2014年5月8日星期四

Nephrotic syndrome and the diseases easily confused ?

Nephrotic syndrome is a group of clinical manifestations similar syndrome, rather than a separate disease. The main clinical manifestations of massive proteinuria , hypoalbuminemia , edema , hyperlipidemia . Many causes of nephrotic syndrome. To sum up can be divided into two categories. Namely: primary and secondary.
Diagnosed with primary nephrotic syndrome , we must first and secondary nephrotic syndrome phase identification. Common clinical shall identify the disease secondary to nephrotic syndrome are the following :
1 nephritis patients with rash , purpura , arthralgia, abdominal pain and blood in the stool and other performance characteristics , but also the characteristics of nephritis hematuria , proteinuria, edema, and hypertension. If purpura characterized by atypical , often misdiagnosed as primary nephrotic syndrome. The disease is often accompanied by an early rise in serum IgA . Renal biopsy diffuse mesangial proliferation is a common pathology , IgA , and C3 immune pathology is the main sediment , it is not difficult to identify.
2 common in lupus nephritis women aged 20 to 40 , many patients have fever, rash and joint pain , serum antinuclear antibodies, anti- ds-DNA, anti- SM antibodies, complement C3 decreased renal biopsy light microscopy in addition to mesangial hyperplasia , the lesions characterized by diversity . Immunopathology was " full house ."
3 nephropathy in diabetes -prone patients over 10 years , can be expressed as nephrotic syndrome. Fundus examination microvascular change. Renal biopsy shows glomerular basement membrane thickening and mesangial matrix , typical damage to Kimmelstiel-Wilson nodules. Renal biopsy can confirm the diagnosis .
4 hepatitis B virus associated glomerulonephritis may manifest as nephrotic syndrome , viral serum examination confirmed viremic renal pathology found free of hepatitis B virus antigen components .
5.Wegner granulomatous inflammation of the nose and pneumonia , necrotizing glomerulonephritis three characteristics of the disease necrotizing sinuses . Clinical features of renal damage is rapidly progressive glomerulonephritis or nephrotic syndrome. Serum γ -globulin , IgG, IgA increased.
6 amyloid early nephropathy can only proteinuria, usually after 3 to 5 years nephrotic syndrome, serum γ globulin , heart, liver, splenomegaly, skin serum γ -globulin lichen-like myxoid edema, diagnosed rely on biopsy .
7 . Nephrotic syndrome caused by a variety of malignant tumor can cause nephrotic syndrome by the immune system, even for the early clinical manifestations of nephrotic syndrome . Therefore, nephrotic syndrome patients should do a comprehensive examination to exclude malignancy.
8 transplanted kidney after renal transplantation recurrence after renal allograft nephropathy syndrome recurrence rate of about 10 %, usually after 1 week to 25 months, proteinuria , recipients often severe nephrotic syndrome, and in six months to 10 years, the loss of the transplanted kidney .
9 drug-induced nephrotic syndrome organic gold, mercury , D- penicillamine , captopril ( captopril ) , non-steroidal anti-inflammatory drugs have been reported to cause nephrotic syndrome ( such as membranous nephropathy ) in . It should be noted medication history, timely withdrawal may cause remission .
10 The human immunodeficiency virus -associated nephropathy human immunodeficiency virus-associated nephropathy (HIV-AN) is AIDS complications in patients with kidney , common in early HIV infection , before other serious infections. According to whether patients with HIV risk factors ( such as intravenous drug abuse , homosexuality , HIV -prone areas and population ) , early detection of HIV testing and anti- HIV antibodies , combined with other clinical manifestations of HIV ( eg asymptomatic infection , persistent lymph node swelling, secondary tumors ) can help HIV-AN diagnosis. Combined with kidney disease and proteinuria in patients with nephrotic syndrome range syndrome, especially for the short-term possibility of renal disease in patients with focal segmental glomerulosclerosis deterioration should expect. Science nephropathy is characterized by HIV-AN : global or segmental glomerular capillary wall contraction and collapse, with marked podocyte proliferation ; optical microscopy was as long as any one of the glomerulus , or 20 percent of global collapse the above segmental glomerular collapse of the disease should be considered ; electron microscope HIV-AN glomerular endothelial cells, the white blood cells have a lot of interstitial mesh tube inclusions ( present in 80% to 90 % of HIV -AN patients ) , to help establish the diagnosis .
11 mixed connective tissue disease patients with renal impairment have both systemic sclerosis, systemic lupus erythematosus and dermatomyositis, polymyositis or mixed performance of the three diseases , but one can not diagnose the disease , can be detected in the serum and more high antibody titers of anti- RNP , anti- SM antibody negative serum complement almost normal. Only about 5% of renal damage , mainly as proteinuria and hematuria , nephrotic syndrome may also occur , with normal renal function , renal biopsy pathology mostly mesangial proliferative glomerulonephritis or membranous nephropathy . Glucocorticoid response is good, the prognosis is good .
12 rheumatoid arthritis and kidney damage occurs in women aged 20 to 50 , a lower incidence of renal damage , renal damage may be caused by the following conditions : rheumatoid arthritis pain medicine kidney disease, rheumatoid arthritis complicated by renal amyloidosis , rheumatoid arthritis, glomerulonephritis concurrently . Rheumatoid arthritis complicated by nephrotic syndrome is very rare , its rheumatoid factor positive , the most common biopsy mesangial glomerulonephritis, after application of corticosteroids may improve .
13 cryoglobulinemia kidney damage encountered in clinical purpura , arthralgia , Raynaud's phenomenon , hepatosplenomegaly , lymphadenopathy , visual disturbances, syncope and cerebral vascular formation , concurrent glomerulonephritis, should consider this disease , and further confirmed in cold blood globulin , can confirm the diagnosis. Cryoglobulinemia can cause kidney damage. 1 /3 of patients with chronic renal disease in clinical practice , mainly for microscopic hematuria and proteinuria , nephrotic syndrome can occur frequently and hypertension , the prognosis is poor. A few patients showed acute nephritic syndrome , and some can be presented rapidly progressive nephritic syndrome, directly to the development of end-stage failure.
14 kidney damage sarcoidosis Sarcoidosis is a rare multisystem unknown etiology granulomatous disease , renal involvement is less common, clinical renal damage by about 1% , biopsy and autopsy nephropathy were found up to 20% , mainly in the 30 to 50 years old , men and women may be the disease . Diagnosed mainly rely on laboratory tests and chest photo , lymph nodes, skin , liver, renal biopsy , elevated serum angiotensin- converting enzyme activity , such as the diagnosis. Kidney damage sarcoidosis can be divided into : a direct violation of granulomatous kidney , kidney damage caused by abnormal calcium metabolism and glomerulonephritis .
15 Cellulose glomerular disease more common 40 to 60 years the incidence of male ones, the majority of patients with microscopic hematuria , almost all patients had proteinuria, 60% to 70 % of patients with nephrotic syndrome, proteinuria category , more than 50% of patients with hypertension, the vast majority of non- systemic disease , there are a few cases of malignant tumor . Electron microscope shows the presence of amyloid fibrils similar to silk -like material or fiber -like substance was slightly hollow tube-like structure , but for Congo red staining , such as Thioflavin T staining. Currently, tend to both as the same disease.
16. Collagen Ⅲ glomerular disease in adults and children may be the disease , more common in men . Autosomal recessive , often characterized by proteinuria and nephrotic syndrome , renal function over the long term , and then gradually progress to chronic renal failure , renal biopsy examination is the only means of diagnosis of the disease , immunofluorescence visible widened mesangial area and throughout the capillaries were seen strongly positive collagen Ⅲ.
17 fibronectin glomerular disease onset in 14 to 59 years, more common in young people under the age of 30 , men and women can be affected , the disease is autosomal dominant . Proteinuria is a common clinical manifestation of the disease , 50% of patients with hypertension. Renal biopsy pathology transparent optical microscope -like substance visible under mesangial and endothelial homogeneous (PAS -positive ) , Congo red staining was negative, electron microscope examination showed capillary loop cavity filled with fine granular electron-dense material , basement normal thickness , these negative Congo red staining fiber diameter than the tubular immune diseases fiber is small, its distribution seen with an optical microscope PAS -positive material consistent ; immune pathology department glomerular fibronectin staining strongly positive , and diffuse mesangial area and in the endothelium , to help confirm the diagnosis.

18. Lipoprotein glomerular disease is more common in men, the majority was sporadic , a small number of familial disease . The presence of proteinuria in all patients , and some gradual progression to nephrotic range proteinuria , lipoprotein not renal embolization . The pathology is characterized by the presence of highly inflated glomerular capillary cavity layered change " lipoprotein emboli ," lipoprotein positive staining , confirmed that " lipoprotein embolism " under the electron microscope , and the presence of serum lipid metabolism diagnosis is not difficult to establish . The disease is no precise and effective treatment .

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