2014年12月5日星期五

Nephrotic syndrome and the diseases easily confused

Diagnosis of primary nephrotic syndrome, we must first and secondary nephrotic syndrome differentiated. Common clinical shall identify the disease secondary to nephrotic syndrome are the following:

1. nephritis patients with rash, purpura, arthralgia, abdominal pain and bloody diarrhea and other performance characteristics, but also the characteristics of nephritis hematuria, proteinuria, edema, hypertension and so on. If purpura characterized by atypical, often misdiagnosed as primary nephrotic syndrome. The disease is often associated with an early increase in serum IgA. Diffuse mesangial proliferative renal biopsy is a common pathology, immunopathology is mainly IgA and C3 deposits, it is not difficult to identify.

2. lupus nephritis is more common in the 20 to 40 year-old female, many patients have fever, rash and joint pain, serum antinuclear antibody, anti-ds-DNA, anti-SM antibodies, complement C3 decreased renal biopsy light microscopy in addition to mesangial hyperplasia, the lesions characteristic diversity. Immunopathology was "full house."

3. diabetic nephropathy patients prone to diabetes for more than 10 years, can be expressed as nephrotic syndrome. Fundus examination microvascular change. Renal biopsy shows thickening of the glomerular basement membrane and mesangial matrix, typical damage for Kimmelstiel-Wilson nodules. Renal biopsy can confirm the diagnosis.

The hepatitis B virus associated nephritis with nephrotic syndrome virus confirmed viremic serum, kidney biopsy found free of hepatitis B virus antigen components.

5.Wegner nose and granulomatous inflammation, pneumonia, three characteristics of necrotizing glomerular disease-oriented necrotizing sinuses. Clinical features of renal damage is Rapidly progressive glomerulonephritis or nephrotic syndrome. Serum γ-globulin, IgG, IgA increased.

6. Early amyloid nephropathy can only proteinuria, usually after 3 to 5 years nephrotic syndrome, serum γ-globulin increased, heart, liver, splenomegaly, skin serum γ-globulin lichen-like myxoid edema, diagnosed rely on biopsy.

7. malignancy nephrotic syndrome nephrotic syndrome can be caused by a variety of malignant tumors by the immune system, and even for the early clinical manifestations of nephrotic syndrome. Therefore, nephrotic syndrome patients should do a comprehensive examination to exclude malignancy.

8. after renal transplantation relapse after kidney transplant kidney transplant nephropathy syndrome recurrence rate of about 10 percent, usually after 1 week to 25 months, proteinuria, recipients often severe nephrotic syndrome and 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 are causing nephrotic syndrome (such as membranous nephropathy) reported. 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 a kidney complications in patients with AIDS, more 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 populations), early detection of HIV testing and anti-HIV antibodies, combined with other clinical manifestations of HIV (such as asymptomatic infection, persistent lymph node enlargement, secondary tumors) can contribute to the diagnosis of HIV-AN. Combined with kidney disease and proteinuria in patients with nephrotic syndrome syndrome range, especially for the short-term possibility of renal disease in patients with focal segmental glomerulosclerosis deterioration should think. The pathological features of HIV-AN is: global or segmental glomerular capillary wall contraction and collapse, accompanied by significant podocyte proliferation; light microscope as long as any one of the glomeruli were global collapse or 20% above showed segmental glomerular collapse of the disease should be considered; electron microscope HIV-AN glomerular endothelial cells, white blood cells in the interstitial mesh tubes have a lot of 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 polymyositis or dermatomyositis mixed performance of the three diseases, but not one disease diagnosed, and more can be detected in serum high titers of anti-RNP antibodies, 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 due to renal impairment may have the following conditions: arthritis pain drug nephropathy, rheumatoid arthritis complicated by renal amyloidosis, rheumatoid arthritis complicated by glomerulonephritis. Rheumatoid arthritis complicated by nephrotic syndrome is very rare, its rheumatoid factor positive, biopsy is the most common mesangial glomerulonephritis, after application of corticosteroids may improve.

13. cryoglobulinemia purpura experiencing kidney damage, joint pain, Raynaud's phenomenon, clinical hepatosplenomegaly, lymphadenopathy, visual disturbances, syncope and cerebral vascular formation, concurrent glomerulonephritis, should consider this disease, further confirmed in cold blood globulin, to confirm the diagnosis. Cryoglobulinemia can cause kidney damage. 1/3 patients with chronic renal disease in clinical practice, mainly for proteinuria and microscopic hematuria, nephrotic syndrome and can often occur with hypertension, the prognosis is poor. A few patients presented with acute nephritic syndrome, and some showed a rapidly progressive nephritic syndrome, the development of direct to end-stage failure.

14. Sarcoidosis Sarcoidosis is a rare kidney damage of unknown etiology multisystem granulomatous disease, renal involvement is less common, clinical renal damage by about 1%, biopsy and autopsy found that kidney disease by up to 20% , mainly in the 30 to 50 years old, men and women may be the disease. Diagnosis relies on laboratory tests and chest photo, lymph nodes, skin, liver, renal biopsy, serum angiotensin-converting enzyme activity increased and so contribute to the diagnosis. Sarcoidosis kidney damage can be divided into: a direct violation of granulomatous kidneys, kidney damage caused by abnormal calcium metabolism and glomerulonephritis.

15. Cellulose glomerular disease onset is more common 40 to 60 years, 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 few cases of malignant tumor. Electron microscope shows a similar amyloid-like filaments were fiber-like substance or micro hollow tube-like structure, but for Congo red staining, Thioflavin T and other were negative. Currently, the disease tends to put the two as the same.

16. collagen Ⅲ glomerulopathy adults and children may be the disease, more common in men. Autosomal recessive, often characterized by proteinuria and nephrotic syndrome, renal function over a long period, 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 in the strong positive collagen Ⅲ.

17. fibronectin glomerular disease 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 examination under an optical microscope hyaline substance visible mesangial and subendothelial homogeneous (PAS-positive), Congo red stain was negative, electron microscopy examination showed capillary loop cavity filled with fine granular electron-dense material, basement normal thickness, these negative for Congo red staining fiber diameter than the tubular immune diseases fiber small, its distribution and optical microscopy seen consistent PAS-positive substance; immune pathology department glomerular fibronectin staining strongly positive, and diffuse distribution in mesangial and subendothelial area to help confirm the diagnosis.

18. lipoprotein glomerular disease is more common in men, the majority was sporadic, and the minority of familial disease. The presence of proteinuria in all patients, and some gradual progression to nephrotic range proteinuria, lipoproteins are not formed outside the kidney embolism. The pathology is characterized by the presence of highly inflated glomerular capillary cavity layer change "lipoprotein embolus" lipoprotein positive staining, confirmed that "lipoprotein embolism" under the electron microscope, and the presence of abnormal blood lipid metabolism the diagnosis is not difficult to establish. The disease is not exact and effective treatment.

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