Section IV Nephrotic syndrome [Diagnosis] I. Clinical manifestations There is often a precursor infection, mostly in the upper respiratory infection or after overwork; generalized or local edema of varying degrees, initially seen in the ankles, depressed, face puffy in the morning, and gradually spread to the whole body; in severe cases, there are thoracic and abdominal effusions or pericardial effusion; patients with severe blood volume insufficiency may have postural hypotension, such as cold extremities, poor venous filling, dizziness, etc. Patients over 50 years old with large amount of proteinuria are easy to combine with idiopathic acute renal failure, which is characterized by sudden onset of oliguria, anuria and rapid deterioration of renal function. Wei Lianbo, Department of Nephrology, Zhujiang Hospital, Southern Medical University (SMMU) (2) Examination (a) Urine routine examination: Qualitative urine protein +++～++++, 24h urine protein quantitative ≥3.5g is mostly non-selective; some patients have hematuria in combination. (b) Blood biochemical examination, total plasma protein is reduced, serum albumin <3.0g/L; (c) Lipid examination, blood cholesterol and triglyceride are elevated, and some patients do not have elevated blood lipids; (d) Patients often have a decrease in the level of blood IgG and complement; (e) Special examination, renal tissue biopsy is a necessary means of obtaining a correct pathological diagnosis and guiding the treatment, and according to the pathological characteristics of the renal biopsy, primary nephrotic syndrome is divided into the following types According to the pathological characteristics of renal biopsy, primary nephrotic syndrome is mainly classified into the following types: 1. micropathic nephropathy: the glomeruli are basically normal under light microscopy; there is no deposition of immunoglobulin or complement under immunofluorescence; and fusion of glomerular epithelial cell pedicles can be seen under electron microscope. 2. Proliferative glomerulonephritis is mainly characterized by diffuse glomerular mesangial cell proliferation and different degrees of mesangial stroma increase. There is no abnormality of glomerular capillary wall and basement membrane. According to its immunopathology, it can be divided into two types: IgA nephropathy (mainly IgA deposition) and non-IgA nephropathy. 3. Focal segmental glomerulosclerosis is mainly characterized by focal lesions in some glomeruli and segmental damage in damaged glomeruli, with glass-like substances characteristically deposited under the endothelial cells of damaged capillary collaterals. Renal tubules often have lesions, manifested by focal thickening of the basement membrane and tubular atrophy. 4. Membranous nephropathy Its main pathological feature is the diffuse deposition of immune complexes under the epithelial cells of the glomerular basement membrane with diffuse thickening of the basement membrane. Generally, there is no proliferation of mesangial, endothelial and epithelial cells. (5) Tethered capillary nephritis The main lesions are glomerular basement membrane and tethered membrane area, manifested as glomerular basement membrane thickening, tethered membrane cell hyperplasia and tethered membrane stroma dilatation. Some patients have persistent hypocomplementemia. Diagnostic criteria (a) massive proteinuria; (b) hypoproteinemia; (c) edema; (d) hyperlipidemia; of which (a) and (b) are required. To establish the diagnosis of primary nephrotic syndrome, secondary nephrotic syndrome must first be excluded. [Treatment] I. Chinese medicine treatment 1. Spleen and kidney yang deficiency type: white face, edema, or accompanied by ascites, pleural fluid, urination is unfavorable or short, cold limbs and cold, poor nutrition and loose stools. The tongue is pale and chubby, the moss is white and greasy or thin white, and the pulse is thin. It is mostly seen in 1 week before hormone treatment or in the stage of not using hormone. Treatment: warming the spleen and kidney, inducing diuresis and eliminating swelling. Prescription: Zhenwu Tang plus reduction. 10g, hyssop 10g, cinnamon 3g, zedoary 6g, dandelion 10g, if accompanied by little gas and laziness, shortness of breath and fatigue, add ginseng 12g, astragalus 30g, if proteinuria is more than the addition of cuttlebone 10g, 15g of gold cherry. 2. Yin deficiency and hyperthermia type: mild swelling, excitement, insomnia and night sweating, redness on cheekbones, hirsutism, acne, heartburn and heat, dry mouth and pharynx, red tongue with little fluids and fine pulse. It is mostly seen in the hormone initial treatment stage of renal syndrome. Treatment: Nourish Yin and reduce fire. Formula: Er Zhi Wan combined with Da Tonic Yin Pill. Chasteberry 12g, Drynaria 15g, Radix Rehmanniae Praeparata 24g, Radix Dampness 12g, Rhizoma Dioscoreae 15g, Rhizoma Zhi Mu 30g, Tortoise Plate (Decoction) 30g, Radix et Rhizoma Ginseng 15g. Add: For oedema, add Radix et Rhizoma Ginseng 30g, Rhizoma Donggua Pi 12g, Poria 15g, for dampness-heat, add Cortex Phellodendron Bidentatae 6g, Gardenia jasminoides 9g, Gentiana Longdianensis 12g, Reedum Vulgaris 9g, Fructus Qiangcao 12g, Cortex Pseudostellariae 6g, for deficiency of Yin, add Erythrina 9g, heat toxicity, add Erythrina 9g. The heat poison person adds two flowers 9g, the plate blue root 10 g, the yellow cypress 6g and so on. 3. Yin and Yang Deficiency: The symptoms include: dropsy that does not go away, recurrent episodes of aggravation, unfavorable urination, lumbago, weakness of legs, dizziness, tinnitus, dry mouth and pharynx, heartburn and heat, lack of warmth in the limbs, whiteness of complexion, insomnia, night sweating, dreaming of spermatoglyphics, pale tongue with white moss, and thin or delayed pulse. It is mostly seen in kidney syndrome that is prolonged and intractable. Treatment: Yin and Yang double tonic. Formula: Jisheng Kidney Qi Jiu or Dihuang Drink with subtractions. 15g of ripened earth, yam 30g, cornelian cherry 10g, poria 30g, ze lagarde 10g, danshi 15g, 6g of epimedium, cinnamon (h) 6g, psyllium (another package) 30g, hyssop 15g, maitake 12g. Addition and subtraction: yin deficiency can be removed from epimedium, cinnamon, plus 12g of wolfberries. 4. Spleen and kidney qi deficiency: yellowish color, edema, edema, or oedema or the original high degree of oedema already! Diuresis, and the edema is reduced, less breath and lazy speech, less food and loose stools, waist and knees, short urine, tongue pale, fat and tender or edge of the teeth marks, moss white greasy or white slippery, pulse is thin and feeble. This type is mostly seen in the maintenance therapy stage of hormone and used in the consolidation of the efficacy of frequent recurrent renal syndrome. Treatment: tonifying the spleen and kidney, inducing diuresis and removing dampness. Prescription: Ginseng Ling Bai Zhu San combined with Right Angelica Pill. Radix Astragali 30~50g, Radix et Rhizoma Ginseng 20g, Rhizoma Atractylodis Macrocephalae 20g, Poria 20g, Semen Coix lacryma 10g, Chinese yam 20g, Cortex Eucommiae 15g, Cornu Cervi Pantotrichum 10g, Lycium barbarum 12g, Semen Cuscutae 12g. Addition and subtraction: for those who have more urinary protein, add cuttlebone 10g, golden cherry seed 15g; for those who have low serum protein, and for those who have unresolved edema, add deer antler gelatin 10g, Zihechuan 10g. 5. Liver and kidney yin deficiency type. Lower limb edema, waist and knee pain and weakness, dizziness, tinnitus, upset and sleeplessness, dry mouth and throat, short and astringent urination, constipation, red or reddish tongue, thin white or yellow moss, and thin pulse. Most often seen in the maintenance phase of hormone therapy, adults often recurrent renal syndrome. TREATMENT: Nourish the liver and kidneys. Six-flavored Dihuang Pill plus subtractions. Radix Rehmanniae Praeparata 24g, Cornu Cervi Pantotrichum 12g, Rhizoma Dioscoreae 15g, Ze Xie 9g, Dan Pi 9g, Poria Cocos 35g, Fructus Gorgonii 30g. Addition and subtraction: If kidney yang is deficient, add tonic fat 9g, Cistanchia Cistanches 9g, Semen Cuscutae 9g, if edema is heavy, add Poria Cervi Pantotrichum 12g, Dong Gua Pi 9g. 6. Agreement formula: proteinuria formula for those with proteinuria as a main manifestation of TCM deficiency of qi and blood stasis. 7. For proteinuria as the main manifestation of qi deficiency and blood stasis in Chinese medicine, use Kidney Health Pill 6g, orally, 3 times a day. 8. Torch flower root tablets 5 tablets, oral, 3 times a day or Lei Gongteng polyglucoside tablets 20mg, oral, 3 times a day. Western medical treatment 1, diet and rest Nephrotic syndrome patients should pay attention to rest, reduce external contact and prevent infection. Appropriate activities are necessary to prevent venous thrombosis in stabilized patients. Those with obvious edema should limit the intake of sodium and water. People with good renal function do not need to limit protein intake, but patients with nephrotic syndrome intake of high protein diet will lead to an increase in proteinuria, aggravate glomerular damage, so most scholars do not advocate a high protein diet. 2.Diuresis General patients in the use of hormones, after restricting the intake of water, salt can achieve diuresis. For edema is obvious, after limiting sodium, water still can not reduce swelling can be appropriate choice of diuretics. Diuretics can be divided into: (1). Osmotic diuretics Mannitol, low molecular dextrose, hypertonic glucose and so on. (2). Diuretics with collaterals Fuchsimide (tachyphylaxis, 20-100mg/d, oral or intravenous, in severe cases, 100-400mg intravenous drip), Bumene (butyluretamine, 1-5mg/d); (3). Thiazide diuretics dihydrochlorothiazide (75 ~ l00mg / d); (4). Antialdosterone diuretics Antibiotics (20-120mg / d) and amphotericin (150-300mg / d), the effect of this type of drug alone is not good, and thiazides can be used in combination to enhance the diuretic effect, and to reduce electrolyte disorders; (5). Albumin is mostly used in patients with hypovolemia or diuretic resistance. Because intravenous use of albumin can increase glomerular filtration and tubular epithelial cell damage, most scholars now believe that non-essential should not be used. 3, hormones and cytotoxic drugs Glucocorticoids and cytotoxic drugs are still the main drugs in the treatment of nephrotic syndrome. (1). Glucocorticoid hormones, the use of hormones for the principle of the dose to be sufficient (1.0mg/kg body weight per day), the course of treatment to be long enough (6-8 weeks), slow reduction (every 1-2 weeks to reduce 10%). Currently, the commonly used hormones are prednisone, prednisolone and methylprednisolone. Dexamethasone is now less commonly used due to its long half-life and side effects. (2). Alkylating agents are mainly used for "hormone-dependent" or "hormone-ineffective", synergistic hormone therapy. The drugs available for clinical use are: cyclophosphamide, nitrogen mustard and nitrogen mustard phenylbutyrate. Cyclophosphamide is mostly used in the clinic, and its dosage is 100~200mg per day orally in divided doses or 200mg intravenously every other day, with the total amount not exceeding 150mg/kg body weight. Blood routine and liver function should be regularly observed during use. (3). Cyclosporin A can be used in hormone-insensitive or hormone-dependent nephrotic syndrome patients, the initial dose of 3 ~ 5mg / kg per day, and then adjusted according to the blood cyclosporin A concentration. The general course of treatment is 3~6 months. Long-term use has hepatic and renal toxicity. (4). Mycophenolate mofetil (Mycophenolateroofell MMF) MMF is a new type of effective immunosuppressant, mainly inhibiting the proliferation of T and B lymphocytes. It can be used in hormone resistance nephrotic syndrome, the recommended dose is 1.5~2.0g/d, and its exact clinical effect needs to be confirmed by more clinical data. 4, lipid-lowering treatment Hyperlipidemia can accelerate the development of glomerular diseases and increase the incidence of cardiac and cerebral hemorrhagic diseases, therefore, it should be actively treated. Commonly used drugs include ① 3-hydroxy-3-methylglutaryl monoacyl coenzyme A (HMCCCoA) reductase inhibitors: lovastatin (lavastatin, 20~60mg/d), simvastatin (simvastatin, 20~40mg/d). Simvastatin (simvastatin, 20~40mg/d). The course of treatment is 6-12 weeks. ② Fibric acid drugs (fibricacid): fenofibrate (fenifibrate, 100mg / times, 3 times a day), gemifibrozil (gemifibrozil, 30 ~ 60mg / times, 2 times a day) and so on. (iii) probucol (probucol, 0.5/times, 2 times daily). 5, anticoagulation therapy Some scholars suggest that anticoagulants should be routinely used when plasma albumin is less than 20g/L. As to whether patients with nephrotic syndrome need long-term use of anticoagulants need more clinical data to confirm. [Clinical symptoms disappear, hematuria and proteinuria are negative, and renal function is normal. Clinical symptoms have basically disappeared, proteinuria is within 3g or reduced by more than 50%, and renal function is normal or mildly abnormal. Clinical symptoms are still obvious, hematuria and proteinuria persist, urine protein quantification is still >3.5g, renal function is normal or abnormal.