Section IV nephrotic syndrome
I. Clinical manifestations
There are often antecedent infections, mostly after upper respiratory infections or overexertion; generalized or localized edema of varying degrees, mostly in the ankle initially, depressed, facial swelling in the morning, and gradually all over the body; in severe cases, there are pleural and abdominal effusions or pericardial effusions; patients with severe blood volume deficiency may develop postural hypotension, such as cold extremities, poor venous filling, dizziness, etc. Patients over 50 years of age with large amounts of proteinuria are easily combined with idiopathic acute renal failure, which is manifested by sudden onset of oliguria, anuria and rapid deterioration of renal function.
II. Examination
(A) Urine routine examination Qualitative urine protein examination ++++～++++, 24h urine protein quantification ≥3.5g is mostly non-selective; some patients have combined hematuria.
(II) Blood biochemical examination Total plasma protein is reduced, serum albumin <3.0g/L;
(Blood cholesterol and triglycerides are elevated, but some patients do not have elevated blood lipids;
(iv) Patients often have decreased blood IgG and complement levels;
(v) Special tests Kidney tissue biopsy is necessary to obtain the correct pathological diagnosis and guide treatment, according to the pathological characteristics of kidney biopsy, primary nephrotic syndrome is mainly divided into the following types.
1, micro lesional nephropathy The glomerulus is basically normal under light microscopy; there is no deposition of immunoglobulin or complement under immunofluorescence examination; glomerular epithelial cell peduncle fusion is visible under electron microscopy.
2. Thylakoid proliferative glomerulonephritis is characterized by diffuse glomerular thylakoid cell hyperplasia and varying degrees of thylakoid stromal increase. The glomerular capillary wall and basement membrane are not abnormal. According to its immunopathology, it can be divided into two types: IgA nephropathy (predominantly 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 characteristic deposition of glassy material under the endothelial cells of damaged capillary collaterals. The renal tubules are often lesioned, showing focal thickening of the basement membrane and tubular atrophy.
Membranous nephropathy is characterized by diffuse immune complex deposition under the epithelial cells of the glomerular basement membrane with diffuse thickening of the basement membrane. There is generally no proliferation of thylakoid, endothelial and epithelial cells.
The main sites of lesions are the glomerular basement membrane and the thylakoid region, manifesting as glomerular basement membrane thickening, thylakoid cell hyperplasia and thylakoid stromal expansion. Some patients have persistent hypocomplementemia.
Diagnostic criteria
(a) Massive proteinuria;
(ii) Hypoproteinemia;
(c) edema;
(iv) hyperlipidemia;
Among them, items (i) and (ii) are required.
To establish the diagnosis of primary nephrotic syndrome, secondary nephrotic syndrome must be excluded first.
I. Traditional Chinese medicine treatment
1.Spleen and kidney Yang deficiency type
The main symptoms are: white face, edema, ascites, thoracic fluid, unfavorable urination or shortage, cold body and cold limbs, poor appetite and loose stools. The tongue is pale and fat, the coating is white and greasy or thin, and the pulse is sunken and thin. Most often seen in the 1 week before hormone treatment or in the stage of no hormone use.
Treatment: Warming the spleen and kidney, inducing diuresis and eliminating swelling.
Formula: Zhen Wu Tang with addition and subtraction.
Poria 20g, Atractylodes macrocephala 12g, Radix et Rhizoma Atractylodis Macrocephalae 6g, Cao Guo 10g (later down), Dry Ginger 10g, Daggerbelly Bark 15g, Betel nut 15g, Plantago ovata 15g (separately wrapped), Ze Di 12g.
Adding and subtracting: with hand and foot fever, night sweats and other yin and yang deficiency can be more with Jisheng Kidney Qi Pill; 15g of Radix Rehmanniae, 15g of Radix Rehmanniae, 30g of Chinese yam, 15g of Cornu Cervi Pantotrichum, 15g of Poria, 10g of Plantago ovata (separately wrapped), 10g of Radix Achyranthes, 3g of Cinnamon, 6g of Zedoaria, 10g of Dampi. if accompanied by less breath and lazy speech, shortness of breath, add 12g of Radix Codonopsis, 30g of Radix Astragali. 10g, 15g of gold cherry.
2.Yin deficiency and fire type
Main symptoms: mild swelling, excitement and excitement, insomnia and night sweating, redness of the cheekbones, hairiness, acne, irritability of the heart, dry mouth and throat, red tongue with little fluid and fine pulse. Most commonly seen in the first hormonal treatment phase of renal syndrome.
Treatment: Nourishing Yin and lowering fire.
Direction: Er Zhi Wan combined with Da Yin Tonic Pill.
Radix et Rhizoma Maidenhair 12g, Radix et Rhizoma Drynaria 15g, Radix et Rhizoma Dioscorea 24g, Radix et Rhizoma Danpi 12g, Radix et Rhizoma Dioscorea 15g, Radix et Rhizoma Zhi Mu 30g, Radix et Rhizoma Tortoise 30g, Radix et Rhizoma Yuan Shen 15g.
Addition and subtraction: in case of edema, add 30 g of thatch root, 12 g of winter melon skin, 15 g of poria, 6 g of yellow cypress, 9 g of mountain gardenia, 12 g of gentian grass, 9 g of stone reed, 12 g of money grass, 6 g of white flowered snake tongue grass, and in case of yin deficiency and heat toxicity, add 9 g of erhua, 10 g of panax quinquefolium, 6 g of yellow cypress, etc.
3.Yin and Yang deficiency type
Evidence: Delayed swelling, recurrent episodes of aggravation, unfavorable urination, lumbar soreness and weakness, dizziness and tinnitus, dryness of the mouth and throat, feverishness of the five hearts, lack of warmth in the extremities, white complexion, insomnia and night sweats, dreaming and spermatorrhea, pale tongue with white fur and thin or late pulse. Most often seen in renal syndrome with prolonged or refractory renal syndrome.
Treatment: Double tonification of yin and yang.
Remedy: Jisheng Kidney Qi Jiu or Dihuang Drink plus or minus.
Radix Rehmanniae 15g, Rhizoma Dioscoreae 30g, Cornu Cervi Pantotrichum 10g, Poria 30g, Zedoary 10g, Dampi 15g, Radix et Rhizoma Pallidum 6g, Cinnamon (h) 6g, Plantago Asiatica (separately wrapped) 30 g, Radix Achyranthes Bidentatae 15g, Madonnas 12g.
Addition and subtraction: for heavy Yin deficiency, remove Radix et Rhizoma Polygonati, Cinnamon plus Fructus Lycii 12g.
4.Spleen and kidney Qi deficiency type
Main symptoms: withered face, swelling around the body, or edema is light or the original high edema has been diuretic, and the swelling is reduced, less breath and lazy speech, less food and loose stools, waist and knee soreness, short urine, light fat and tender tongue or side with tooth marks, white greasy moss or white smooth, sunken and weak pulse. This type is mostly seen in the maintenance treatment phase of hormone and for consolidating the efficacy of often recurrent renal syndrome.
Treatment: Tonifying the spleen and benefiting the kidney, inducing diuresis and resolving dampness.
Recipe: Ginseng Ling Bai Zhu San combined with Right Return Pill. Astragalus membranaceus 30-50g, Radix Codonopsis pilosulae 20g, Rhizoma Atractylodes Macrocephalae 20g, Semen Coicis 10g, Semen Yam 20g, Cortex Eucommiae 15g, Cornu Cervi Pantotrichum 10g, Fructus Lycii 12g, Semen Cuscutae 12g.
Addition and subtraction: add 10g of mulberry cuttlebone, 15g of gold cherry, 10g of deer antler gum and 10g of purple river carp in case of low serum protein and unremitting edema.
5.Yin deficiency of liver and kidney type
Main symptoms: swelling of the face and lower limbs, soreness and weakness of the waist and knees, dizziness and tinnitus, sleeplessness, dry mouth and throat, short urine and constipation, red or reddish tongue, thin white or yellow fur, thin pulse. Most often seen in the maintenance treatment phase of hormones, and often recurrent renal syndrome in adults.
Treatment: Nourishing the liver and kidney.
Formula: Liu Wei Di Huang Wan with reduction.
Radix Rehmanniae Praeparata 24g, Cornu Cervi Pantotrichum 12g, Yam 15g, Zeligia 9g, Danpi 9g, Fu Ling 35g, Gorgonzola 30g.
Addition and reduction: If kidney yang deficiency, add 9g of bone marrow, 9g of cistanches, 9g of cuscuta, if edema is heavy, add 12g of poria, 9g of dongguo skin.
6.Agreement formula: Proteinuria formula is used for those whose main manifestation is Qi deficiency and blood stasis in TCM.
7. If proteinuria is the main manifestation of Qi deficiency and blood stasis in TCM, use Kidney Health Pill 6g, taken orally 3 times a day.
8. Torch flower root tablets 5 tablets, orally, 3 times a day, or Lei Gong Gong Fu Polysaccharide tablets 20mg, orally, 3 times a day.
II. Western medical treatment
1, diet and rest Patients with nephrotic syndrome should pay attention to rest, reduce outside contact and prevent infection. Proper activity is necessary for stable patients to prevent venous thrombosis.
Those with obvious edema should limit the intake of water and sodium appropriately. However, the intake of high protein diet in patients with nephrotic syndrome will lead to an increase in proteinuria and aggravate glomerular damage, therefore, most scholars do not advocate a high protein diet.
2.Diuretic General patients can achieve diuretic purpose after restricting water and salt intake after using hormone. For patients with obvious edema, diuretics can be used appropriately if the edema cannot be reduced even after sodium and water restriction. Diuretics can be divided into the following according to their site of action.
(1) osmotic diuretics mannitol, low molecular dextrose, hypertonic glucose, etc.
(2) Tab diuretics Fotiamide (tachyphylaxis, 20-100mg/d, orally or intravenously, 100-400mg intravenously in severe cases), Bumetanide (butanuric acid, 1-5mg/d);
(3) Thiazide diuretics dihydrochlorothiazide (75~l00mg/d);
(4) Anti-aldosterone diuretics Ativan (20-120mg/d) and aminoglutethimide (150-300mg/d), these drugs alone are not effective, and combined with thiazide can enhance the diuretic effect and reduce electrolyte disturbances;
(5) Albumin is mostly used in patients with low blood volume or diuretic resistance. Since intravenous use of albumin can increase glomerular filtration and tubular epithelial cell damage, most scholars now believe that it should not be used more than necessary.
Hormones and cytotoxic drugs Glucocorticoids and cytotoxic drugs are still the main drugs for the treatment of nephrotic syndrome.
(1) Glucocorticoids The principle of hormone use is that the dose should be sufficient (1.0mg/kg body weight per day), the course of treatment should be long enough (6~8 weeks), and the reduction of drug should be slow (10% every 1-2 weeks). Currently, the commonly used hormones are prednisone, prednisolone and methylprednisolone. Dexamethasone due to the long half-life, side effects, is now less used.
(2) Alkylating agents are mainly used for “hormone-dependent” or “hormone-ineffective”, in cooperation with hormone therapy. The drugs available for clinical use are: cyclophosphamide, nitrogen mustard and nitrogen mustard phenylbutyrate. Cyclophosphamide is mostly used clinically at a dose of 100-200 mg orally or 200 mg intravenously every other day, with the total amount not exceeding 150 mg/kg body weight. The blood routine and liver function should be observed regularly during the use.
(3) Cyclosporine A can be used for hormone-insensitive or hormone-dependent patients with nephrotic syndrome. The initial dose is 3~5mg/kg per day, and then adjusted according to the blood cyclosporine A concentration. The general course of treatment is 3-6 months. Long-term use has hepatic and renal toxicity.
(4) Mycophenolateroofell MMF MMF is a new effective immunosuppressant, mainly inhibiting the proliferation of T and B lymphocytes. It can be used in hormone resistant nephrotic syndrome, the recommended dose is 1.5-2.0g/d, and its exact clinical effect needs more clinical data to be confirmed.
4.Lipid-lowering treatment Hyperlipidemia can accelerate the development of glomerular diseases and increase the incidence of cardiac and cerebral blood vessels diseases, therefore, it should be actively treated. Commonly used drugs include ①3-hydroxy-3-methylglutaryl monoacyl coenzyme A (HMCCCoA) reductase inhibitors: lovastatin (lavastatin, 20~60mg/d), simvastatin (simvastatin, 20~40mg/d). ~40mg/d). The duration of treatment is 6-12 weeks. ②Fibric acid drugs (fibricacid): fenofibrate (fenifibrate, 100mg/d, 3 times a day), gemifibrozil (gemifibrozil, 30-60mg/d, 2 times a day), etc. ③ Propofol (probucol, 0.5/dose, 2 times daily).
5. Anticoagulation therapy Some scholars suggest that anticoagulants should be used routinely when plasma albumin is lower than 20g/L. As for whether patients with nephrotic syndrome need long-term use of anticoagulants, more clinical data is needed to confirm.
[Efficacy criteria]
I. Cured Clinical symptoms disappear, hematuria and proteinuria are negative, and renal function is normal.
Improvement Clinical symptoms basically disappeared, proteinuria within 3g or reduced by more than 50% on the basis of the original, normal and mildly abnormal renal function.
Third, not healed Clinical symptoms are still obvious, hematuria and proteinuria persist, and urine protein quantification is still >3.5g with normal or abnormal renal function.