Pediatric nephrotic syndrome

Nephrotic syndrome in children is one of the common urinary system diseases in children. According to the statistics of the Chinese Academy of Pediatrics, nephrotic syndrome in children ranks second in the number of hospitalizations. The clinical manifestations of nephrotic syndrome include high edema, hyperlipidemia, large (high) proteinuria, and hypoproteinemia. Edema is an early clinical manifestation of pediatric nephrotic syndrome, which often attracts the attention of parents and clinical workers. In addition to the four major symptoms mentioned above, there are still some cases accompanied by hypertension, hematuria, or azotemia. If there are no symptoms of the three mentioned above, it is called "simple kidney disease". If there is one of the three symptoms, we call it "nephritic kidney disease". There are differences in pathological changes, treatment plans, and prognosis between the two.

Age stage of onset of pediatric nephrotic syndrome

Nephrotic syndrome is more common in children aged 3-6 years old, with more boys than girls. Its etiology is unknown, and it is prone to recurrence and extension, with a long course of disease. Children with nephrotic syndrome can attend kindergarten during the stable period of their condition. As long as the kindergarten strengthens the care of the children, it is conducive to their comprehensive recovery.

Symptoms of pediatric nephrotic syndrome

The most prominent symptom of pediatric nephrotic syndrome is high swelling. Children can have swelling in their lower limbs, head, face, and trunk, especially in areas with loose tissue, such as the eyelids. In boys, the scrotum can become swollen like a light bulb, and there is also fluid accumulation in the visceral serous cavity, such as pleural effusion and ascites. People with severe swelling have thin and transparent skin, and slight skin damage can cause water seepage.

Edema affects blood circulation, reduces local resistance, and is highly susceptible to infection. Urine in nephrotic syndrome contains a large amount of protein, and routine urine examination shows that urine protein can reach+++to++++, with an increase in 24-hour urine protein excretion. Blood tests can reveal a decrease in plasma albumin, causing the normal ratio of white blood to globulin to change from 1-1.5 to 0.5, resulting in an inversion of the ratio and an increase in plasma cholesterol. Some children may have white or purple skin lines similar to those of pregnant women on the inner thighs and upper arms, abdomen, and chest, resulting in a significant decrease in urine output.

Due to the long-term loss of a large amount of protein from urine, protein malnutrition can occur, such as dry and withered hair, keratinized hair follicles, dry skin, white stripes on fingernails, delayed development, anemia, and susceptibility to infection. Some children have hematuria and hypertension.

Nephrotic syndrome has a longer course and is prone to recurrent attacks. The biggest danger is secondary infections, such as skin erysipelas, intestinal infections, pneumonia, primary peritonitis, and sepsis. Any secondary infection can cause death.

Nephrotic syndrome is a clinical comprehensive syndrome characterized by excessive proteinuria, hypoproteinemia, hyperlipidemia, and edema. Protein is mainly caused by damage to inherent kidney cells, ischemia hypoxia necrosis, increased permeability of glomerular filtration membrane, and protein leakage from it.

Treatment of pediatric nephrotic syndrome

At present, the principle of primary kidney disease in children is to strengthen the spleen and kidney, control the side effects of Western medicine, and adopt a comprehensive treatment of traditional Chinese and Western medicine, mainly with adrenal cortical hormones. This includes maintaining a balanced supply of water and electrolytes, controlling edema, providing appropriate nutrition prevention, controlling concomitant infections, cooperating with the use of immunosuppressive drugs such as traditional Chinese medicine for hormone resistant individuals, and correctly using adrenal cortical hormones for recurrent episodes.

Treatment tips for pediatric nephrotic syndrome

1. Corticosteroid therapy

Although hormones have some side effects and have not yet resolved the issue of recurrence, clinical practice has shown that they are still effective drugs that can induce the silent disappearance of proteins and serve as the preferred drug for the treatment of kidney disease. The mechanism of action has not yet been elucidated:

① Diuretic effect.

② Improve the permeability of the glomerular filtration membrane and reduce urinary protein filtration.

③ Immunosuppressive effect.

Medication principles:

① The selection of drugs should be based on a medium acting formulation with a biological half-life of 12-36 hours, such as prednisone, which can not only quickly induce remission but also is suitable for interval therapy At the beginning of treatment, sufficient and divided doses of Xinkuai should be taken to induce negative urinary protein conversion;

③ The maintenance treatment stage after urinary protein negative conversion should be taken overnight, as the secretion of cortisol by the adrenal gland shows a diurnal fluctuation pattern of high morning and low night. The inhibitory effect of the hypothalamus pituitary adrenal axis (HIP) is minimal when taken overnight.

④ Maintenance therapy should not be too short and should wait until the condition stabilizes before discontinuing medication to reduce recurrence and ease urinary protein recurrence

2. Immunosuppressive therapy

① Cyclosporin A: This drug can specifically inhibit the activation and proliferation of helper T cells and cytotoxic T cells without affecting the dosage of B cells and granulocytes. The daily dose of 6-8mg/kg often requires monitoring of blood concentration to adjust the dosage for eight weeks. Its therapeutic effect on nephrotic syndrome can be summarized as a hormone effector. This drug is also effective for such patients. When the side effects of hormone toxicity are significant, this drug can be switched to, but it may still be effective after discontinuing the drug; The toxic and side effects of steroid resistant patients can be alleviated if they are used as soon as possible. The most obvious acute nephrotoxic effect is prenephric azotemia, which is generally reversible change and dose related chronic nephrotoxicity. During the action, there are changes in renal tissue structure, which are interstitial and tubular lesions. There are hypertension, hyperuricemia, sodium retention, hyperkalemia, creatinine clearance rate reduction, nephrotoxicity reduction, hairy gingival hyperplasia, and low blood magnesium.

② Phenylbutyrate nitrogen mustard: can reduce the recurrence rate of hormone sensitive individuals. The commonly used dosage is 0.2mg/kg per day, and the total dose does not exceed 10mg/kg for 6-8 weeks of treatment. The side effects are similar to those of cyclophosphamide, and there are also reports of leukemia and solid tumors.

③ Hydrochloric acid nitrogen mustard: Rapid intravenous infusion or slow intravenous injection every other day: Ten to twenty times is a course of treatment. The first dose of 1-2 mg can gradually increase until 0.1 mg/kg. Side effects include gastrointestinal symptoms, and sedatives can be given before use to prevent local phlebitis. Therefore, larger intravenous administration should be chosen.

④ Cyclophosphamide: can reduce recurrence and prolong the remission period; Partial hormone sensitivity can be induced to complete remission after addition; Hormone resistant patients can sometimes improve their response to hormones at a dose of 2-2.5mg/(kg · d) After three months of treatment, the total dose is 200~250mg/kg for one year. The side effects of repeated use are contraindicated: in the near future, there may be gastrointestinal reactions, liver function damage, hair loss, bone marrow suppression, hemorrhagic cystitis, and increased susceptibility to bacterial viruses. In recent years, attention has been paid to the long-term effect on gonads. After the use of this drug in adolescent or prepubertal boys, it can affect testicular spermatogenesis, causing infertility, gonad damage, and dosage related. Therefore, the indications and dosage should be mastered when using this drug.

⑤ Tripterygium wilfordii glycosides: Extracts from the roots of Tripterygium wilfordii, a plant of the Weimao family, have immunosuppressive effects. The dosage is 1mg/kg, with a maximum daily dose of 30mg, taken three times a day. The course of treatment is 3 months, and the side effects are reduced white blood cells, gastrointestinal reactions, skin pigmentation, and may also affect gonadal function

3. Integrated Traditional Chinese and Western Medicine Therapy

Edema and urinary insufficiency can be treated with Plantago, Lysimachia, Polygonatum, and Cornbeard. For those with symptoms of blood stasis, Zelan Leaf can be added.

For those with insufficient spleen and kidney deficiency and lack of firmness, they should be given a nourishing spleen and kidney gluttony.

If there is redness in the tongue, pulse strings, face, and excitement during hormone induction, give nourishing yin and reducing inflammation gluttony.

If there is qi deficiency and kidney deficiency during the process of hormone reduction, supplementing qi and tonifying the kidney should be added; Yang deficiency plus psoralen fairy spirit spleen yin deficiency plus virginia.

The decrease in white blood cell count during the use of immunosuppressants can provide nourishment for qi and blood.

Complications of pediatric nephrotic syndrome

1. Hypovolemic shock and acute renal failure

① Acute renal failure: Prerenal acute renal failure can be caused by many factors, such as renal interstitial edema or/and renal tubular obstruction, acute renal vein thrombosis, low blood volume formation (RVT), etc.

② Hypovolemic shock: Some sick children have a low blood volume and are in a "fragile" state. Once induced factors such as insufficient intake, infection, vomiting, and diarrhea occur, hypovolemic shock is prone to occur. If a large dose of hormone is taken for a long time, once suddenly stopped, it can manifest as an "adrenal crisis".

2. Hypercoagulability and thromboembolism: When kidney disease patients suddenly experience low back pain (spinal and costal angle tenderness), hematuria, renal dysfunction, and hypertension, RVT should be highly suspected. Thromboembolic complications can also occur in its veins or arteries, with an incidence rate of 8.5% -44%; Such as femoral vein, pulmonary artery, femoral artery, mesenteric artery, cerebral artery, coronary artery, leg thrombophlebitis can also be seen.

3. Infection: Infection is a common cause of death and complication in pediatric nephrotic syndrome. Common infections are bacterial infections such as Haemophilus influenzae, Klebsiella pneumoniae, Streptococcus pneumoniae, etc; Occasional cases of pneumocystis carinii infection. The peritoneum, lungs, and skin are often affected. The reasons for the susceptibility of this disease to infection include humoral, cellular immune deficiency, and complement factors; Ascites can become a culture medium, and the application of hormones and immunosuppressants can reduce immune function and make it more susceptible to infection.

4. Trace element deficiency: zinc, copper, and iron deficiency due to the loss of zinc binding protein, ceruloplasmin, and transferrin in the urine; Dysfunction in the synthesis of 1,25- (OH) 2D3 and long-term use of hormones can lead to renal bone disease and growth retardation.

5. Renal tubular dysfunction: Multiple substance transport disorders may occur. Such as diabetes, amino acid urine, increased urinary potassium, and decreased urine concentration function.

6. Malnutrition: caused by long-term loss of a large amount of protein in the urine of children with nephrotic syndrome.

Tips for maintaining children with kidney disease

1. Prevention of infection: children with kidney disease and children with infectious diseases were treated in separate rooms. Avoid getting cold and avoid crowded places.

2. Skin care: Keep the skin clean and dry, avoid scratches and pressure, and roll over regularly. The bedding should be loose and soft. Rubber air cushions or cotton loops can be placed on the buttocks and limbs, and an air cushion bed can be used if conditions permit. Edematous scrotum can be lifted with a cotton pad or sling, and the area of skin rupture should be covered with disinfectant dressing to prevent infection.

3. Observe changes in swelling: Record 24-hour intake and output, daily abdominal circumference and weight, and submit urine routine tests 2-3 times a week.

4. Individuals with severe edema should try to avoid intramuscular injection of food. Severe edema often leads to food retention, poor absorption, or the leakage of food from the needle hole after injection, resulting in local dampness, erosion, or infection. When intramuscular injection is necessary, pay attention to strict disinfection, and press for a slightly longer time after injection to prevent overeating

Diet for pediatric nephrotic syndrome

The diet for pediatric nephrotic syndrome should contain sufficient amounts of vitamin A, vitamin C, and B vitamins, as well as abundant iron. Calcium should also be supplemented to avoid osteoporosis caused by calcium deficiency. Avoid consuming stimulating foods and strong seasonings.

Children with nephrotic syndrome have a large amount of protein in their urine, which can lead to hypoproteinemia and hypercholesterolemia due to long-term protein excretion. In response to these situations, parents should develop a reasonable diet based on the patient's condition. However, due to the child's frequent loss of appetite and unwillingness to eat, high protein and high calories cannot be consumed. Reasonable diet is an important link in the treatment of pediatric kidney disease. Nephrotic syndrome is a chronic wasting disease characterized by systemic edema.

Therefore, parents of children with kidney diseases should regularly adjust the color, aroma, and type of diet, improve the quality of diet, meet the dietary habits of children, and encourage children to actively cooperate to meet the requirements of kidney disease nutrition therapy.

In severe edema and protein phase, a salt free and high protein diet such as eggs and lean meat should be given; Protein intake should be restricted during edema and oliguria periods; It is advisable to give an appropriate amount of protein during the non edema and non high protein period. After using diuretic gluttons, a low salt diet can be recommended. During the high diuretic period, sodium containing foods such as noodles and vegetable soup can be added.

After the normal urine volume and edema subside, it is important not to excessively limit salt intake. To avoid loss of appetite.