The cause is not very clear, with a variety of factors. Most scholars believe that this disease is to contain IgA circulating immune complexes deposited in the kidney and disease. Complex antigen may be infected with respiratory or gastrointestinal mucosa viruses, bacteria, or food ingredients.
(B) in the pathogenesis
. Pathogenesis of renal tissue of IgA, C3, and (or) IgA, IgG deposition, IgA nephropathy is an immune complex glomerulonephritis, the incidence of IgA immune disorders are closely related, the present study has depth to IgA molecular structure level.
(1) the structure and characteristics of immunoglobulin A: IgA is an important immune globulin, accounting for 15.2% of the total serum immunoglobulin, 80% of serum IgA in the form of the monomer four chain monomer The inter-connected by disulfide bonds and the stability of the J chain. By α heavy chain different antigenic IgA is divided into two serotypes, i.e., IgA1 and IgA2.
IgA1 is the main subtype in the serum, accounting for 80% ~ 90%, IgA2, only 10% to 20%. IgA1 hinge region is 1 times longer than IgA2 IgA2 can be divided into the the IgA2m (1) and the IgA2m (2), although the concentration in serum IgA2 only IgA1 1/4, but the secretion of fluid equal to the 1gA2 concentration IgA1. Structure IgA2m (1) alpha chain and light chain disulfide bonds, connected to rely on non-covalent bonds, but between the light chain and α chain disulfide bonds connected.
Another form of IgA called secretory IgA (SIgA) exist in the outer secretions, such as saliva, tears, intestinal secretions and colostrum. Secretory IgA and serotype different, it is a dimeric molecule with a J chain, and the other outer secretory component (SC), composition (IgA) 2-J-SC complexes. Serotype is (IgA) 2-J composition.
J chain consists of 137 amino acids, molecular weight of 1500, is an acidic glycoprotein containing 8 cysteine residues, six with intra-chain disulfide bond formation, and the two with the connection of the α chain. 18 additional amino acid residues of the C-terminus of the known α chain, J chain is connected through the first two cysteine residues with an α-chain with the C-terminus of the α chain. Both of which are produced by plasma cells, and secretion would be connected together.
SC is synthesized by the epithelial cells in the mucosal tissue or endocrine body, connected through one of the two monomer IgA disulfide colleagues SIgA, SC is from 549 to 558 amino acids of the polypeptide chain, a molecular weight of about 7 Wan, glycosyl content of up to 20%. 5 homologous region in its polypeptide chain, each of the homologous region from the 104,114 amino acids, these homologous regions in the three-dimensional structure similar to and Ig. It has been known that is connected to the α-chain in the Fc region, but the precise positioning is not yet clear. The SIgA configuration may add up to the Y-shaped arrangement: ① a heap; ② The end of the end of the arrangement, the two IgA Fc α connected double Y-shaped structure.
Local tissue plasmacytomas (IgA) 2-J through the: ① with epithelial cell surface of the substrate side of the SC combination formed IgA-J-SC, and transferred to the top surface of a vesicle and secreted; ② (IgA) 2-J lymphatic vessels into the blood circulation, SC combined with the surface of the liver cells and clear, then turn to enter the biliary vesicle mechanism of liver cells, and eventually into the intestines.
The serum IgA terminal connected to each other may be formed to open at the end of polymer, and an obvious feature is a poly body size heterogeneity, the serum IgA 20% of polymer present in the form, and the sedimentation coefficient of 10s 13s, 15s ranging addition IgA Ease of a tendency to form a complex with other proteins, which are vulnerable due to the α-chain of the amino acid residues in the formation of intermolecular disulfide bonds. IgA molecular structure of these characteristics is important in IgA nephropathy.
(2) IgA deposition in the mesangial area: IgA nephropathy, IgA deposition and glomerular pathological changes parallel. Associated with mesangial proliferative mesangial IgA deposition, deposition on the capillary accompanied by changes of the vascular endothelial.
IgA deposition caused by pathological factors: ① antigen from the mucous membrane into the body and stimulate IgA immune system, the antigen component of a wide range of microorganisms, Food (ovalbumin, bovine serum albumin, casein), etc.. ② IgA immune response abnormalities lead to high molecular weight poly-IgA formation. (3) antigen-binding poly-IgA by electrostatic (λ chain), receptor (Fear) or connected to the fiber to protein binding and deposition in the kidneys, it has been found that the serum IgA-fibronectin complex is characterized IgA nephropathy. ④ Other IgA clearance mechanisms (such as liver) damage or saturation.
Existing studies show that the deposition in the glomeruli of the IgA IgA nephropathy is mainly poly λ-IgA1, the average IgA nephropathy patients, serum IgA1, poly-IgA, λ-IgA1 water visible increased. B cells in patients with reduced end link of galactose, β-1, 3 galactosyl transferase (β-1, 3GT) defects, resulting in O-glycosylation of IgA1 hinge region, this change may affect IgA1 and oligonucleotide on the sialic acid in the liver cell receptor (ASGPR) binding affect the clearance of IgA, but also increase the combined deposition and kidney tissue.
Harpel by in situ hybridization study found that of IgA nephropathy intestinal mucosa express an essential component of the synthesis of poly-IgA J chain mRNA levels decreased, while the bone marrow is increased. In addition tonsil PIgA1 also increased. The due production of tonsil PIgA is far lower than the mucosa and bone marrow, it is deposited in the kidney tissue PIgAl mainly derived from bone marrow rather than tonsils and mucosal.
(3) IgA nephropathy immune abnormalities: IgA nephropathy humoral and cellular immune extensive research shows that patients with IgA nephropathy immune abnormalities, including:
① autoantibodies: Fornesier, has been found in the serum of kidney patients the cytoplasmic macromolecules ingredients mesangial cells in the antibody. In addition to antibodies against the basement membrane Ⅰ, Ⅱ type III collagen fibers laminin, Gliadin ingredients. IgA anti-neutrophil cytoplasmic antibodies (IgA-ANCA) is also found in the blood of some patients. IgA nephropathy received renal allograft re-appear in the transplanted kidney the IgA nephropathy pathological changes by as much as 40% to 50%, these data are autoantibodies play an important role in the pathogenesis of IgA nephropathy.
② cellular immunity: studies have shown that the cellular immune function disorders are IgA nephropathy play an important role in the pathogenesis. IgA specific inhibition of the decline in the activity of T cells resulting in B lymphocytes increased synthesis of IgA. T helper cell (Th) of activity in IgA nephropathy increased activity of Th / Ts increased. IgA specific receptor of T cells referred to as a the Tα cell, Talpha cells increase the role of IgA production. It was found that IgA nephropathy, especially for patients with gross hematuria Tα increased significantly, Tα helper cells resulted in significantly increased IgA synthesis increased.
③ cell factor and inflammation of the media: many cytokines involved in the regulation of the immune system, including lymphokines White mediated hormone (interleukin, of IL), tumor necrosis factor, a polypeptide growth factor, these cytokines for the exercise of normal immune function from the important role in the abnormal circumstances will cause the offset of the cytokine network, thereby generating immune injury. Mesangial cell proliferation, cytokine and inflammatory mediators (complement components MAC, IL-1, MCP-1, reactive oxygen species, etc.) play an important role.
④ immune genetic: there are family members has suffering from IgA nephropathy reported, suggesting that genetic factors important role in IgA nephropathy. IgA nephropathy associated HLA antigen sites also reports vary, Europe and the United States Bw35, Japan and China DR4 common, also reported in northern China Han DRWl2 most common, in addition to B12, DR1 and IL-RN.2 bit gene, ACED / D genotype reported.
These sediments have diagnostic value. I, III, type IV collagen and laminin, fibronectin in IgA nephropathy glomerular capillary loop expression increased significantly, I type III collagen expression in mesangial area also increased significantly, the majority of patients with tubular basement IV also increased the expression of collagen type.
And accompanied by large lumpy electron dense deposits.
IgA nephropathy (IgA nephropathy IgAN) Also known as Berger disease glomerular mesangial IgA or IgA deposition with or not associated with other immunoglobulin deposition in the mesangial area of primary glomerular disease clinical manifestations: recurrent episodes of gross hematuria or microscopic hematuria may be associated with varying degrees of proteinuria in some patients severe hypertension or renal insufficiency. Classification of Diseases Primary IgA nephropathy: common disease caused by the kidney itself Secondary IgA nephropathy: caused by diseases other than kidney: of purpura nephritis HIV infection seronegative spondyloarthritis tumor leprosy liver disease familial IgA nephropathy  Causes The exact pathogenesis of IgA nephropathy is not yet entirely clear that a variety of factors and the incidence of views about the relatively consistent IgAN glomerular disease caused by the case of immune complexes Relationship with the immune system: IgA nephropathy in multimeric IgA (PIgA) show that in the glomerular deposition of IgA immune system leads to the deposition of the circulatory system of the PIgA molecules and mesangial area Relationship with the bone marrow: glomerular mesangial deposits of IgA and present in the blood of IgA similar hinge region glycosylation abnormalities suggest that patients with IgA nephropathy IgA deposition in glomerular mesangial area of bone marrow-derived IgA Relationship with cytokines: patients with IgA nephropathy mesangial area of IgA deposition caused mesangial cells secrete inflammatory cytokines   Pathophysiological The IgA nephropathy iconic pathological changes in IgA deposition in glomerular mesangial area in most patients with concomitant CIgG IgM deposition LEE classification: Level I: glomerular lesions: the vast majority of normal occasional mild mesangial widening (segments) with or without cell proliferation; the tubulointerstitial changed: normal Grade Ⅱ: glomerular lesions: glomerular focal mesangial proliferation and sclerosis (%) rare small crescent; tubulointerstitial change: Grade Ⅲ: glomerular lesions: diffuse mesangial proliferation and widened (occasionally focal stage) and occasionally small crescent balloon adhesions; the tubulointerstitial changes to: Board Zaojian interstitial edema occasionally the cell infiltration rare tubular atrophy Ⅳ: glomerular lesions: severe diffuse mesangial proliferation accompanied by hardening of some or all of glomerulosclerosis visible crescent (<%); tubulointerstitial change: tubular atrophy, interstitial infiltration occasionally interstitial foam cells Ⅴ grade: glomerular lesions: lesions similar Ⅳ level, but more serious visible crescent (%); tubulointerstitial change: similar Ⅳ grade but more serious
Children with nephrotic syndrome, hormone therapy need to be careful. Children with nephrotic syndrome primary to the more common lesions and increased permeability of the glomerular basement membrane. The clinical is often expressed as proteinuria, hypoalbuminemia, hyperlipidemia, high degree of edema three high and one low symptoms. The most important treatment to hormone therapy, prednisone, and other commonly used drugs.
People treat nephrotic syndrome, fully aware of the dangers of hormone induced by a variety of infections, general Do not use hormone therapy. Nephrotic syndrome patients before the laboratory tests nephropathy, taken to exclude lupus kidney disease, hepatitis B associated glomerulonephritis secondary nephropathy, active use of antimicrobial drugs to control the original infection focus, practice chest X-ray film, do tuberculin test, control and cure latent infection, and then be given hormone treatment.
Children with nephrotic syndrome need to consider carefully hormone therapy, hormonal medication during regular follow-up chest X-ray to check blood, urine; Once the infection should prompt use of antibiotics to control infection, in order to promote early remission and cure nephrotic syndrome.
The hormone is indeed the most effective way to treat nephrotic syndrome, but the application of the hormone is undeniable that the cause of nephrotic syndrome in children vulnerable to infection, thus affecting its efficacy. The rare infections including: acute upper respiratory tract infection, pneumonia, skin infections, sties, conjunctivitis, gingivitis, ulcers, or the occurrence of intestinal, biliary tract and urinary tract infection. Some can cause the resurgence and spread of tuberculosis. Addition, it is also easy to induce chickenpox, mumps, lymphadenitis and appendicitis and other diseases. Hormone side effects should not be underestimated, but also have a certain impact on the growth and development of children, and therefore need to consider carefully hormone treatment of children with nephrotic syndrome.
Children with nephrotic syndrome is a serious harm to the health of children diseases. For the treatment of children with nephrotic syndrome, involves the use of many drugs, following treatment of children with nephrotic syndrome commonly used drugs for everyone.
Children with nephrotic syndrome treatment commonly used drugs:
1. The Tripterygium 1 to 1.5mg / (kg · d) orally 3 times for kidney periods of each type.
2. Nephritis edema piece every time two, 2 to 3 times a day, the cold dampness spleen card suitable for renal disease.
3 of Niaoduqing particles each time 9g, 2 to 3 times a day for nephropathy in renal insufficiency uremia.
Nourishing the liver and kidney pills each time 3g, 3 times a day for a recovery period of liver and kidney nephropathy card.
5. Qiangshen piece 0.3g per piece, each 2 to 3, 3 times a day for yin and yang deficiency of kidney qi, not astringent edema.
In addition to the above commonly used drugs in children with nephrotic syndrome treatment, the following recommended several Pianfangyanfang for everyone, we want to help:
1. Astragalus 30 ~ 60g, Motherwort 15 ~ 30g, of Rhizoma Imperatae 30 ~~ 60g, jujube 10, decoction, one day, fractional service for nephropathy spleen and blood stasis, hot and humid.
Corn 60g, decoction, times service for nephrotic edema, proteinuria, hyperlipidemia.
Health Astragalus the Shi Wei 15g, Corn the Rhizoma Imperatae the 30g, Salvia 9g. Shuijianbi.
Children find out how to cure nephrotic syndrome? Adrenal corticosteroids for the treatment of children with nephrotic syndrome, better effect, commonly used in formulations as prednisone, dexamethasone. Available prednisone 1 to 2 mg / kg / day.
Diseases of children, parents should have the confidence to cooperate closely with the doctor, lasting uninterrupted treatment. Regular treatment in children should be done under the guidance of a doctor, longer course of treatment, do not easily stop. Treatment of nephrotic syndrome require large doses of assault treatment takes about a month or so, the patient can be alleviated BZX urine protein disappeared. To alleviate the side effects of hormone brought remission under the guidance of a doctor less drug treatment, until the final withdrawal.
Children find out how to cure nephrotic syndrome? Obesity medication will round fat face, body hirsutism and other side effects, and can disappear after drug withdrawal.
Such as the simple use of hormone therapy, the effect is not obvious, switch or combination of immunosuppressive agents such as cyclophosphamide, pay attention to changes in blood leukocytes during take this medication, and may have hair loss and other side effects. Edema severe cases, temporary diuretic, such as hydrochlorothiazide in urine thiophene, etc..
Because the hormone can suppress the body's immune system, so that patients with severe secondary infection can also cause high blood pressure and Cushing syndrome. Concurrent infection treatment available, such as penicillin or erythromycin. After a period of hospitalization in children, the condition is stable, you can go home cultivation.
Nephrotic syndrome is a stubborn illness, the basic characteristics of high proteinuria, hypoalbuminemia, edema and hyperlipidemia, difficult to treat, and great harm. At the same time, the nephrotic syndrome is often associated with many concurrent symptoms. Concurrent symptoms of nephrotic syndrome today detailed analysis for everyone, we want to help.
Parsing concurrent symptoms of nephrotic syndrome:
1 infection: mainly for pneumococcal pneumonia and peritonitis or sepsis. Due to the protein malnutrition · IgG level is low and complement protein component of low levels of leukocyte function decline, low ferritin and low zinc hyperlipidemia can lead to reduced immune function.
2 renal tubular dysfunction: because the massive proteinuria ultimately caused proximal tubular dysfunction. Showed renal Boring urine, urinary amino acids and proximal renal tubular acidosis.
Renal injury: the original glomerular diseases induced filtration rate decreased, renal arteriosclerosis, so the decline in glomerular filtration capacity.
4. Precipitating cardiovascular disease: long-term hyperlipidemia, especially plasma LDL concentrations, can trigger the hardening of the coronary arteries.
Thrombosis: antithrombotic factor, and serotonin original activity decreased ability to increase platelet aggregation, blood viscosity makes blood hypercoagulable state.
Whether patients with nephrotic syndrome can be recovered, in addition to whether the active treatment, there is a very important point is that diet is not scientific and rational. Scientific and reasonable diet is particularly important for the rehabilitation of patients with nephrotic syndrome, many patients are poorly understood, but what to eat in the end. Nephrotic syndrome diet introduction gave you today, and hope that through our introduction, allow kidney patients to understand.
Nephrotic syndrome diet:
Food: rice, millet, glutinous rice, corn, flour;
2, a small amount of fruit: apples, pears, peaches, red dates;
3, a small amount of lean pork, beef, chicken, duck, fish, day and night are not more than twelve (twelve / 24h), in patients with severe inedible;
4, a small amount of salt, the daily total does not exceed 3g small amount of monosodium glutamate (no more than 1g of salt per day in children).
5, oil: canola oil, sesame oil and a small amount of soybean oil, peanut oil, cooking oil;
Vegetables: melon, cucumber, tomato, lotus root, cabbage, radishes, cabbage, red amaranth, mushroom, white fungus, mushroom, pumpkin, snake gourd, sponge gourd,
The child had nephrotic syndrome should be how care? Difficult to cure children with nephrotic syndrome, harm is great. In order to achieve good results in the treatment, in addition to the active drug treatment, must also help children develop good habits, and more help for the rehabilitation of the child.
The child had nephrotic syndrome, how should I care? Common childhood nephrotic syndrome nursing considerations:
Children with nephrotic syndrome in children in stable condition, still need medication. Children with nephrotic syndrome in children are generally required to take the diuretic hormone medicines, children increased urine output, so parents must be with the teachers communicate better, to give them more help.
Children with nephrotic syndrome in children will be a substantial drain protein, and thus lead to protein deficiency phenomenon often occurs in vivo, it is to supplement the diet should be. The daily diet should contain adequate amounts of protein, such as fish, lean meat, poultry, soy products and so on. In addition, you should limit the intake of salt, a day is generally not more than two grams.
3 children with nephrotic syndrome in children with long-term use of hormones, which would lead to decreased immunity, easy concurrent respiratory tract infection, it should strictly limit the amount of children with nephrotic syndrome, children's activities, avoid strenuous exercise.
The child had nephrotic syndrome should be how to care for your introduction here, wished the children a speedy recovery.
Nephrotic syndrome should be diagnosed how? Timely discovery of nephrotic syndrome, early treatment, it is particularly important to check the understanding of the means and measures for nephrotic syndrome. Well, nephrotic syndrome diagnosis should pay attention to what? Diagnosed with nephrotic syndrome, how can it?
Nephrotic syndrome should be how to check the diagnosis? Check when diagnosed with nephrotic syndrome, the following points are very important:
Diagnosis of nephrotic syndrome should be specific history, search with or without latent causes as much as possible to find out the primary etiology.
Diagnosis of nephrotic syndrome should pay attention to the patient's age and gender, to pay attention to the young female patients with systemic lupus erythematosus. 7-10 years old, children and young people will have to pay attention to is allergic purpura, middle-aged patients to pay attention to exclude diabetic nephropathy, multiple myeloma, nodular artery go far.
(5) also renal biopsy diagnosis of nephrotic syndrome
(3) the diagnosis of nephrotic syndrome should pay attention to laboratory tests, patients with systemic disease manifestations, should be targeted for the appropriate checks.
(2) the diagnosis of nephrotic syndrome should also be noted renal manifestations of systemic disease, and not only pay attention to force entirely focused on the performance of kidney disease.
Treatment of nephrotic syndrome has been the problem of the medical profession. Nephrotic syndrome mainly massive proteinuria, edema, hyperlipidemia, hypoalbuminemia four symptoms With deepening of nephrotic syndrome, the treatment of nephrotic syndrome also in various fields continue to study Chinese medicine and Western medicine treatment are more common. That Which better? Below do the advantages and disadvantages of the treatment of nephrotic syndrome contrast, from which we can get some information to help you choose the right treatment.
Using traditional Chinese medicine in the treatment of nephrotic syndrome: Select Chinese medicine treatment or acupuncture and other auxiliary treatment.
Lee: side effects, and can be treated fundamentally, is particularly suitable for hormone-insensitive crowd.
Disadvantages: treatment time is too long, slow to bear fruit, the complexity of the treatment process, resulting in treatment is not in place and prone to relapse.
Western medicine treatment of nephrotic syndrome: mainly taking hormones, immunosuppressant drugs such treatment.
Lee: rely on hormones, immunosuppressants to clear a variety of illnesses, quick.
Disadvantages: You can not eliminate the root cause side effects, often recurrent phenomenon.
Nephrotic syndrome is not a serious disease? Widely nephrotic syndrome is an intractable disease, lingering course of the disease, the pathological changes are complex, easily recurrence of the disease, so clinical treatment is very difficult.
Nephrotic syndrome is not a serious disease? Nephrotic syndrome generally hyperlipidemia, proteinuria, and a high degree of edema and hypoproteinemia symptoms of, these symptoms although serious, but generally as long as early detection and early treatment of nephrotic syndrome patients can often obtain a better therapeutic effect.
Routine treatment of nephrotic syndrome, the use of hormone treatment, hormones can play the role of anti-inflammatory, anticoagulant, in a relatively short period of time, the body's inflammatory response of patients with nephrotic syndrome can be corrected quickly eliminate the adverse symptoms of the patient, the patient's condition was temporarily alleviate.
Already know from the above treatment of nephrotic syndrome, that since there is a reasonable treatment of nephrotic syndrome is not a serious disease? Fact, absolutely not to be taken lightly, once the patients suffer cold, fatigue, infections and other incentives role nephrotic syndrome easily relapse, the patient's condition and increase the level of patients with kidney damage, led to the aggravation of the disease difficult to treat.