chronic glomerulonephritis in children
Introduction
Introduction to chronic glomerulonephritis in children Glomerulonephritis with a course of more than 1 year, accompanied by renal dysfunction and/or hypertension and anemia, is called chronic glomerulonephritis. According to the recommendations of the National Pediatric Kidney Disease Research Collaboration Group, the disease course is more than 1 year, with varying degrees of renal insufficiency and/or persistent hypertension, and the prognosis of glomerulonephritis is called chronic glomerulonephritis. Chronic nephritis, chronic glomerulonephritis is a group of long-term disease (even decades) caused by various primary glomerular diseases with proteinuria, hematuria, edema, and hypertension as clinical manifestations. basic knowledge The proportion of illness: 0.013% Susceptible people: children Mode of infection: non-infectious Complications: hypertension, anemia, renal failure, uremia, malnutrition, heart failure
Cause
Causes of chronic glomerulonephritis in children
Primary (30%):
It can be delayed by acute nephritis, the inflammation progresses, and finally enters the stage of chronic nephritis. Some patients have no obvious acute nephritis, and the symptoms are concealed, but the inflammation progresses slowly and becomes chronic nephritis several years later.
Complement activation causes tissue damage (30%):
Most of them are immunological pathogenesis, most of which are immune complex diseases, including circulating immune complexes and in situ complexes. Complement activation causes tissue damage, and can participate in renal inflammatory lesions through classical pathways and bypass pathways. In addition, repeated infections, the body has inherent immunodeficiency and certain types of human leukocyte antigen (HLA) (such as HLA-A10, A2, Bw35, etc.) are considered to be associated with chronic nephritis, various types of chronic nephritis can be seen early Pathological changes, including proliferative glomerulonephritis, focal segmental glomerulosclerosis, membranous nephropathy, etc., when the chronic nephritis develops to the advanced stage, the renal tissue is severely damaged and forms a "terminal pyknosis kidney", which is lost. Function, as mentioned above, chronic nephritis includes a variety of etiology and pathological changes of glomerular disease, the cause of continuous and sustained progression after onset may be: 1 primary disease persistent activity. 2 The local hemodynamic changes of the glomeruli, that is, the residual renal unit without lesions, the glomerular local compensatory hyperperfusion, high filtration changes, and eventually hardening. 3 sustained proteinuria-induced glomerular mesangial phagocytosis clearance function is too long-term overload, and can cause renal tubules and interstitial lesions. 4 At the same time, the effect of hypertension on glomerular capillaries, many of the above factors lead to more glomerular sclerosis, renal dysfunction, until the development of uremia.
Secondary (15%):
Can be secondary to systemic diseases, such as allergic purpura, systemic lupus erythematosus, diabetes and so on.
Hereditary (15%):
Hereditary nephritis, polycystic kidney disease, etc.
Prevention
Prevention of chronic glomerulonephritis in children
The fundamental prevention of primary chronic nephritis is to prevent streptococcal infection. On weekdays, exercise should be strengthened. Pay attention to skin cleanliness and hygiene to reduce respiratory and skin infections. If infection occurs, it should be thoroughly treated in time. 2 to 3 weeks after infection, urine should be checked. Regular and timely detection of abnormal and timely treatment, so as not to develop into chronic nephritis, for the treatment of chronic nephritis secondary to systemic diseases, such as allergic purpura, etc., in order to avoid chronic nephritis.
Complication
Complications of chronic glomerulonephritis in children Complications, hypertension, anemia, renal failure, uremia, malnutrition, heart failure
Later, it can develop into renal insufficiency, high blood pressure, anemia, kidney failure, and development of uremia, heart failure, etc., which are mainly caused by impaired renal parenchyma, reduced red blood cell production and malnutrition, anemia and heart failure. The severity is directly proportional to renal disease and renal dysfunction, and it is easy to be complicated with infection and other complications.
Symptom
Chronic glomerular nephritis symptoms in children Common symptoms Loss of appetite, glomerular filtration rate, nocturnal urinary tract, proteinuria, urinary urinary tract, urinary tract, arterioles, glomerular sclerosis
1. Onset and progression
Clinical onset and progression of chronic nephritis are: (1) onset of acute nephritis or nephrotic syndrome: prolonged course of disease, and eventually enter chronic nephritis, (2) insidious onset: can be examined in physical examination, screening Urinary abnormalities or high blood pressure were found, and further examination was diagnosed as chronic nephritis. (3) It started with non-specific symptoms: such as paleness, fatigue, growth retardation, etc., when the doctors had varying degrees of renal failure, in children. More acute onset, and the subsequent development of the condition is closely related to the nature of primary glomerular lesions, and secondly related to the treatment measures taken, such as the timely control of concurrent infections and hypertension, children with acute onset Some of the progress is rapid, edema, high blood pressure, oliguria and renal function are deteriorating, and can die within 1 to 2 years. In other cases, although the edema subsides, the urine is abnormally persistent, often infected, and repeated after the fatigue. Attack, kidney function continues to deteriorate, after several years or decades into chronic renal insufficiency, once there is significant chronic renal failure, such as no dialysis and transplantation, often died within a few months to 1-2 years.
2. Classification
Generally divided into common type, kidney disease type and high blood pressure type.
(1) Ordinary type: 1 General performance: pale, fatigue, growth retardation, loss of appetite, etc. 2 specific performance: including varying degrees of edema, hypertension, anemia, abnormal urine (proteinuria, hematuria, tubular urine), nocturia, varying degrees of renal dysfunction (glomerular filtration rate decreased and urine Concentrated function is impaired.
(2) Kidney disease type: It is characterized by a large amount of proteinuria, which is called nephropathy.
(3) Hypertensive type: those with severe hypertension are called hypertensive.
3. Classification by pathology
The clinical features of various chronic nephritis classified by pathological type are as follows:
(1) Membrane proliferative nephritis: more common in school-age children and adolescents, more women than men, onset of disease: 1 acute nephritis syndrome, that is, acute onset, hematuria, edema, high blood pressure. 2 repeated episodes of gross hematuria, these two often use non-specific upper respiratory tract infection as the precursor of the episode. 3 onset of nephrotic syndrome. 4 a small number of urine routine screening tests found abnormal urine [proteinuria and / or hematuria] and the systemic symptoms are not obvious at the time, one of the clinical features of this disease is complement (C3) is low, it is also known as low-complement nephritis, Anemia is also relatively heavy, and the decrease of plasma protein and hyperlipidemia is inferior to that of small lesions, and the course of disease is prolonged. Although there are intermittent clinical symptoms, most of the patients have chronic renal insufficiency, and there is hypertension in the early stage of the disease. Patients with hypofunction have poor prognosis, are often insensitive to conventional corticosteroid therapy, and sometimes develop hypertensive encephalopathy. They can also relapse on transplanted kidney after transplantation. However, in recent years, many cases have reported that corticosteroids have been used for a long time. Dayton clothing) combined with immunosuppressive agents, anti-platelet aggregation agents and other treatments, there are quite a few cases to ease or maintain stability.
(2) Focal segmental glomerulosclerosis: Most of them start with nephrotic syndrome, some are asymptomatic proteinuria, and 1/3 to 1/2 cases have high blood pressure, hematuria and serum creatinine The patients with renal disease are mostly steroid-resistant or hormone-sensitive. After repeated recurrence, they are converted to steroid resistance. The course of the disease is generally progressive deterioration. The so-called malignant children can enter renal failure within a few months to three years. Such pathological changes are often caused by collapse-type changes, and 20% to 30% of cases after renal transplantation recur in the transplanted kidney.
(3) membranous nephropathy: primary is rare in children, most manifested as nephrotic syndrome, a small number of asymptomatic proteinuria, proteinuria is non-selective, often accompanied by microscopic hematuria, early generally without hypertension and kidney Incomplete function, the prognosis is relatively better in children than in adults. Habib followed up to 69 years of pediatric patients for 5 years, and 10% entered chronic renal insufficiency.
(4) sclerosing glomerulonephritis: a part of the history of acute nephritis, followed by or prolonged unhealed, or after the asymptomatic stage and then again, can also conceal the onset, often accompanied by hypertension and renal dysfunction, can be The upper respiratory tract infection is sharply aggravated.
(5) mesangial proliferative nephritis: clinically or after acute nephritis prolonged unhealed, or hematuria and / or proteinuria, or nephrotic syndrome onset, although the course of disease is delayed, but the true entry into renal dysfunction is a minority, More manifestations of prolonged nephritis, a history of nephritis more than 1 year, accompanied by hypertension, renal insufficiency and anemia, can make a clinical diagnosis, renal biopsy can confirm the diagnosis and understand the severity of the lesion and guide treatment.
Examine
Examination of chronic glomerulonephritis in children
Urine check
(1) General urine examination: abnormal urine is a must-have phenomenon of chronic nephritis. The change of urine volume is related to edema and renal function. The urine volume is reduced during edema, the urine volume of edema is mostly normal, and the renal function is obviously reduced. Patients with dysfunction often have nocturia and polyuria, urine specific gravity (no water for 10 ~ 12h) does not exceed 1.020, urine osmotic concentration is lower than 550mOsm / (kg · H2O), urine protein content varies, generally 1 ~ 3g / d It can also be a large amount of proteinuria (>3.5g/d), and granular tube type can be seen in urine sediment with mild to moderate hematuria.
(2) Other urine examinations: In addition to the above-mentioned urine routine examination and various clearance rate examinations, the following urine examinations may be helpful for diagnosis: 1 urine C3 measurement: the highest positive rate of membrane proliferative nephritis and crescentic nephritis , up to more than 90%; followed by focal segmental glomerular sclerosis, membranous nephropathy, mesangial proliferative nephritis (including IgA nephropathy), with minimal lesions and focal segmental nephritis with the lowest positive rate. 2 urine FDP measurement: the results are similar to C3 determination, diffuse proliferative nephritis and focal segmental glomerular sclerosis positive rate, focal segmental nephritis and small lesions are lower. 3 urinary disk electrophoresis: the presence of high molecular proteinuria is more common in mesangial proliferative nephritis, mesangial glomerulonephritis and focal segmental glomerulosclerosis. The main molecular proteinuria is more common in microscopic lesions, IgM nephropathy. , early mesangial proliferative nephritis and membranous nephropathy. 4 urinary protein selectivity index: its clinical significance is similar to urinary disk electrophoresis, membrane proliferative nephritis, focal segmental glomerulosclerosis and IgA nephropathy mostly non-selective proteinuria (SPI>0.2) minimal lesions, IgM More than half of the cases of nephropathy, mesangial proliferative nephritis and membranous nephropathy have SPI 0.2.
2. Renal function test
The renal insufficiency of glomerulonephritis mainly refers to the decrease of GFR. The creatinine clearance rate (Ccr) of most patients is slightly decreased at the time of treatment, and it does not fall below 50% of the normal value. Therefore, serum creatinine and urea nitrogen can be in the normal range. If Ccr falls below 50%, serum creatinine and urea nitrogen will increase. In other words, when serum creatinine reaches 133 mol/L, Ccr is below 50% of normal, followed by renal tubular insufficiency, such as urine concentration. The dysfunction and phenol red excretion rate are significantly reduced, and the phenol red excretion rate is greatly affected by the amount of renal plasma, which cannot fully represent the renal tubular function.
3. Blood examination Chronic nephritis In addition to the above-mentioned urine routine and renal function tests, there are other tests that are helpful for diagnosis and prognosis.
(1) Peripheral blood test: Chronic nephritis in mild edema, there is mild anemia, may be related to blood dilution, such as patients with moderate anemia, most related to the reduction of erythropoietin in the kidney, indicating nephron damage and kidney Dysfunction has been more serious. At the end of glomerulonephritis (sclerosing, atrophic glomerulonephritis), severe anemia occurs. If the patient has no obvious malnutrition, the anemia is mostly positive cells and positive pigmentation.
(2) Serum complement determination: Determination of pre-complement components (such as C1q, C2, C4 and C3) can identify two complement activation pathways in this disease mechanism. General hospitals only do total complement CH50 or C3 determination, now Hayslett et al ( The data reported in 1975) are summarized as follows: 1 Normal serum complement: minimally pathological nephropathy, focal and segmental glomerulonephritis, membranous nephropathy, sclerosing glomerulonephritis, systemic diseases such as allergic vasculitis , nodular polyarteritis, Wegner granuloma, allergic purpura, diabetic glomerular sclerosis, amyloidosis, hemolytic uremic syndrome, gestational kidney, thrombotic thrombocytopenic purpura and other glomerular lesions. 2 initial serum complement reduction: capillary endovascular and mesangial proliferative glomerulonephritis, extra-optic proliferative nephritis. 3 serum complement continued to reduce: membrane proliferative nephritis, lupus membranous nephritis and lupus diffuse proliferative nephritis.
(3) Determination of serum 2-macroglobulin and immunoglobulin: serum 2-macroglobulin (2-macroglobulin) is increased in persistent renal damage, and can be reduced to normal or below in chronic uremia, 2-giant Increased globulin, inhibition of plasminogen and plasmin, is also the cause of hypercoagulable state, according to Zhou Xijing et al (1986), 96.4% of 30 patients increased, Chen Huiping et al reported that membrane proliferative nephritis, focal section The positive rates of segmental glomerulosclerosis and IgA nephropathy were 83.9%, 66.7% and 79%, respectively. Serum immunoglobulin has diagnostic and differential diagnosis significance in chronic nephritis. Serum IgG of minimally pathological nephropathy and chronic nephritis nephropathy And IgA decreased, mainly IgG reduction, but in the case of minimally pathological nephropathy, most patients have elevated serum IgM, and some patients with chronic nephritis increased, so Giangiacomo et al believe that IgG and IgA decrease in small lesions, and IgM increase is antibody The formation of cells does not convert IgM into IgG and IgA, supporting the view that there is insufficient T cell function (immune immune function). In addition, excessive IgG loss from urine is also a cause of decreased serum IgG, and serum IgG is increased. Most secondary glomerulonephritis seen, such as multiple myeloma nephropathy, lupus nephritis, nephritis, vasculitis syndrome caused by abnormal hypergammaglobulinemia and the like.
(4) Determination of serum 2-microglobulin: serum 2-microglobulin (2-microglobulin) is closely related to glomerular filtration rate. The normal value of adult is 12.7mg/L. When the GFR is decreased, serum 2- Microglobulin and creatinine are both increased. Some people think that the former is more obvious than the latter, and it is more sensitive, so it can be used as an early indication of renal dysfunction of glomerulonephritis.
(5) Coagulation factor examination: There are different degrees of hypercoagulability in glomerular diseases, the degree of which is parallel with the severity and activity of the lesions. The hypercoagulable state is the imbalance of coagulation and fibrinolysis processes, plasma coagulation factors. Activated, the number of platelets increased, and the activity of fibrinolytic system (plasminogen) decreased. According to domestic and foreign literature reports, the partial thromboplastin time (KPTT) and prothrombin time (PT) of white clay were shortened during chronic nephritis. Increased proteinogen and increased FDP, thrombocytic factor VIII and its related antigen (VIIIRAg), decreased antithrombin III (ATIII) activity, increased thromboxane B2 (TXB2) and abnormal thromboelastogram, indicating the presence of hypercoagulable state. The presence of hypercoagulable state of glomerulonephritis can be used as an indication for anticoagulant therapy.
(6) blood lipid examination: persistent glomerular disease, often accompanied by abnormal blood lipids, hyperlipidemia can accelerate kidney damage and atherosclerosis,
4. Renal biopsy Renal biopsy determines the pathological type and the severity of the lesion, which is important for treatment and prognosis.
5. Kidney B-ultrasound To understand the size of the kidney and the thickness of the cortex, the thickness of the renal cortex is <1.5cm. It is judged that CRF is better than the size of the kidney, and the kidneys are atrophied to support the end-stage diagnosis.
6. Other
Conventional electrocardiogram, X-ray, bone and gastroscopy.
Diagnosis
Diagnosis and diagnosis of chronic glomerulonephritis in children
diagnosis
Diagnosis can be based on the cause, symptoms and related tests.
Differential diagnosis
The etiology of this disease should also be distinguished as primary, secondary or hereditary nephritis as much as possible, and should be identified with the following diseases:
1. Congenital renal hypoplasia or malformation, with or without infection.
2. Chronic urinary tract infections, especially pyelonephritis.
3. Acute exacerbation of chronic nephritis should be distinguished from acute glomerulonephritis.
4. Chronic nephritis common type needs to be differentiated from chronic pyelonephritis.
5. Chronic nephritis nephropathy type and hypertensive type, secondary and hereditary chronic nephritis must be identified.
