what causes?
(A) etiology
According to their different IgG heavy chain can be divided into four sub-categories, namely IgG1, IgG2, IgG3 and IgG4. In which the highest proportion of IgG1, IgG2 is about a half of, IgG3 only the 1 / 10, IgG4 volume is minimal. IgG1, IgG3 is the T cell-dependent antibody, usually for protein antibodies. Non-T cell-dependent IgG2 antibodies, mainly for polysaccharide antigens. IgG4 is known closely related with type â…  allergy. IgG subclass imbalance or lack of, is known as IgG subclass deficiency (IgG subclass deficiency). Reduce the total serum IgG or normal, and one or more IgG subclasses lower than normal.
Selective IgG subclass deficiency is not very clear cause, which may be stable region IgG heavy chain (CH) gene disorders. There are two cases:
1. Immunoglobulin CH gene deletion or mutation of the corresponding IgG subclass deficiencies occur, and may be accompanied by IgA subclass deficiencies.
2.CH obstacles or rearrangement of recombinant heavy chain gene transcription and transcriptional regulatory exception that the majority of B cells and T cells to the regulation disorders. T cells produce interferon γ (IFN-γ) to reduce defects can be caused by IgG2, IL-4 to IgG1 and IgG4 to reduce the defects.
Simple nephrotic syndrome in children, nutrition disorders, long-term survival after bone marrow transplantation, acquired immunodeficiency syndrome (AIDS), some autoimmune
diseases (such as SLE), refractory epilepsy and allergies (hives, eczema , asthma), etc. may be associated with secondary IgG subclass deficiency.
(B) of the pathogenesis
Pathogenesis of this disease is complex, involving diverse abnormalities, in addition to hereditary, the B cells themselves may have dysfunction, T cell dysfunction, including reducing the number of CD4 cells, proliferation activity of the lower and the lower part of the lymphatic and other factors. Now that the IgG subclass deficiency was due to the long arm of chromosome 14, IgG heavy chain constant region gene partial deletion, mutation and subclasses of transition adjustment mechanism abnormality. Recent studies have confirmed, IgG and its sub-class transformation mechanism is still with the IL-4 and CD40 cells and other factors. CD40 cells in the IgG subclass play an important role in the final stages of its loss of function can also lead to some of the IgG subclass deficiency. In addition, when the helper T cells Th1/Th2 function imbalance between the number of IgG subclasses can also lead to defects.
When the IgG2 defect, high incidence of pneumonia, streptococcus and Haemophilus influenzae type b and other infections caused by bacteria; when the IgG1, IgG3 class antibodies against the protein defects, high incidence of viral infections and bacterial infections have toxoid.
Children with selective immunoglobulin G subclass deficiency and how to diagnose what performance?
Selective IgG subclass deficiency is the most common childhood disease of immune deficiency, clinical manifestations of disease can not also be manifested as recurrent respiratory tract infections in children with recurrent respiratory tract infection in the incidence rate of 20% to 35% . Including the upper respiratory tract infection, sinusitis, otitis media, rhinitis, bronchitis, bronchiectasis, and pneumonia. Some patients showed recurrent purulent meningitis, skin infections and diarrhea. In general, the infection is not serious, it will not endanger life.
IgG subclass deficiencies in the performance of the most common respiratory tract infection, the diagnostic criteria for recurrent respiratory tract infections in Table 1. Although IgG subclass deficiencies of individual clinical manifestations vary widely, but many researchers have observed that some of the characteristics of the performance.
1.IgG1 IgG1 defective defects associated with other IgG subclasses majority of defects, and often have low serum IgG levels. IgG1 defect cases. There may be some of the common variant immunodeficiency syndrome (CVID), particularly associated with other Ig classes defective. Often susceptible of purulent infection of the history of life, may develop persistent, progressive increase of pulmonary infection, diphtheria, tetanus toxin antibody response often flawed.
2.IgG2 defects often associated with defective IgG2 IgG4, IgA defects associated with, but also one exists. Children usually presents with recurrent respiratory tract infections. Defects and IgG2 antibody response to polysaccharide antigen is closely related defects, for diphtheria, tetanus toxin antibody response to antigen often normal. Interestingly, in a group of children with similar clinical manifestations, some patients have normal levels of IgG subclasses, but then an abnormally low response to polysaccharide antigens, that the normal content of IgG subclasses, but there is dysfunction. This suggests that low IgG2 level may be a sign of the existence of these children are often responses to some antigen defects, not defects in response to other antigens. IgG2 defect has also been reported for normal antibody response to polysaccharide antigens, this is the IgG1, IgG3 compensatory increased due to play a role in compensation. IgG2 subclass deficiencies in addition to clinical manifestations of patients with upper respiratory tract infection, but also may show the grounds of Neisseria meningococcal meningitis caused by repeated or recurrent pneumococcal infections. Most patients with selective IgG2 deficiencies have normal serum immunoglobulin levels.
3.IgG3 defective antibody against protein antigens, including anti-viral protein antibodies are mainly IgG1 and IgG3 subclasses. IgG3 antibodies may be the most effective medium and virus antibodies. IgG3 deficiencies primarily result in chronic lung disease, recurrent infection. A study in Sweden to collect 6580 cases (1864 cases of children, 4716 cases of adults) in serum of patients with recurrent infections, 313 patients presented with low serum IgG levels. The 313 patients, 186 cases for individual IgG3 defect, defects in 113 cases of IgG1 with IgG3, IgG3 with IgG2 deficiencies in 14 cases, 11 patients with IgG3 IgG4 deficiencies.
4.IgG4 defects IgG4 defect diagnosis is difficult to determine, because most infants and children, the serum IgG4 measured using standard methods can not only use more sensitive techniques (such as radioimmunoassay, enzyme-linked immunosorbent assay ELISA) studies to unequivocally IgG4 deficiencies identified patients (IgG4 concentrations <0.05? g / ml). These patients showed severe recurrent respiratory tract infections, including bronchiectasis. A case of selective IgG4 deficiencies of the patients had recurrent chalazion, since the development of lung infection. Most patients have normal or IgG4 deficiencies elevated total IgG and IgG subclasses, IgA, IgM and IgE concentrations. A small group of patients with IgG2 and IgG4 deficiencies associated with IgA defect. However, IgG4 deficiencies significance is unclear, because a substantial proportion of normal children, IgG4 concentration is low. Therefore, it is difficult to make a single IgG4 subclass defect.
Selective IgG subclass deficiency diagnosis is difficult, because the recurrent respiratory tract infections are nonspecific performance. Serum IgG, IgA, IgM often normal, may increase or decrease, therefore, determination of serum IgG subclass and antigen-specific antibody, the disease diagnosis is particularly important. Now that the selective IgG subclass deficiency diagnostic criteria:
1.IgG serum IgG subclass deficiencies subclasses measured values below the normal average of 1.96 children the same age or 2 standard deviations below.
2.IgG subgroups normal serum IgG subclass low measured values between normal children age 1 to the mean between the standard deviation of 1.96.
3. Antigen-specific IgG subclass antibody defects.
However, IgG4 in normal infants and children is very low, its level may be less than 1? G / ml. It is difficult to determine the baby IgG4 defects.
Children with selective immunoglobulin G subclass deficiency should do what check?
Selective IgG subclass deficiency laboratory tests, including serum IgG, IgA, IgM as a screening test depends mainly on the level of serum IgG subclasses; conditions should determine antigen-specific IgG subclass antibodies, including anti-polysaccharide measured antigen: such as resistance to Bacillus influenzae type b capsular polysaccharide, anti-Streptococcus capsular polysaccharide, anti-23-valent pneumococcal polysaccharide antigen, anti-meningococcal polysaccharide antigens specific IgG subclass antibodies; measured against protein antigen: such as anti-diphtheria toxin, tetanus toxoid, pertussis bacterial protein, measles virus and other viral coat protein antigen-specific IgG subclass antibodies. IgG subclass deficiency on the main issue is difficult to establish sensitive, specific and reliable method. Because the stable region in the IgG subclass exists a high degree of homology is difficult to produce in animals produce specific antiserum and monoclonal antibodies resolution; addition, the need for the international standard method, and the establishment of the normal range in all laboratories, this should include all age groups, each sex and the Gm types. Children's Hospital of Chongqing Medical University, Laboratory of Immunology, the WHO67/97 standard serum and monoclonal antibody ELISA, the serum IgG subclasses normal children (Table 2).
IgG subclass deficiencies may be normal when the serum IgG, lower or higher, but serum IgG subclass or antigen-specific IgG subclass antibody defects. IgG2 subclass deficiencies in 60% of children in the existence of anti-pneumococcal polysaccharide (PnPs) the lack of specific antibodies, 73% had anti-meningococcal polysaccharide (MPs)-specific antibody deficiency; IgG1 subclass deficiencies in children with 24% 29% of the existing anti-diphtheria vaccine (DTP), anti-measles vaccine (MV) specific antibody deficiency.
Conventional chest X-ray done, B-ultrasound, combined with clinical need to select other additional tests.
Children with selective immunoglobulin G subclass deficiency and the diseases easily confused?
Defect often shows the total IgG1 IgG also reduced, should pay attention to ordinary differential variant immunodeficiency syndrome, which often exist IgA deficiency. Ataxia telangiectasia often a lack of IgG2 and IgG4; severe combined immunodeficiency disease after bone marrow transplantation, there may be a temporary type of immunoglobulin or sub-class of the same kind of imbalance.
Children with selective immunoglobulin G subclass deficiency can be complicated by the diseases?
Recurrent respiratory tract infection can to bronchiectasis; repeated suppurative sequelae of meningitis may be to the nervous system; repeated diarrhea can to nutritional diseases.
Children with selective immunoglobulin G subclass deficiency should be how to prevent?
1. Maternal care are known to the occurrence of some immune deficiency and poor embryonic development are closely related. If the pregnant woman by irradiation, to accept the treatment of certain chemical substances or the occurrence of viral infection (especially rubella virus infection), etc., can damage the fetus's immune system, especially in early pregnancy, can include multiple systems including the immune system involvement. Therefore, strengthening the maternal health care, especially in early pregnancy is very important. Pregnant women should avoid receiving radiation, caution some chemical drugs, injected rubella vaccine, as far as possible to prevent infection. Also to make women better nutrition, timely treatment of some chronic diseases.
2. Genetic counseling and family surveys can not determine the genetic disease, although most of the way, but the way in determining the genetic disease genetic counseling is valuable. If adults have a genetic immune deficiency syndrome will provide the development of their children risk; If a child with autosomal recessive or sex-linked immunodeficiency disease, we must tell the parents, their next child the possibility of sick children how much. The antibody or complement deficiencies in patients with immediate family members should check the level of antibody and complement to determine the prevalence of the family way. For some have been able to locate genetic diseases such as chronic granulomatous disease, the patient parents, siblings and their children, locate genetic testing should be done, if there are patients, the same shall be in his (her) family members to check , the children were born in the beginning should be careful to observe whether the disease.
3. Prenatal diagnosis of certain immune deficiency diseases can be carried out prenatal diagnosis, such as amniotic fluid cells cultured examination can diagnose enzyme adenosine deaminase deficiency, nucleoside phosphorylase deficiency and certain combined immunodeficiency disease; fetal Immunological detection of blood cells to diagnose CGD, X linked agammaglobulinemia-free, severe combined immunodeficiency disease, which termination of pregnancy to prevent birth of children. Selective immunoglobulin G subclass deficiency is the most common childhood disease of the immune defect, early accurate diagnosis, early treatment and given specific genetic counseling (prenatal diagnosis or even intrauterine treatment) is very important.
Children with selective immunoglobulin G subclass deficiency should be treated?
(A) treatment
Repeated injection of polysaccharide and protein vaccines (such as Haemophilus influenzae type b capsular polysaccharide meningococcal outer membrane protein complex vaccine) may improve the body's antibody response. The application of IVIG should be limited to serious clinical symptoms and no response to antibiotic treatment of sick children, the recommended dose per month 200 ~ 400mg/kg. Defect of IgA anti-IgA patients should carefully monitor the production of antibodies. Immunostimulants, such as astragalus, Klebsiella glycoprotein extract (Biostim), transfer factor, recombinant cytokines, IgG subclasses for the effect to be further confirmed. Infection should be given appropriate anti-infectives. Secondary IgG subclass deficiencies, shall be treated for primary disease due to such person by the correction of nutritional disorders, you can return to normal.
(B) prognosis
Selective IgG subclass deficiency in the good prognosis, most patients with age, serum IgG subclass levels increased gradually, reducing the chances of infection to the serum IgG subclass concentration in adolescence return to normal. Part of the sick child persistent IgG subclasses, the defect type can be changed, no obvious regularity. Received the combination of polysaccharide and protein vaccines, after repeated vaccination, most patients can promote the production of corresponding antibodies, but also reduce the chance of infection. Combined with other primary immunodeficiency patients, must be long-term replacement therapy with IVIG.
Children with selective immunoglobulin G subclass deficiency worry 4 (content expertise) to edit entries summary
There is no summary of the contents of the directory welcome to add the edit summary - [hide] 1 Overview 2 Diagnostic 4 3 principle of selection of drug-related 5 Note 6 Note that point 7 of side effects and treatment recommendations that Editing this paragraph 8 | Back to the top of the overview of children with selective IgA G subclass deficiency is an X-linked primary immunodeficiency disease, the immune deficiency, eczema and thrombocytopenia typical triad of clinical manifestations, atypical cases may be mainly as thrombocytopenia, and no obvious immune defects performance, this time to be with idiopathic thrombocytopenic purpura identification. Often more prominent blood system performance, can occur after birth, bleeding tendency, including purpura, melena, hematuria, decreased platelets, platelet volume decreases.
Edit this section | Back to the top of the reduction in the level diagnosis of serum IgM, IgG levels decreased slightly or normal, IgA and IgE can be elevated significantly increased the number of peripheral blood B cells, and T cells was significantly reduced. The criteria for the disease: male infant with recurrent infections, eczema, thrombocytopenia, hemorrhagic rash, accompanied by increased serum IgA and IgE, IgM decreased, the lack of the same type of hemagglutinin, the protein polysaccharide antibody response decreased. Platelet volume decreased particularly helpful in the diagnosis of children with WAS.
Treatment: the main treatment for this disease, including stem cell transplantation in reconstruction of immune function, bleeding could be considered a bigot splenectomy. Drug treatment for the prevention and treatment of infection is also very important.
Edit this section | Back to the top of the related drug intravenous human immune globulin (Intravenous immunoglobulin, IVIG, intravenous immunoglobulin, intravenous gamma globulin human blood)
Globulin purity above 98%, mainly composed of IgG monomer, while IgA trace elements. For the treatment of XLA, common variant immunodeficiency disease, immunoglobulin G subclass deficiency disease, a variety of secondary immunoglobulin deficiency and certain autoimmune autoimmune diseases.
Edit this section | Back to the top of the selection principle 1, a typical WAS patient after diagnosis is required to start using antibiotics to prevent Pneumocystis carinii infection.
2, infection or serious infection frequent use IVIG, to maintain normal levels of serum IgG.
3, current infection should be chosen when there is sensitive to antibiotic treatment, so as to clear the infection.
Edit this section | Back to the top of the Notes Agent or freeze-dried powder: 2.5g or 5g / bottle.
Usage: Application of freeze-dried powder dissolved in water to the required injection volume (loading capacity of 2.5g / bottle are dissolved to 50ml, installed capacity of 5g / bottles were dissolved to 100ml). Usually 3-4 weeks infusion once per kilogram of body weight 400-600mg, dosage should be individualized to be able to control the infection, so that relief of symptoms in children, access to normal growth and development for the scale.
Edit this section | Back to the top of the side effects and pay attention to point 1, when individual patients in the infusion of a transient headache, palpitation, nausea and other reactions, infusion may be too fast or individual differences. The reactions were mild, and often occur 1 hour after the infusion. Therefore should be closely observed, if necessary, slow down or pause infusion infusion rate, which generally do not need to deal with their own relief.
2, the product intended for intravenous infusion, should be a separate infusion, infusion mixed with other drugs ban.
3, the product should be dissolved in colorless clear liquid, if any foreign body, floc and sediment should not be used. After the first infusion should be dissolved, shall not be graded or lost to others to use.
4, with severe acid-base metabolism disorders in children should be used with caution.
5, 10 ° C the following stored.
6, gamma globulin on human blood or other serious allergies allergies are prohibited, there is selective IgA deficiencies IgA antibodies were not used.
Usage: Generally for the first time use per kilogram of 400mg, after the serum IgG levels according to clinical improvement and adjust the dose.
Edit this section | Back to top recommendation 1 of bleeding is critical, long-term monitoring of blood platelet levels and the timely use of blood transfusion in children can save lives during a given period.
2, severe, recurrent bleeding can be used for splenectomy.
3, with the typical triad show poor prognosis patients, the need stem cell transplantation to restore immune function. Immune to normal siblings matching the best donor.
Edit this section | Back to the top of the treatment that should pay attention to symptomatic treatment. Severe bleeding to lose fresh platelets, but be careful, because
there are a growing trend of autoantibodies. Although some of the line of sick children after splenectomy, platelet increase, reduce bleeding, but increased the risk of severe infection, often so sick child died of sepsis, it should take a cautious approach. Systemic glucocorticoid treatment fails, and increase the already immunocompromised, can local treatment of chronic eczema. The time of infection to the effective antibiotics. But not intramuscular injection of gamma globulin to prevent local hemorrhage. Regularly enter the plasma on infection control is effective. Many reports successful application of transfer factor. Thymosin and the application of thymus transplantation is still experimental. Successful bone-marrow transplant normal immune function can correct the thrombocytopenia. Edit entry Open Category:
Disease, deficiency disease term evaluation: Evaluation of the authority to participate in a total of 0 0% 0% rich professional good 0% 0% 0% I have to evaluate the poor:
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