Introduction: what happened to the lovely child?Alice is a 10 year old girl, always lively and lovely, a week ago, Alice suffering from resp
Introduction: what happened to the lovely child?
Alice is a 10 year old girl, always lively and lovely, a week ago, Alice suffering from respiratory tract infection (cold), after treatment, the symptoms have improved significantly, this morning, his family found that red bilateral eyelid slight swelling at the beginning, the family did not care how, can more and swollen eyelids more serious, 3 days after lower extremity edema appeared to concave
The family worried, ask what Alice uncomfortable, just know red urine volume significantly reduced, urine and a lot of foam, hurried to take the children to the hospital, urine routine urine protein (+ + + +), 24 hour protein 7.81g/L, blood lipid total cholesterol after 10.31g/L, glycerin three greases 5.37mmol/L, check, the doctor according to the patient's medical history and clinical manifestations and related examinations, the initial diagnosis of nephrotic syndrome
Question 1: what is nephrotic syndrome (nephrotic, syndrome, NS)
L first of all, we should understand a concept, the so-called syndrome, is not a simple disease, but a feeling by the patient's officers (symptoms), physical examination (abnormal signs), auxiliary examination (test, ECG, X- tablets) composed of pathological syndrome. Nephrotic syndrome is a group of clinical syndromes caused by a variety of reasons, including chronic nephritis, clinical manifestations of a large number of proteinuria, severe edema, hypoproteinemia and hyperlipidemia.
L nephropathy syndrome is a group of clinical syndrome with a certain internal relationship, which is caused by renal pathological damage. Therefore, it is defined as defined by clinical manifestations, including: proteinuria (> 3.5g/d); low albumin (ALB < 30g/L < 25g/L, children); the hyperlipidemia (serum cholesterol, 6.5mmol/L); the edema. Among them, two items are necessary for diagnosis. In addition, it should be pointed out that the nephrotic syndrome is caused by a variety of etiology, pathology and clinical syndrome in a group, so the "fever", "anemia" term, cannot be used as the final diagnosis of disease. The need for follow-up renal biopsy and other related checks for a clear diagnosis.
Question two: how nephrotic syndrome?
The kidney is the purification of the blood, the body's metabolism of blood and other impurities need to be removed through the glomerular filtration membrane, and ultimately excreted by the urine. The filtration membrane acts as a barrier to protein filtration. In nephrotic syndrome, this barrier is impaired and the protein is filtered out, resulting in proteinuria. A large number of loss of protein in the urine to plasma protein decreased, decreased blood colloid osmotic pressure, changes in capillary and tissue fluid exchange balance, edema formation water retention in tissues. Due to the reduction of effective blood volume, promote the secretion of renin, angiotensin, aldosterone system increased, resulting in retention of water and sodium, in addition to reduced renal blood flow to reduce glomerular filtration rate also contributed to the occurrence of edema.
A large number of persistent proteinuria, so that plasma proteins, especially albumin concentration decreased, the proportion of white and globulin inversion. Patients often accompanied by malnutrition, generally negative nitrogen balance.
The mechanism of lipid metabolism disorder in nephrotic syndrome is not clear, most of which are associated with the long-term loss of protein in the kidney to promote the synthesis of albumin in the liver, but also stimulate the formation of lipoprotein.
Question three: how to determine whether a child with nephrotic syndrome
Clinical manifestations of children:
A. systemic edema: almost all appear different degrees of edema, edema in the face, lower limbs, the most obvious scrotum. Edema lasts for several weeks or months, or during the course of the disease. In the infection (especially streptococcal infection), often make the recurrence or aggravation of edema, or even can appear.
B. digestive tract symptoms: gastrointestinal edema, often without thinking about diet, nausea, vomiting, abdominal distension and other symptoms of gastrointestinal dysfunction. The symptoms were aggravated when the patients were treated with endotoxemia.
C.: hypertension important clinical manifestations of non nephrotic syndrome, but there is water and sodium retention and increased blood volume, there may be transient hypertension. Which type of primary renal intercalated syndrome may be associated with hypertension.
D. proteinuria: proteinuria is the most important condition for diagnosis.
E. hypoproteinemia: mainly the decline of plasma protein, the extent of which is significantly related to the degree of proteinuria.
F. hyperlipidemia: the increase of three glycerol in blood.
Nephrotic syndrome examination
A. urine routine: in addition to a large number of proteins in the urine, there may be a transparent tube or granular tube type, and sometimes can also be fat tube type, type II: centrifugal urine red blood cell 10 / HP; type II / HP.
Determination of C3 and FDP in selective proteinuria and urine: type I was selective proteinuria, urine C3 and FDP values were normal, type II was non selective proteinuria, urinary C3 and FDP values were more than normal.
C. blood biochemical examination: in addition to the total plasma protein, white / ball can be inverted, blood cholesterol type I, type II can not increase.
D. renal biopsy: a renal biopsy technique was used to remove a small amount of renal tissue, which was observed by ultrastructural and immunohistochemical methods.
According to the situation of children, may the doctor will do ESR, protein electrophoresis, renal function tests, in order to guide the diagnosis and treatment.
In clinical work, the doctor is mainly based on the typical manifestations of this disease, namely, proteinuria (Daily > 3.5G / 1.73m2 body surface area) and serum albumin (ALB < 30g/L), edema with or without the diagnosis of hyperlipidemia. As a result of hypoproteinemia, hyperlipidemia and edema is largely a consequence of a large number of proteinuria, therefore, the diagnostic criteria should be based on a large number of proteinuria.
Question four: how do doctors generally treat?
Chinese medicine combined with western medicine for the treatment of adrenal cortex hormones. Treatment includes edema control, maintenance of water electrolyte balance, adequate supply of nutrients, prevention and control of concomitant infection, proper use of adrenal cortical hormone. Application of immunosuppressive agents in patients with recurrent or steroid resistance. At present, traditional Chinese medicine is the main treatment of invigorating spleen and kidney, and controlling the side effects of Western medicine.
1 general treatment
(1) rest and living system: in addition to high degree of edema, concurrent infection, generally do not need absolute bed. The activity increased gradually after remission. After 3 to June, we can gradually participate in the study.
(2) diet: low salt diet. Severe edema and high blood pressure to avoid salt. Highly edema and / or oliguria children should be appropriate to limit the amount of water, but a large number of diuretic or diarrhea, vomiting, salt, salt and water should be properly added.
2 general symptomatic treatment with hormone after 7 ~ 14 days, most of the patients started diuretic swelling.
3 adrenal cortical hormone (hereinafter referred to as the hormone) hormone therapy despite some side effect, and has yet to solve the problem of relapse, but the clinical practice proved effective drugs is still the hormone can induce protein disappeared, and as the drug of choice for treatment of kidney disease.
The principle of drug medication; the choice of the biological half-life of 12 ~ 36 hours of preparation in effect is appropriate, such as Fa prednisone, in addition to rapid induction of remission, also applies to relieve the alternate day therapy. The start of treatment should be sufficient, in divided doses, fast heart induced disappearance of urinary protein; the urine protein negative after maintenance therapy in the next day morning meal is appropriate; the maintenance treatment should not be too short, should be a stable condition to stop, in order to reduce the recurrence, and urinary protein is easy to appear repeatedly relieved.
(1) the initial treatment plan: there are two categories: medium and long term therapy: more domestic use. Fa prednisone daily 2mg/dk (total of not more than 60mg), 3 times a day. If within 4 weeks of urinary protein negative, while negative after at least 2 weeks consolidation reduction, instead of every shade, is negative after at least 2 weeks consolidation reduction, instead of every day 2mg/kg Yin morning meal service, followed by 4 weeks, every 2 ~ 4 weeks by 2.5 ~ 5mg, until the withdrawal of treatment 6 months (range therapy). If within 4 week of treatment of urinary protein negative conversion, can be negative after 2 weeks following the service to the general, medication for 8 weeks, the longest no more than 12 weeks, then changed to 2mg/kg the next day, after 4 weeks, the reduction method is the same. The course of treatment was 9 ~ 12 months (Cheng Liaofa). Short term treatment: Europe and the United States and other countries to adopt this method, less domestic use.
(2) recurrent cases: extend the next day medication time is given in the long course of treatment. More than 2 times of relapse can be considered with immunosuppressive agents.
(3) for hormone dependent patients: to reference drug and repeated exploration history, can maintain remission day dose, long-term maintenance, reduction of at least six months. Try again later. That day Fa prednisone 1.4mg/kg, without hormone side effects of mycophenolate. Immunosuppressive agents.
(4): Director of steroid resistance day medication time and / or combined with immunosuppressive agents, can make some cases achieved remission or partial remission, and may delay the process of renal dysfunction. This is a kind of non small lesions, so it is best to carry out renal biopsy, in order to clarify its pathological type, determine the treatment plan
(5) intravenous methylprednisolone methylprednisolone pulse therapy with high-dose intravenous administration of immunosuppressive and anti-inflammatory effects of more powerful, can quickly induce urinary protein negative. Although this method can be used for the initial treatment, it is widely used in our country for the treatment of refractory nephropathy, which is used in the treatment of steroid resistance or the need to maintain a large dose, and obvious side effects of hormones. The dose was 15 ~ 30mg/kg (not more than 100mg of total), with a dose of 5 ~ 10% glucose diluted by 200ml ~ ~ ~ ~ ~ 1 ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ 100 ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~. Daily or every other day for 1 times, 3 times for 1 course of treatment, when necessary and repeated after 1 weeks. After the shock of 48, followed by intermittent oral administration of hormone. The side effects were nausea, tremor, occasionally change red taste intravenous drip, also can cause a transient hypercoagulable state and hypertension, arrhythmia, hemorrhage of digestive tract.
4 immunosuppressive therapy indications: refractory nephropathy and / or severe side effects, can be added or replaced with immunosuppressive agents.
Cyclophosphamide (1): it can reduce the recurrence and prolong remission period, and the hormone sensitive patients can be induced to complete remission after being added.
(2) nitrogen mustard: can reduce the recurrence of hormone sensitive.
In addition, cyclosporin A, tripterygium glycosides, etc..
5 other treatments
(1) the application of anticoagulants: nephrotic syndrome often is in hypercoagulable state, so in recent years, some people advocate should be combined with anticoagulation or antiplatelet agents, such as heparin, Pan Shengding, Chinese herbal medicine Danshen etc..
(2) levamisole: immunomodulator. Often used in the adjuvant treatment of hormones, especially in cases of recurrent or hormone dependent.
(3) captopril: angiotensin converting enzyme inhibitors in recent years, some people think that the state can improve glomerular hemodynamics reduce urine protein, can be used for hormonal adjuvant therapy, especially those with high.
6 traditional Chinese and Western medicine treatment of traditional Chinese and Western medicine treatment of nephrotic syndrome has good curative effect, less side effects, low cost and other obvious advantages. We further discussed in the following.
Question five: what are the complications of this disease?
1 infection is the most common complication and the main cause of death. Infection is often the cause of repeated and / or exacerbation and lead, and can affect the efficacy of hormones.
Bacterial infections are common respiratory infections, urinary tract infections, skin erysipelas and primary peritonitis. Generally do not advocate the use of prophylactic antibiotics, because the effect is not reliable, and easy to cause the proliferation of drug-resistant strains and flora imbalance; but once the infection should be treated in a timely manner.
Children with viral infection is also more sensitive, especially in the process of receiving corticosteroids and immunosuppressive agents, concurrent chickenpox, measles, herpes zoster is often more severe than the average child.
2 the coagulation and fibrinolytic system dysfunction can be caused by coagulation and fibrinolysis system in patients with hypercoagulable state and thromboembolic complications. Acute patients with sudden onset of gross hematuria and abdominal pain. Chronic renal vein thrombosis formation of clinical symptoms was not obvious, often only aggravated edema and proteinuria does not alleviate the need to pay close attention to, such as had similar performance, in time to visit the hospital.
3 calcium and vitamin D metabolic disorders in the blood of vitamin D binding protein is lost in the urine, the body of vitamin D deficiency, the influence of intestinal calcium absorption, and feedback led to hyperparathyroidism. The clinical manifestations of vitamin D deficiency, hypocalcemia, circulating bone hypocalcification. These changes are particularly prominent in growing children.
4 low blood volume due to plasma albumin, low plasma colloid osmotic pressure is reduced, the syndrome often have insufficient blood volume, some children of long-term inappropriate to avoid salt, when there is a sharp loss of body fluids (such as vomiting, diarrhea, diuresis, application of large volume paracentesis etc.) can appear different degree of blood volume the lack of symptoms, such as orthostatic hyponatremia, prerenal azotemia, and even shock.
5 acute renal failure due to the onset of transient mild hypoxemia is not uncommon. During the course of the disease, acute renal failure may occur.
6 renal tubular dysfunction can be manifested as diabetes, amino acid urine, urinary potassium loss of phosphorus, lack of concentration, etc..
7 atherosclerosis can continue to occur in children with hyperlipidemia. Coronary artery involvement may be chest tightness, angina, ECG changes, and even sudden death.
8 children can occur even headaches, convulsions, visual disorder and other neurological symptoms, perhaps by hypertensive encephalopathy, cerebral edema, hyponatremia, hypocalcemia and hypomagnesemia and other causes.
Question six: how to treat the recurrence of nephrotic syndrome?
Recurrent attack is the biggest problem in children with nephrotic syndrome. How to ensure that the disease does not recur? For nephrotic syndrome, if you can keep the disease does not recur for a long time, in a sense, is a cure for the disease. The patient must try to avoid the cold, not cold. In addition, the mood is particularly important for patients with kidney disease, if a patient of disease recurrence, no doubt, not to catch a cold is not good mood, appeared nervous, angry, work pressure etc.. High blood pressure is a major complication of nephrotic syndrome, so it is important to monitor blood pressure and low salt diet. The body is in motion, the kidney is also the movement, only to adhere to exercise, is really conducive to the recovery of the disease.
As long as the body allows, it is necessary to do something within the power of life should be the law, can not stay up all night. Children with nephrotic syndrome are more likely to relapse than adults. This may be because the child does not pay attention to health, can not be a good constraint on their own. But do not have to worry too much, many children's prognosis is good, with the growth of age, the arrival of puberty, the body's immune system gradually increased, fewer and fewer opportunities for recurrence of the disease, the body will be better. Vaccination in children, parents must be vaccinated with the doctor to explain the child's physical condition, because some of the children in the clinical vaccine after the recurrence of the disease.
Question seven: daily care should pay attention to what?
Nephrotic syndrome should be based on bed rest. Bed rest can increase renal blood flow, is conducive to diuresis, and reduce exposure to the outside world to prevent cross infection. However, it is necessary to maintain proper bed and bedside activities in order to prevent the formation of thrombosis. When the remission of nephrotic syndrome can gradually increase the activity, it is helpful to reduce complications, reduce blood fat, but should try to fresh air, to avoid public air pollution, and to avoid skin damage when the activity, in order to avoid infection and aggravating illness or change syndrome. Such as activity after the increase in urinary protein should be appropriate to reduce activity.
Remission period need to pay attention to the following points
First, the child should not be tired. Children's self-restraint ability is poor, from the hospital back home will feel very fresh, easy to play too tired, lack of sleep, parents should pay special attention to arrange the children's work and rest time, as far as possible to get enough rest.
Second, should not eat salty food. Diet should pay attention to less salt, blood pressure has not been reduced to normal children, this is very important. But the food without salt, and will affect the appetite, should use low salt diet. After edema and hypertension disappear, can improve the ordinary diet, but also light, not salty. Bread and soda biscuits also contain sodium, it is best not to give the child to eat. Allows children to eat some fresh vegetables and fruits to supplement the body of vitamins.
Third, children should not wear clothes for a long time. Infection is often the cause of kidney disease. Often take a bath Huanyi, keep the skin clean, can prevent skin infections.
Fourth, children should not go to public places. To keep the indoor air fresh, try not to take the children to the shops, theaters and other public places. Note that according to climate change clothes, prevent colds.
Fifth, it is not appropriate to reduce or stop drug. Treatment of kidney disease, most need to take hormone drugs. Take hormone children, must be under the guidance of a doctor, with the improvement of the condition, gradually reduced to stop drug. Parents should supervise their children the amount of medication on time, must not be reduced and stopped at random, so as to avoid repeated illness.
Question eight: Children's diet should pay attention to what?
The diet of nephrotic syndrome must be characterized by a large number of proteinuria, edema, hypoproteinemia and hyperlipidemia.
(1) a lot of people think that since there is protein, the body must have lost a certain amount of protein, so be sure to add protein, eat more protein food. In fact, this is a misunderstanding. If the appearance of proteinuria in kidney compared to a Water Leakage kettle, the kettle to pour water, drain more powerful. The same is true of the kidney, eat more protein, the heavier the burden on the kidneys, if the restriction of protein intake, it will reduce the burden on the kidneys, and gradually restore the function of the kidney. Of course, you can consult a doctor, according to the specific circumstances of the child to determine protein intake.
(2) patients with different degrees of edema, should be given less salt, no salt or low sodium diet. After taking a large dose of hormone (prednisone) treatment, easy to make sodium retention and cause edema, should also be appropriate to limit salt intake.
Less salt diet: daily diet intake of salt is not more than 2 ~ 3G (1g sodium salt content of 400mg), no longer eat other foods containing salt.
The salt free diet: the daily diet in cooking is no longer salt or other salty food, often with sweet and sour taste to increase, but the sodium content in food should not exceed 1000mg.
Less sodium diet: one day in addition to the diet when cooking is no longer add salt or other salt containing food, but also to calculate the amount of sodium in the food does not exceed 250 ~ 500mg.
Patients with long-term consumption of low salt diet, often eating braised food, according to the local salt concentration of soy sauce with soy sauce, salt, the amount of adjustment. General soy sauce 4 ~ 5ml about 1g of salt.
(3) severe edema should be restricted and strictly record the amount of liquid intake and drainage.
(4) severe hyperlipidemia patients to limit fat intake, should use less oil and low cholesterol diet, but hyperlipidemia caused by hypoproteinemia eating high protein diet supplement, lipid intake also increased, so the fat food restrictions can be properly mastered, not too strict.
(5) in accordance with the appetite, the daily supply of sufficient heat to ensure the full use of protein (~ 252kJ ~ kg-1, according to the Chinese people's eating habits, the general appetite is difficult to achieve this heat).
Question nine: nephrotic syndrome can be cured?
There are great differences in the prognosis of nephrotic syndrome due to different etiology, pathological type and reasonable treatment. The following factors may be used as a reference in estimating the prognosis of the renal complex:
(1) age: in children with primary nephrotic syndrome, minimal change disease accounted for 80%, in the 2 - to 6 year old age group, the minimal change nephropathy reached to 85%, and gradually decreased with age. In adults over 30 years old accounted for only 20%. Because of the good prognosis of nephrotic syndrome, 54% of children with nephrotic syndrome can be completely alleviated, only about 21% of adults can be completely alleviated. But in children, the younger the age of onset, the higher the mortality.
(2) hematuria: hematuria is not obvious in the nephrotic syndrome type 1, pathological lesions are mostly small or mild mesangial proliferative nephritis. Nephrotic syndrome with obvious hematuria, poor prognosis.
(3) proteinuria: selective proteinuria showed a mild lesion, a better prognosis, and a poor prognosis of non selective proteinuria. If the early severe proteinuria, renal failure occurred earlier, poor prognosis.
(4) hypertension and hyperlipidemia: common in nephrotic syndrome type II, not sensitive to hormone therapy, poor prognosis.
(5) serum cholesterol: elevated serum cholesterol in minimal change disease, other diseases increased low, caused by systemic lupus erythematosus secondary nephrotic syndrome, normal serum cholesterol, it can be considered that the increase of serum cholesterol, better prognosis. However, children's serum cholesterol was higher than 20.8mmol / L, the mortality was high.
(6) the beginning of treatment: early diagnosis, early treatment, the prognosis is relatively good. Adult onset of nephrotic syndrome 6 months after the onset of corticosteroids treatment is poor.
(7) the time of edema: statistics show that the incidence of end-stage renal failure in adults with nephrotic syndrome at the end of the first third to 4 years.
(8) response to hormones: Patients with nephrotic syndrome who initially respond to corticosteroid therapy may be difficult to treat and have poor prognosis.
Question ten: what are the advantages of traditional Chinese medicine treatment of this disease?
Integrated traditional Chinese and Western medicine treatment of nephrotic syndrome showed obvious advantages. Not only improve the clinical efficacy and reduce the side effects of hormones, we have based on the principles of TCM, according to hormone therapy, treatment duration and early discontinuation of the late stages in patients with nephrotic syndrome evolution, develop western medicine combined treatment in renal in three stages.
Start a large dose of hormone, because hormone is masculine, taking long time, there may be iatrogenic adrenal hyperfunction, patients with facial flushing, five upset hot, dry mouth, eat easy to hunger, red tongue, less moss, pulse count and prone to external symptoms, according to TCM is Yin Huo Wang, when treatment should choose nourishing Yin, Eclipta, rehmannia, Ligustrum lucidum, medlar, tortoise shell, Anemarrhena, Phellodendron bark. Combination of traditional Chinese and Western medicine can not only reduce the adverse effects of large doses of hormones, but also improve the body's immune system, thereby increasing the remission rate.
In the hormone reduction phase, when hormone withdrawal to a certain amount, can appear different degree of cortical hormone withdrawal syndrome, patients will gradually appear fatigued, yaotuisuanruan, less gas lazy words such as Qi, aversion to cold, and even less satisfied loose stools, Yang and the performance means to the syndrome of deficiency of both qi and yin or yang two virtual transformation. You should continue to use the medicine of nourishing yin and tonifying kidney and Invigorating Qi add wet kidney drugs such as dodder, epimedium, Cynomorium, morinda root, Cistanche deserticola etc..
To maintain the amount of hormone, has small side effects, but the disease often at this time due to exogenous factors such as relapse, the patient showed deficiency gradually disappeared, and kidney deficiency, guard not solid. Treatment should be to Qi of kidney, spleen and blood. Add qihm such as dangshen, Astragalus and salvia, angelica, motherwort and blood circulation of goods. With these traditional Chinese medicine, can improve immunity, enhance efficacy, promote the secretion of adrenal cortex and reduce hormone withdrawal syndrome, can reduce rebound phenomenon and help consolidate the efficacy.
Daily nursing stage
Patients with nephrotic syndrome is the deficiency of the disease for a long time, have different degrees of deficiency of Qi and blood stasis, or due to the use of hormones, cell poison caused by poor physique, low resistance, Wei Qi is not solid, if accidentally Waixie, are more easily repeated, induce or aggravate the disease. The nephrotic syndrome patients should pay attention to rest, long-term spleen, masue Gubiao, improve immunity, the virtual table is not solid, can often take Yuping Fengsan or Fangji Huangqi decoction. Is kidney qi deficiency were treated with Guifu dehuang soup Decoction; of deficiency of kidney were treated with mahuangfuzixixin decoction. Disease later period, it should be appropriate, whatever exercise, to reconcile Qi and blood, meridians tongli.
Hormone induction process such as a red tongue, pulse string, red excited give ziyinjiangwei inflammatory drugs, the basic prescription is no ginseng, Rhizoma Anemarrhenae, rehmanniae, Cortex Moutan, Alisma, licorice root, phellodendron bark, gentian. The tapering process shows such as Qi deficiency, kidney deficiency, kidney qi and medicine, the basic prescription is Astragalus, licorice, Wen silk, Schisandra, Yang and psoralen, epimedium, Ligustrum, Eclipta Yin and other clothing, Rehmannia six. In the process of using immunosuppressive agents decreased white blood cell count, to Qi tonic drugs, such as angelica, Rhizoma Polygonati, Caulis Spatholobi, motherwort, agrimony etc..
In conclusion, nephrotic syndrome is caused by Kidney Pathological Damage of a group of related clinical syndrome in renal biopsy examination to confirm the diagnosis based on the standard use of hormones and immunosuppressive drugs, combined with TCM therapy, nursing and the parents of the daily diet seriously, we have enough reason to believe that this disease is preventable and controllable, a considerable part of patients can achieve complete remission, children's health and happiness is our common wish!