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Minimal change disease diagnosis and treatment of kidney disease

2017-01-13 14:16

Kidney disease is a group of pathology is minimal change disease in glomerular epithelial cell foot process fusion characteristics, clinical disease presenting with simple nephrotic syndrome, thanks to its basic normal, then glomerular and proximal renal tubular epithelial cells steatosis, so called "lipid renal disease" at the turn of the century. Pathological features: lens: the basic normal glomerulus. Occasional epithelial cell swelling, vacuoles degeneration and very light mesangial cell proliferation, wider matrix. Proximal renal tubular epithelial cells in the fat droplets containing double refraction. Can also be stove shaped renal tubular epithelial damage and interstitial lesions. Fluorescence microscope: without immunoglobulin and complement both dyeing is one of the characteristics of this disease. Occasional immunoglobulin and complement C3 ingredients calm. This feature with IgA and IgG light phase identification of mesangial proliferative glomerulonephritis. Electron microscopy (sem) : a wide range of epithelial cell foot process fusion with epithelial cell vacuoles degeneration, microvilli forming, protein absorption drops and the lysosome increased. Within the basement membrane is normal, but irregular thickening of the thin layer. Along the basement membrane two side electron dense AD cool-headed. I have a visible small electron dense mesangial area. Clinical manifestation: most patients have disease without any cause. But some patients after onset in upper respiratory tract infection or allergy. Edema is often after the onset of the first performance. Facial edema and posture sex is swollen, serious body cavity effusion, inspection is a typical simple nephrotic syndrome. Daily can express several grams of urine protein, it can be a selective proteinuria, but adult patients often assumes the non-selective proteinuria. Urinary sediment visible fat body, transparent tube type and particle shape, and more glomerular epithelial cells, such as do not pay attention to identify easily mistaken for white blood cells. Rarely appear to the naked eye hematuria, microscopic haematuria can be found in a third of the patients, the adult patients. High blood pressure or orthostatic hypotension, pressing of arteries and veins (related to blood volume decline). Plasma albumin significantly decreased, can reach 10 g/L below, so that the plasma colloid osmotic pressure drop, with serious hyperlipidemia. To ease and recurrent as feature in the clinical setting. Before application of glucocorticoid hormones and antibiotics mortality rate high mortality rate of 36% (two years), the patient died of massive proteinuria main cause of the pathophysiological changes and infection. Application of glucocorticoids in time control a large amount of proteinuria, with antibiotics to control infection, 10 years survival rate reached 95%. But the natural development of cortical hormone does not change the disease process, cannot prevent recurrence. The disease in adults (including the elderly) clinical course differs from the typical children acute onset of idiopathic nephrotic syndrome, has the following characteristics: (1) onset is given priority to with pe attack type; (2) always microscopic haematuria and high blood pressure; (3) high incidence of kidney damage, recovery was slow; (4) slow for prednisone treatment response, but the recurrence rate is low. Nephrotic syndrome is minimal change disease pediatric patients with good treatment response to glucocorticoids (efficiency more than 90%) and fast (2 weeks or so); But slower in adult patients (6 to 20 weeks), about 80% efficient: corticosteroids: loose dragon has much used prednisone or strong. The latter than the former is expensive, and liver function is normal, the former into the latter, the former can be used commonly so. Usage and dosage is not very consistent, taking some points in time, some time, some day. Domestic commonly used method for starting dose 40-60 mg/d, points taken 3-4 times or in the early morning of a suit, maintain 8 and 12 weeks. Effective (in a week or so of diuresis, first two weeks of urine protein gradually reduce, or even disappear) gradually decrease medicine, about every 2 to 3 weeks to reduce 5-10% of the original dose, to the daily amount of 10-15 mg, can be changed to day meal served a total (the 2nd day morning taking), hormone reduced to the minimum effective volume, maintain a 6 to 12 months, this is critical to the success of the treatment began dosage should be enough, large dose of drug induced time to fully, effective drugs to slow. According to this classic therapy, most patients can obtain cure curative effect, even with proximal convoluted tubule function returned to normal after treatment. A main difficulties in the treatment of these diseases is high recurrence rate. Cytotoxic immune inhibitors: the drug treatment of nephrotic syndrome curative effect than glucocorticoid alone. But for the hormone dependent and steroid resistance type and combination treatment with glucocorticoid, can have a supporting role, commonly used drugs with cyclophosphamide, nitrogen mustard, benzyl butyrate nitrogen mustard, specific usage can be found in the book 59-73. Other cytotoxic drugs such as azathioprine, consult for pp, vincristine, etc, but not sure curative effect, side effect is larger. Ring spore, A (CyA) : the drug treatment of kidney disease in recent years, although in children or adults hormone dependent and curative effect of hormone resistant frequently reported in recent years, but there are also negative reports, especially this drug nephrotoxicity caused interstitial nephritis (), recurrence after the drug was stopped, and expensive drugs made the use of these drugs has great limitations. This disease is easy to ease and repeated recurrence, the attacks about half can alleviate commonly, after a few years to remission rate is reduced, recurrence rate is lower than adult children. Sensitive to hormone therapy of children with nephrotic syndrome, 25 years of follow-up, < 5% for the development of end-stage renal disease (ESRD), so that good prognosis. Frequent recurrence or never ease and secondary invalid prognosis is poorer, appear in renal biopsy increased glomerular volume was later developed into the signs of ESRD and hormones is invalid, higher serum IgE often heavy doses of hormones is likely to get relief.

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