Clinical classification of nephrotic syndrome

Children with nephrotic syndrome with clinical manifestations of these four, but different causes pathological changes in the different variety of glomerular diseases, and therefore researchers from different angles to give a classification or type, in order to guide clinical work and to explore disease is the essence.
Clinical classification of pediatric clinical traditional view, this syndrome is divided into three categories namely primary, secondary and congenital three kinds.
1) The primary nephrotic syndrome refers to the etiology is not clear, the primary lesion in glomerular diseases. In the process of primary glomerular diseases, such as acute glomerulonephritis, rapidly progressive glomerulonephritis, chronic glomerulonephritis, the NS can be found in the disease process. Domestic clinical classification can be divided into simple-type (Ⅰ) and nephritis (Ⅱ). The former only have these massive proteinuria, hypoalbuminemia, hyperlipidemia, and edema of the four characteristics; the latter in addition to have these four clinical manifestations, but also have one or more performers in the following four:
① urine red blood cells> 10 / high power field (three times within two weeks centrifuged urine tests);
The ② repeated or sustained hypertension: school-age children> 17.3/12.0kPa (130/90mmHg), pre-school children> 16.0/10.7kPa (120/80mmHg), and the exclusion caused by the use of corticosteroids;
③ azotemia: plasma urea nitrogen> 10.7mmol / L (30mg/dl), to exclude hypovolemia due;
④ serum total complement the lower body activity or complement C3 repeatedly. Clinical common type Ⅰ: Ⅰ type is 68.4%, according to our data analysis of 1462 cases of hospitalized cases 31.6% of type II.
2) secondary nephrotic syndrome refers to the secondary to systemic disease (such as systemic lupus erythematosus), or with a clear cause (eg, infection). Its etiology is broad and complex, the following list only the more common of the children during the
(1) the generalized systemic disease: systemic lupus erythematosus, purpura, nodules nodosa, mixed connective tissue disease, dermatomyositis, etc..
(2) infections: bacterial infections: post-streptococcal glomerulonephritis, bacterial endocarditis, cardiac shunt infection nephritis; viruses and other infections: hepatitis B, hepatitis C, cytomegalovirus, varicella and Epstein-Barr virus; malaria, congenital or secondary syphilis.
(3) Drug Allergy: penicillamine, probenecid, mercury, three pairs of ketones, captopril, nonsteroidal anti-inflammatory drugs, interferon, serum and vaccination and so on.
(4) family of genetic diseases: Alport syndrome, a patella syndrome and sickle-cell anemia.
(5), metabolic diseases: diabetes mellitus, myxedema.
(6) tumor: Wilms' tumor, leukemia, Hodgkin's lymphoma and multiple myeloma and other.
(7): the response of chronic renal allograft Exhaust malignant glomerulosclerosis and renal artery stenosis
.
3), congenital nephrotic syndrome is often caused by genetic factors of the Finnish type and non-Finnish type congenital nephrotic.
1990 Steffensen three months will be born with congenital kidney disease is divided into five categories:
① Finnish type of congenital kidney disease: an autosomal recessive genetic disease;
② diffuse mesangial sclerosis: more common in full-term children, there is a tendency of familial disease, the rapid development of the disease, more than before the 3-year-old died of renal failure. Glomerular involvement, by a majority of the small ball of capillary lumen occlusion with fibrosis.
③ children with congenital nephrotic: the country is more common, the incidence in the 3 months to 3-year-old children, more common in 1-3 year-old infant. Diversify its pathological type, such as minimal change disease, focal glomerulosclerosis, proliferative nephritis (including diffuse, exudative, Department of membranous, crescent form of focal, membrane proliferative, etc.) as well as renal ball sclerosis. With primary nephrotic organizations to learn different points of this disease is mesangial proliferative glomerulonephritis without immune deposits visible.
④ secondary to congenital kidney disease: the majority of secondary syphilis infection and its pathological types of mesangial proliferative glomerulonephritis and membranous nephritis, basement membrane thickening is a major exception. Optical microscopy and electron microscopy showed subepithelial deposits (IgG, fibrin). Secondary to toxoplasmosis, renal pathology, diffuse mesangial small hardening. Secondary to cytomegalovirus inclusion virus infection, renal pathology showed the expansion of proximal tubule the ball moderate mesangial cell proliferation and interstitial inflammation.
⑤ congenital nephrotic combined with other genetic diseases such as nail - patella dysplasia (autosomal dominant disease), genital abnormalities, and eye - diaphragm - with renal syndrome (sex-linked recessive inheritance).