Pediatric nephrotic syndrome is referred to as nephropathy. The cause of most nephropathies is unknown, so they are mostly referred to as primary nephropathies. It is mostly seen in babies aged 2 to 8 years.
Four common manifestations.
1, severe edema: eyelid or lower limb edema, serious can diffuse the whole body, with pleural fluid, ascites, edema is concave
2, a lot of proteinuria: urine protein + + + +, quantitative > 3.5g / day
3, hypoproteinemia: Albumin reduction is the main factor
4.Blood cholesterol is increased
If the baby has clinical manifestations of nephrotic syndrome on the basis of diseases such as allergic purpura, systemic lupus erythematosus and hepatitis B, these are called secondary nephrotic syndrome. Nephrotic syndrome that starts within the first 12 months of life is called congenital nephropathy and is usually genetically related, with poor treatment and a poor prognosis, usually dying from renal failure or infection within 3 years of age.
It is generally accepted that nephrotic syndrome is an immune disease, so the treatment of babies starts with hormone therapy, and some babies do not do well with hormone therapy, so some cytotoxic drugs may be added, mainly including cyclophosphamide, rhodopsin and azathioprine, etc. Other treatment measures include rest and nutrition, maintenance of water-electrolyte balance, infection control and symptomatic Other treatment measures include rest and nutrition, maintenance of water and electrolyte balance, infection control and symptomatic treatment.
A kidney biopsy is to remove a bit of tissue from the baby’s kidney with a very long needle and look at the specific lesion of the baby’s kidney under a microscope.
The prognosis of nephrotic syndrome in babies is very different. Generally speaking, babies who are sensitive to hormone therapy have a better prognosis, but those who are not effective in hormone therapy have a poorer prognosis.
Focus on prevention
The cause of nephrotic syndrome is not very clear so far, so it is difficult to prevent it from the cause. It may be helpful to strengthen physical exercise, enhance the body’s ability to resist disease, and actively prevent various infections. At the same time, you should try to avoid the use of drugs that can damage the baby’s kidneys.
Seeking help from a doctor
(1) When should I see a doctor?
When your baby has edema or low urine output, you should take your baby to the doctor promptly.
(2) What should I tell the doctor?
(1) Has your baby had any past symptoms such as rash, hematuria, prolonged fever, joint pain, etc.?
② Health condition of parents and family members: whether parents and family members have similar illnesses, any history of hereditary diseases, etc.
(3) How to cooperate with the doctor for examination and treatment?
(1) Physical examination: the most important thing is to observe whether the baby has signs of infection, the degree of edema, and the level of blood pressure
②We need to do many laboratory tests for the baby, including: blood routine, urine routine, liver and kidney function, lipid analysis, blood complement, blood sedimentation, anti-“O”, hepatitis B and C antibodies, autoantibodies, blood immunoglobulin, 24-hour urine protein quantification, etc. Ultrasound examination of both kidneys can give a general idea of the kidney damage.
③Kidney biopsy pathology examination-very necessary
④Therapeutic measures needed: including general treatment and special treatment.
General treatment
Rest is generally not necessary when there is no comorbidity. When the kidney disease is in remission, that is, after the edema subsides and the protein in the urine becomes negative, the activity can be gradually increased.
Diet There is no need for special avoidance of mouth, but salt avoidance is limited to edema and hypertension, and children with obvious hypertension are generally given a low salt diet. Protein intake should be moderate, so that it can replenish urine loss and meet the needs of normal growth and development. Approximately 1.5-2 grams per kilogram of body weight per day should be given. The fat content should be low and various vitamins should also be supplemented.
Prevention of infection It is important to induce remission, avoid exacerbation, and reduce recurrence.
Diuresis Diuretics can be appropriately considered when severe edema is causing distress in the child, but are generally not mostly advocated because they a can cause electrolyte disturbances, b hinder the observation of drug efficacy, and c cause other comorbidities by making the effective circulating blood volume even less.
Special treatment
Adrenocorticotropic hormone (hormone for short)
Since the application of hormone therapy began in the 1950s, the outcome of nephropathy has been greatly improved, and most children can obtain remission. To date, hormones are still the most effective, cheapest, available everywhere, and with relatively few side effects, so they are still used as the drug of choice for treating nephropathy. The commonly used preparations are prednisone and prednisolone. Hormone therapy can bring more than 80% of children with kidney disease into remission within 8 weeks, but 60-90% of children can still relapse later.
Prednisone therapy can be short-course, medium-course or long-course. Short-course therapy has a high relapse rate and is not used. In China, medium and long course therapy is more commonly used, with the medium course lasting for 6 months and the long course for 9-12 months.
Hormone therapy can be divided into three stages: initial continuous administration for a certain period of time to induce remission (initial therapy), followed by long-term maintenance by intermittent administration (maintenance therapy), and finally, gradual reduction and discontinuation of the drug (withdrawal therapy).
Initial therapy is designed to achieve initial efficacy; maintenance therapy aims to sustain efficacy and minimize adrenocortical suppression side effects; and withdrawal therapy is carried out while preventing the occurrence of withdrawal syndrome.
Immunosuppressants (cytotoxic drugs)
For the treatment of refractory nephropathy, raptor polysaccharide, 6-TG, cyclophosphamide, cyclosporine, mycophenolate
Others: methylprednisolone shock therapy
Home treatment care
1. Babies with nephrotic syndrome should have a low-salt, low-fat, low-protein diet.
(1) The food chosen should be high in calories, rich in high quality protein, low in fat, low in sodium, high in calcium and rich in vitamins. Babies can eat more foods rich in high quality protein, such as fine meat, milk, fish and eggs, and less foods rich in vegetable protein, such as soybeans and soy products. To ensure that your baby has the energy needed for normal growth and development, you can eat more sugar-rich foods such as potatoes, lotus root powder and sweet potatoes. Babies can eat vegetable oils and less non-vegetarian oils.
(2) Diet for babies with nephrotic syndrome can be given a low-salt diet during the edema stage. Water is not limited, enough calories should be supplied, and general foods suitable for the baby’s appetite can be used without any special taboos. During the period of taking a lot of hormones, the appetite increases, so care should be taken not to overeat, so as not to cause excessive obesity. As for the protein intake, as long as it can ensure the growth and development needs, plus the amount lost from the urine, do not give high protein diet because of the existence of hypoproteinemia in babies with kidney disease, and more use of plasma and albumin and other blood products, high protein diet can not make the plasma albumin rise, but increase the burden on the kidney, promote glomerulosclerosis, and can also affect the urine protein from positive to negative and make the recurrence of kidney disease The number of times to increase
(3) salt avoidance is limited to patients with edema or hypertension, long-term salt avoidance affects appetite, but also can lead to low blood sodium, edema is serious at the same time pay attention to control water intake. Large amounts of diuresis, diarrhea or vomiting cause a large loss of body fluids, should be timely replenishment of sodium and water.
2, a variety of infections, especially the cold is the main cause of the recurrence of nephrotic syndrome in babies, so at home we should pay special attention to the environmental health of the living room, to keep the air circulation, to prevent the occurrence of respiratory tract infections. If your baby accidentally catches a cold, go to the hospital in time.
3, do not give your baby a variety of preventive vaccinations, must be healed 3 to 6 months before vaccination, otherwise it may lead to the recurrence of the disease
4, most babies with nephrotic syndrome have a better prognosis, partly because of the improved understanding of it in recent years and the progress in the level of diagnosis and treatment. The more important reason is that it is very important for babies and their parents to adhere to long-term follow-up and regular treatment, which to a certain extent determines the prognosis of babies.