I: What is meant by nephrotic syndrome (NS)? First of all, we should understand the concept that the so-called syndrome is not a simple disease, but a pathological syndrome composed of the patient’s primary sensation (symptoms), physical examination abnormalities (signs), and ancillary tests (laboratory tests, electrocardiogram, X-ray films, etc.). Nephrotic syndrome is a group of clinical syndromes caused by various causes including chronic nephritis, with clinical manifestations of massive proteinuria, severe edema, hypoproteinemia and hyperlipidemia. Nephrotic syndrome is a group of clinical syndromes with certain intrinsic connections due to pathological damage of the kidney. Therefore, it is defined by clinical manifestations, including: (i) massive proteinuria (>3.5g/d); (ii) hypoalbuminemia (serum albumin <30g/L, children <25g/L); (iii) hyperlipidemia (serum cholesterol >6.5mmol/L); and (iv) edema. Among them, ① and ② are the necessary conditions for diagnosis. In addition, it should be noted that since nephrotic syndrome is a group of syndromes caused by various etiological, pathological and clinical diseases, it cannot be used as the final diagnosis of the disease, just like the terms “fever” and “anemia”. A follow-up kidney biopsy and other relevant tests are needed to make a definite diagnosis. 2: How does nephrotic syndrome develop? The kidneys are the purifier of blood. The metabolic substances and other impurities in human blood need to be removed through the glomerular filtration membrane and finally eliminated from the body through urine. This membrane acts as a barrier for protein filtration. In nephrotic syndrome, this barrier is impaired and protein filtration increases, resulting in proteinuria. The large loss of protein in the urine decreases plasma protein and decreases the colloid osmotic pressure of the blood, changing the balance of fluid exchange between the capillaries and the tissues, and water is retained in the interstitial spaces of the tissues to form edema. Due to the decrease of effective blood volume, the secretion of renin, angiotensin and aldosterone system increases, causing water and sodium retention, and the decrease of glomerular filtration rate due to the decrease of renal blood flow also contributes to edema. The persistent high proteinuria reduces the concentration of plasma protein, especially albumin, and the ratio of albumin to globulin may be inverted. Patients are often malnourished and generally have a negative nitrogen balance. The mechanism of lipid metabolism disorder in nephrotic syndrome is not well understood, but is mostly thought to be related to the long-term loss of protein from the kidneys, which promotes the synthesis of albumin in the liver and stimulates the production of lipoproteins. The clinical manifestations of children with nephrotic syndrome are: A. Generalized swelling: almost all of them have different degrees of swelling, which is most obvious in the face, lower limbs and scrotum. The swelling may persist for weeks or months, or it may disappear at times throughout the course of the disease. After infection (especially streptococcal infection), swelling often recurs or worsens, and azotemia may even occur. B. Gastrointestinal symptoms: Due to edema of the gastrointestinal tract, there are often symptoms of gastrointestinal dysfunction, such as not thinking about eating and drinking, nausea, vomiting and abdominal distension. These symptoms are aggravated when azotemia is present. C. Hypertension: It is not an important clinical manifestation of nephrotic syndrome, but there is water and sodium retention, increased blood volume, and transient hypertension may occur. Type II primary nephrotic syndrome may be associated with hypertension. D. Proteinuria: A large amount of proteinuria is the most important condition for the diagnosis of this syndrome. E. Hypoproteinemia: mainly a decrease in plasma protein, the degree of which is significantly related to the degree of proteinuria. F. Hyperlipidemia: Significant increase of triglycerides in the blood. Nephrotic syndrome examination A. Urine routine: In addition to large amount of protein in the urine, there may be clear tubular type or granular tubular type, and sometimes fat tubular type. Type II: centrifugal urine red blood cells <10/HP; Type II >10/HP. B. Selective proteinuria and urinary C3 and FDP measurement: Type I is selective proteinuria, urinary C3 and FDP values are normal, Type II is non-selective proteinuria, urinary C3 and FDP values are often more than normal. C. Blood biochemical examination: In addition to the decrease of total plasma protein, white/sphere may be inverted, and blood cholesterol is increased in type I, but not in type II. D. Kidney biopsy: Using the kidney puncture technique, a very small amount of kidney tissue is removed and observed by ultrastructure and immunopathology to provide a histomorphological basis. Depending on the child’s condition, the doctor may do blood sedimentation, protein electrophoresis, and renal function tests to guide the diagnosis and treatment. In clinical work, physicians make the diagnosis mainly based on the typical manifestations of the disease, i.e., massive proteinuria (>3.5g/1.73m2 body surface area per day), hypoalbuminemia (plasma albumin <30g/L), and edema with or without hyperlipidemia. Since hypoalbuminemia, hyperlipidemia and edema are largely a consequence of massive proteinuria, it is considered that the criteria for diagnosis should be based on massive proteinuria. IV: How do doctors usually treat them? A combination of Chinese and Western medicine treatment based on adrenocorticotropic hormone is used. Treatment includes controlling edema, maintaining water-electrolyte balance, supplying proper nutrition, preventing and controlling concomitant infections, and using adrenocorticotropic hormones correctly. For those who have recurrent attacks or are resistant to hormones, immunosuppressive drugs should be used. The main treatment of Chinese medicine is to strengthen the spleen, tonify the kidney and control the side effects of western medicine. 1. General treatment (1) Rest and living system: Except for those with high edema and complicated infection, absolute bed rest is generally not required. The amount of activity is gradually increased after the disease is in remission. After 3-6 months of remission, you can gradually participate in study, but it is advisable to avoid overwork. (2) Diet: low-salt diet. Severe edema and high blood pressure have to avoid salt. Children with high edema and/or oliguria should limit the amount of water appropriately, but in case of massive diuresis or loss of salt from diarrhea or vomiting, salt and water must be supplemented appropriately. 2. Symptomatic treatment Generally, most children start diuresis and swelling reduction within 7 to 14 days after the application of hormones. Adrenocorticotropic hormone (hereinafter referred to as hormone) treatment Despite some side effects and the unresolved recurrence problem, clinical practice proves that hormone is still an effective drug that can induce protein disappearance, and is the first choice for the treatment of nephropathy. The principles of drug use are: ① The choice of drug is preferable to medium-acting preparations with a biological half-life of 12-36 hours, such as o-nisone, which can induce remission more quickly and is also suitable for alternate-day therapy in remission. ②The treatment should be started in sufficient quantity and taken in divided doses to induce negative urine protein quickly at heart. ③The maintenance treatment phase after the negative urine protein turn is appropriate to be taken every other morning. ④Maintenance treatment should not be too short, and should be discontinued when the condition is stable to reduce recurrence, and it is also easy to make the urine protein remit when it appears repeatedly. (1) Initial treatment program: there are two types: ① medium and long course therapy: more commonly used in China. o nisone 2mg/dk daily (total not more than 60mg), divided into 3 oral doses. If the urine protein turns negative within 4 weeks, the dose will be reduced starting from at least 2 weeks after turning negative and changed to spaced negative, then the dose will be reduced starting from at least 2 weeks after turning negative and changed to 2mg/kg morning dose on spaced negative day, followed by 4 weeks, after which the dose will be reduced by 2.5~5mg every 2~4 weeks until stopping, for a course of 6 months (medium course therapy). If the urine protein does not turn negative within 4 weeks of treatment, the dose can be continued until 2 weeks after it turns negative, generally for 8 weeks, the longest period is not more than 12 weeks, then change to 2mg/kg every other day, followed by 4 weeks, and reduce the dose in the same way as above, the course of treatment is 9-12 months (long course therapy). (2) Short-course therapy: This method is mostly used in Europe and the United States and other countries, but is less commonly used in China. (2) Relapsed cases: extend the duration of alternate day dosing, i.e., give medium to long course therapy. Additional immunosuppressant can be considered for more than 2 relapses. (3) For hormone-dependent cases: the alternate-day dose that can maintain remission must be mapped with reference to the medication and repeated history, maintained for a long time, at least six months, and then tried to reduce the dose later. It is generally believed that o nisone 1.4mg/kg every other day, no hormonal mold side effects. Also stay add immunosuppressant. (4) For hormone resistant people: the director of the alternate day dosing time and / or the addition of immunosuppressive drugs, can make some cases to achieve remission or partial remission, and may delay the process of renal decompensation. These are mostly non-microscopic lesions, so it is best to perform renal puncture to clarify their pathological type and decide on a treatment plan (5) methylprednisolone intravenous shock therapy: high-dose intravenous administration has a more potent immunosuppressive and anti-inflammatory effect and can induce a faster negative urine protein. Although this method can be used for primary treatment, but our country is mostly used for refractory nephropathy, that is, for hormone resistance or need larger dose maintenance, and hormone side effects are obvious. The dose is 15-30mg/kg (no more than 1000mg in total), diluted with 5-10% glucose 100-200ml and then given quietly for 1 to 2 hours. Daily or every other day, 3 times as a course of treatment, repeat after 1 week if necessary. The shock is followed by hormones given orally every other day at 48 hours after the shock. The side effects are occasional facial redness and tremor, nausea, and taste changes in the IV, and may also cause transient hypercoagulable state and hypertension, cardiac rhythm disturbance, and bleeding from peptic ulcers. 4. Immunosuppressive therapy Indications are: refractory nephropathy and / or hormonal side effects are serious, can be added or replaced with immunosuppressive drugs. (1)Cyclophosphamide: it can reduce relapse and prolong remission; hormone partial sensitivity can be induced to complete remission after addition; hormone resistant patients can sometimes improve the effect of hormone in children after application. (2) Phenylbutyric acid nitrogen mustard: can reduce the relapse of hormone-sensitive patients. (3) In addition, there are cyclosporine A and tretinoin, etc., which have positive efficacy. 5. Other treatment (1) application of anticoagulants: nephrotic syndrome is often in a hypercoagulable state, so in recent years it has been advocated that anticoagulation or anti-platelet aggregation agents should be added, such as heparin, pansentin, blood circulation and blood stasis Chinese medicine Dan Shen, etc. (2) Levamisole: It is an immunomodulatory agent. It is generally used for adjuvant treatment of hormone, especially for diligent relapse or hormone-dependent cases that are often accompanied by infection. (3) Mercaptopropionic acid: It is an angiotensin II converting enzyme inhibitor. In recent years, it is believed that it can improve glomerular hemodynamic status and reduce urinary protein excretion, and can be used for hormone adjuvant therapy, especially for those with hypertension. 6. Chinese medicine treatment The combination of traditional Chinese and Western medicine in the treatment of nephrotic syndrome has obvious advantages such as good efficacy, few side effects and low cost. We will discuss this in further detail later. V: What are the complications of this disease? 1. Infection is the most common complication and the main cause of death. Infections are also often the cause and precursor of recurrent and/or exacerbated disease and can affect the efficacy of hormones. Bacterial infections commonly include respiratory infections, urinary tract infections, cutaneous tannins, and primary peritonitis. Prophylactic administration of antibiotics is generally not advocated because the effect is not reliable and it is easy to cause the proliferation of drug-resistant strains and dysbiosis; however, once the infection occurs, it should be treated promptly and actively. Children are also more sensitive to viral infections, especially in the process of receiving corticosteroids and immunosuppressants, and the disease is often more severe than in children with chickenpox, measles and herpes zoster. 2. Hypercoagulable state and thromboembolic complications The disorder of coagulation and fibrinolytic system in kidney disease can lead to hypercoagulable state and thromboembolic complications, among which renal vein thrombosis is the most clinically important. Acute cases are characterized by sudden onset of hematuria and abdominal pain. In chronic cases, the clinical symptoms of renal vein thrombosis are not obvious, often only the edema is aggravated, proteinuria is not relieved need to pay close attention, if there are similar manifestations, promptly go to the hospital for follow-up. 3. Calcium and vitamin D metabolism disorders In nephropathy, vitamin D binding protein in the blood is lost from the urine, the body vitamin D deficiency, affecting intestinal calcium absorption, and feedback leads to hyperparathyroidism. Clinical manifestations include hypocalcemia, circulating vitamin D insufficiency, and poor bone calcification. These changes are particularly prominent in the growing pediatric population. Hypovolemia is often associated with hypovolemia due to low plasma albumin and reduced plasma colloid osmotic pressure. In addition, some children are chronically and inappropriately salt-abstinent, so that symptoms of hypovolemia, such as postural hypovolemia, prerenal azotemia, and even shock, can occur in varying degrees when there is a more rapid loss of body fluids (e.g., vomiting, diarrhea, high-dose diuretic application, massive ascites release, etc.). 5. Acute hyperalgesia Temporary mild azotemia is not uncommon in the acute onset of this syndrome. Acute hyperalgesia may occasionally occur during the course of the disease. 6. Renal tubular dysfunction may be manifested as diabetes, amino acid urine, loss of potassium and phosphorus in the urine, and insufficient concentration function. 7. Atherosclerosis Occasionally occurs in children with persistent hyperlipidemia. When the coronary arteries are involved, there may be chest tightness, angina pectoris, electrocardiogram changes, and even sudden death. 8. Occasional neurological symptoms such as headache, convulsions, visual impairment may occur in children, which may be caused by hypertensive encephalopathy, cerebral edema, dilutional hyponatremia, hypocalcemia, hypomagnesemia and other causes. 6: How to view the recurrence of nephrotic syndrome? Recurrent attacks are the biggest problem encountered by children with nephrotic syndrome. How to ensure the disease does not recur? For nephrotic syndrome, if you can keep the disease from recurring for a long time, in a certain sense, the disease is cured. Patients must try to avoid cold and do not catch a cold. In addition, emotions are also particularly important for nephrotic patients. If a patient has a relapse of the disease, there is no doubt that it is either a cold or bad emotions, tension, anger, work stress, etc. Hypertension is a major complication of nephrotic syndrome, so it is important to monitor blood pressure and low salt diet. The body lies in exercise, and the kidneys also lie in exercise, and only by insisting on exercise can we really benefit the recovery of the disease. As long as your body allows, you should do what you can, your life should be regular, and you should not stay up late. Compared to adults, children with nephrotic syndrome are more likely to relapse. This may be because children do not pay attention to health care and cannot discipline themselves well. But there is no need to worry too much, the prognosis for many children is good, as they grow older, adolescence comes, the body's immunity gradually strengthens, the chances of the disease recurring are less and less, and the body will get better. When children are vaccinated, parents must explain the child's physical condition to the vaccinating doctor because clinically there are children who experience disease recurrence after vaccination. 7: What should I pay attention to in daily care? Bed rest should be the main concern in case of nephrotic syndrome. Bed rest can increase renal blood flow, facilitate diuresis, and reduce contact with the outside world to prevent cross-infection. However, moderate bed and bedside activities should be maintained to prevent vascular thrombosis of the limbs. When the nephrotic syndrome is in remission, activity can be gradually increased, which is beneficial to reduce comorbidities and lower blood lipids, but should try to go to places with fresh air, avoid public places with dirty air, and avoid skin damage during activity to avoid aggravating the disease or changing the evidence by causing infection. If urine protein increases after activity, the activity should be reduced as appropriate. The following points should be noted during the remission period First, children should not be strained. The child's self-restraint ability is poor, from the hospital back home will feel very fresh, easy to play too tired, sleep deprivation, parents should pay special attention to arrange the child's work and rest time, try to get sufficient rest. Second, should not eat more salt food. Diet should pay attention to less salt, for children whose blood pressure has not yet dropped to normal, this is very important. However, meals without salt can affect appetite, so it is advisable to use a low-salt diet. Only after the swelling and hypertension disappears, the normal diet can be improved, but it should also be light and not too salty. Steamed buns and soda crackers also contain sodium and are best not given to children. You can let your child eat some fresh vegetables and fruits to replenish the body with vitamins. Third, children's clothes should not be worn for a long time without changing. Infection is often a trigger for the recurrence of kidney disease. Frequent bathing and changing of clothes to keep the skin clean can prevent skin infections. Fourth, the child should not go to public places. Keep the indoor air fresh and try not to take your child to public places such as stores and theaters. Pay attention to increase and decrease clothes according to climate change to prevent colds. Fifth, should not casually reduce or stop the drug. Most of the treatment of kidney disease requires the use of hormonal drugs. The children who are taking hormones must be under the guidance of the doctor, as their condition improves, gradually reduce the dosage until the drug is discontinued. Parents should supervise their children to take the medication on time and in accordance with the dosage, do not arbitrarily reduce the dosage and stop the medication, so as not to cause recurrence of the disease. 8: What should I pay attention to in terms of diet for children with nephrotic syndrome? The diet for nephrotic syndrome must be tailored to patients with large amounts of proteinuria, edema, hypoproteinemia and hyperlipidemia. (1) Many people think that since there is proteinuria, the body must have lost a certain amount of protein, so it must be supplemented with protein and eat more protein foods. In fact, this is a misconception. If you compare a kidney with proteinuria to a leaky kettle, the more water you pour into it, the more the kettle leaks. The kidney is the same, the more protein you eat, the heavier the burden on the kidney, if you limit the protein intake, it will reduce the burden on the kidney and gradually restore the function of the kidney. Of course, you can also consult your doctor to decide the amount of protein intake according to the specific situation of your child. (2) Patients with different degrees of edema should be given a diet with less salt, no salt or less sodium. When taking high doses of hormones (prednisone), sodium retention can easily cause edema, so salt intake should also be appropriately limited. ①Less salt diet: daily salt intake should not exceed 2~3g (1g of table salt contains 400mg of sodium) and no other salt-containing food. ②No salt diet: no more salt or other salt-containing foods are added to the daily diet during cooking, and sugar and vinegar are usually added to enhance the taste, but the sodium content of the food should not exceed 1000mg. ③Less sodium diet: in addition to no more salt or other salt-containing foods are added to the daily diet during cooking, the sodium content of the food should not exceed 250~500mg. After long-term consumption of less salt diet, patients often prefer to eat Braised food, can be adjusted according to the local soy sauce salt concentration, with soy sauce instead of salt, the appropriate amount. Generally, there is about 1g of salt in 4~5ml of soy sauce. (3) Those with severe swelling should limit water and strictly record the amount of fluid in and out. (4) Patients with severe hyperlipidemia should limit fat intake and adopt a diet with less oil and low cholesterol, but when hyperlipidemia is caused by hypoproteinemia eating a high protein diet to make up for it, lipid intake also increases, so the restriction on lipid foods can be controlled appropriately and need not be too strict. (5) According to the appetite, sufficient calories should be supplied daily to ensure the full utilization of protein (210~252kJ?kg-1, according to the eating habits of our people, it is difficult for the general appetite to reach this calorie). IX: Can nephrotic syndrome be cured? The prognosis of nephrotic syndrome varies greatly depending on the cause, the type of pathology, and whether or not it is treated reasonably. In estimating the prognosis of nephrotic syndrome, the following factors can be used as reference (1) Age: microscopic disease accounts for 80% of primary nephrotic syndrome in children, and 85% of microscopic nephrotic disease in the age group of 2-6 years, which gradually decreases with age. It accounts for only 20% of adults older than 30 years. Because of the good prognosis of this type of nephrotic syndrome, 54% of children with nephrotic syndrome can be in complete remission, while only 21% of adults can be in complete remission. However, in pediatric patients, the younger the age of onset, the higher the mortality rate. (2) Hematuria: Hematuria that is not obvious belongs to nephrotic syndrome type 1, pathologically it is mostly microscopic lesions or mild thylakoid proliferative nephritis, and the prognosis is better. Obvious hematuria belongs to nephrotic syndrome type II, with a poor prognosis. (3) Proteinuria: selective proteinuria indicates a mild lesion and a better prognosis, while non-selective proteinuria has a poor prognosis. If there is severe proteinuria early, renal failure appears earlier and the prognosis is poor. (4) Hypertension and azotemia: common in nephrotic syndrome type II, insensitive to hormone therapy, poor prognosis. (5) Serum cholesterol: elevated serum cholesterol is mostly seen in microscopic lesions, and the elevation in other diseases is low, while in secondary nephrotic syndrome caused by systemic lupus erythematosus, serum cholesterol is normal, so it can be considered that those with elevated serum cholesterol have a better prognosis. However, those with serum cholesterol higher than 20.8 mmol/L in children have a high morbidity and mortality rate. (6) The time to start treatment: early diagnosis and early treatment will result in a relatively good prognosis. The prognosis for adults with nephrotic syndrome is poor if treatment with corticosteroids is started more than 6 months after the onset of the disease. (7) Time of appearance of swelling: Some statistics show that the highest number of end-stage renal failure occurs in adults with nephrotic syndrome in the 3rd-4th year after the first appearance of swelling. (8) Response to hormones: Patients with nephrotic syndrome who respond poorly to corticosteroid therapy at the outset are predicted to have difficult treatment and a poor prognosis. X: What are the advantages of Chinese medicine in treating this disease? The combination of Chinese and Western medicine in the treatment of nephrotic syndrome has shown obvious advantages. It not only improves the clinical efficacy but also greatly reduces the side effects of hormones. Based on the principle of TCM evidence-based treatment, we have developed a three-stage treatment plan for nephrotic syndrome based on the evolution of the evidence and symptoms of patients at different stages of nephrotic syndrome, such as the pre-treatment period, the inter-treatment period and the post-discontinuation period. At the stage of starting high dose hormone use, because hormone is a masculine product and is taken for a long time, medical hyperadrenocorticism may occur. Patients mostly have flushed face, hot and bothered heart, dry mouth and tongue, easy to eat, red tongue with little moss, sunken and fine pulse, and easy to have external sensation. The treatment should be nourishing Yin and lowering fire, using dry lotus herb, raw earth, chasteberry, Chinese wolfberry, tortoise plate, Zhi Mu, and cypress. The combination of Chinese and Western medicines can reduce the adverse effects of large doses of hormones and improve the immunity of the body, thus increasing the remission rate. During the hormone reduction stage, when the hormone is withdrawn to a certain amount, corticosteroid withdrawal syndrome of different degrees may appear, and the patient will gradually develop qi deficiency such as fatigue, lumbar and leg weakness, less breath and lazy speech, and even yang deficiency such as fear of cold extremities and loose stools, which means the symptoms are transformed into qi-yin deficiency or yin-yang deficiency. At this time, while continuing to use nourishing Yin and kidney tonics, we should add Qi and damp kidney tonics such as Cuscuta, Xian Ling Spleen, Lock Yang, Bacopa monniera, and Fructus Cuscuta. When the hormone is reduced to the maintenance amount, the side effects are already smaller, but the disease often relapses at this time due to factors such as external sensation, at this time the patient's Yin deficiency gradually disappears, while the deficiency of the kidney element, the external guard is not solid. The treatment should be based on benefitting the qi and consolidating the kidney, strengthening the spleen and activating the blood. The treatment should be based on benefiting qi, consolidating kidney, strengthening spleen and invigorating blood. The addition of these herbs can improve immunity, enhance healing effect, promote adrenal cortex secretion and reduce hormone withdrawal syndrome, reduce rebound phenomenon and help consolidate healing effect. Daily care stage Patients with nephrotic syndrome have different degrees of qi deficiency and blood stasis, or have poorer constitution and lower resistance due to the use of hormones and cytotoxic drugs. Therefore, patients with nephrotic syndrome should pay attention to rest, long-term supplementation of the spleen and kidneys, the benefit of the body to strengthen the surface, improve the immunity of the body, the surface deficiency is not solid, you can often take Yu Ping Feng San or Fang Wei Huang Qi Tang plus reduction. For kidney qi deficiency, add and subtract from Gui Bai Di Huang Tang; for kidney yang deficiency, add and subtract from Ephedra and Fructus Sinensis Tang. In the later stage of the disease, appropriate activities and exercises should be carried out to harmonize the qi and blood and to facilitate the flow of the meridians. During hormone induction, if the tongue is red, the pulse is stringy, and the face is red and excited, give nourishing Yin and lowering inflammatory drugs, the basic formula is Zhi Mu, Wu Shen, Sheng Di, Dan Pi, Ze Di, Sheng Gan Cao, Huang Bai, and Gentian Cao. The basic formula is Astragalus, roasted licorice, |silk seeds, Wu Wei Zi, Yang deficiency plus bone marrow, Xian Ling Spleen, Yin deficiency plus Radix et Rhizoma Ligustrum, Dry Lotus Grass, and Liu Wei Di Huang Wan. In the process of immunosuppression, blood leukocyte count decreases, and blood tonic can be given, such as yellow essence, angelica, chicken blood vine, motherwort, cynthia, etc. In conclusion, nephrotic syndrome refers to a group of clinical syndromes with certain intrinsic links due to pathological damage of the kidney. We have good reasons to believe that the disease can be prevented and controlled, and that a significant number of children can achieve complete remission.
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