- What is Nephrotic syndrome in children
- What triggers / Causes of Nephrotic syndrome in children
- Pathogenesis (what is happening) during Nephrotic syndrome in children
- Symptoms of Nephrotic syndrome in children
- Diagnosis of Nephrotic syndrome in children
- Treatment of Nephrotic syndrome in children
- Prevention of Nephrotic syndrome in children
- Which doctors should be consulted if You have Nephrotic syndrome in children
What is Nephrotic syndrome in children -
Nephrotic syndrome in children is a complex of symptoms and laboratory parameters or a disease that is manifested peripheral or generalized edema (in extreme form, the ascites and anasarca). With regard to laboratory data with the diagnosis, fixed proteinuria more than 2.5 g/day or 50 mg/kg/day, hypoalbuminemia (less than 40 g/l), hypoproteinemia, hyperlipidemia, dysproteinemia and lipiduria.
Complications avtoritetnogo syndrome in children
Complications in this disease is caused by protein loss and hypovolemia. Immunity decreases, the child picks up the infection. You may experience iron deficiency anemia, which is not cured by the preparations of iron. Disrupted transport around the body of cholesterol, increases the risk of atherosclerosis. Can develop riskets-like syndrome, because the body loses vitamin-D-binding protein. Occurs increased bleeding, because there is a lack of procoagulums. Increases the amount of free thyroxine in the blood.
What triggers / Causes of Nephrotic syndrome in children:
In 10 cases out of 100 nephrotic syndrome in children – secondary, that is due to a specific cause (often a systemic disease). To NS in children can lead to such diseases:
- syndrome Alport
- hemorrhagic vasculitis
- thrombosis of renal vein
- thrombotic microangiopathy
Nephrotic syndrome is often a manifestation of primary glomerulonephritis. The researchers also speak of congenital nephrotic syndrome and familial NS.
It is important to know that nephrotic syndrome in children can be secondary. He is accompanied by a number of diseases in which the child is affected kidney:
- rheumatoid arthritis
- hemorrhagic vasculitis
- systemic lupus erythematosus
- renal amyloidosis
- microscopic polyangiitis and other
Pathogenesis (what is happening) during Nephrotic syndrome in children:
Nephrotic syndrome hypoproteinemia develops because of the urine are excreted from the body proteins, mainly albumins (disturbed permeability of the glomerular filter). Hypoproteinemia causes reduced oncotic pressure of plasma. For this reason, the liquid does not completely returned to bloodstream, begins to linger in the tissues. This explains the appearance of the patient's nephrotic edema. Also important is the condition of lymphatic capillaries, as hyperhydration tissue leads to compensatory increase of lymphatic drainage, together with the lymph tissue proteins are removed. This leads to a reduction reduces oncotic pressure interstitial fluid. This mechanism can up to a certain limit to prevent the development of edema with hypoproteinemia.
A clear correlation between the level of hypoproteinemia and degree of edema was not found. Swelling the child may not appear, even if in a reduced blood level of albumin. The explanation may be the inclusion of compensatory mechanisms. If NA the child develops during primary glomerulonephritis, it is called idiopathic nephrotic syndrome. Morphological changes are minimal, focal segmental glomerulosclerosis and nephrotic syndrome with minimal changes, which is denoted in abbreviated form as SMI, glomerular basement membrane is predominantly permeable to low molecular weight protein molecules, because proteinuria has been selective.
Symptoms of Nephrotic syndrome in children:
For nephrotic syndrome in children is characterized by swelling. First they appear on the face, then on the eyelids, can occur in the lumbar region and legs. Also among the characteristic symptoms note the pallor of the skin, oliguria associated with hypovolemia, hyperaldosteronism, lesions of the tubules. For NS in children typical secondary hyperaldosteronism, which causes the delay of sodium in the body. This leads, in turn, to water retention, although they note in the blood hyponatremia.
Hypovolemia in children develops spontaneously at the beginning of nephrotic syndrome, while the rate of loss of protein above the speed of mobilization of extravascular protein from the depot. Nephrotic edema have been generalized in nature. First, they are the eyes and ankles of the baby, then "move" on the torso, reaching the degree of anasarca and abdominal swelling. Swelling is characterized as soft, can be asymmetrical. Enlargement of the liver see if the swelling in the national Assembly strongly expressed.
Incomplete nephrotic syndrome is a form in which there is no swelling. Mixed nephrotic syndrome are diagnosed with a combination of NS with hematuria and hypertension.
Diagnosis of Nephrotic syndrome in children:
Nephrotic syndrome is diagnosed if the child has oedema and proteinuria, which can be detected by passing a urine test. The causes of the disease can be ascertained, carefully collected history. If the cause turns out to be difficult to identify, can take a biopsy of the kidney and/or serological tests. Diagnostic importance of content in the urine collected per day, 3 grams of protein. Also urine analysis determine that it has the erythrocytes and cylinders. The source is present lipiduria – in canalave cells have a free fat – if nephrotic syndrome triggered by the glomerular pathology.
Having simple microscopy, can be detected in urine cholesterol, which is "the Maltese crossing" under polarized light. Staining with Sudan should be used for the detection of triglycerides. Exudative diseases and SLE in large quantities are leukocytes.
To determine the severity and complications doctors may prescribe additional tests. The concentration of creatinine and nitrogen vary depending on the degree of renal dysfunction. Albumin is often less than 25 g/L.
Idiopathic nephrotic syndrome in children often presents with minimal changes (described above). It is usually diagnosed without biopsy, with the exception of cases when the body does not respond to a trial course of corticosteroids (glucocorticoids).
Treatment of Nephrotic syndrome in children:
As pathogenetic treatment of nephrotic syndrome in children using immunosuppressive therapy. Choose traditional, non-selective drugs:
Apply and selective immunosuppressants:
- cyclosporine a
- of mycophenolate mofetil
When the immunosuppressive treatment used GN such glucocorticoids:
Nephrotic syndrome is divided into 2 types depending on the response to hormonal therapy:
If your child was diagnosed with primary NS, prednisolone therapy will help, the disease will be released on remission. If the course of medication there is no reaction, no improvement occurs, then other approaches are necessary.
For the treatment used three modes of GC therapy:
- continuous oral administration of prednisolone at a dosage of 1-2 mg/kg (divided into 2-4 doses) is appointed immediately after the diagnosis of NS
- alternative receive mode (maintenance therapy)
daily dose of prednisone is taken day by day, which supports the clinical effect, but also reduces side effects.
Among the side effects that occur from taking prednisone:
- increased appetite
- skin atrophy
- steroid diabetes
- increased blood pressure
- and many other
- pulse therapy with methylprednisolone (to achieve very high concentrations of GC in plasma). Intravenously administered for 20 to 40 minutes to about 30 mg/kg MP once per 2 days.
Cyclophosphamide is taken orally or in the form of "pulses". If the assigned ingestion, the dose in most cases is 2.0–2.5 mg/kg/day, the course lasts from 8 to 12 weeks. Pulse therapy with cyclophosphamide is conducted on the background of alternating courses of prednisone in hormone-dependent and hormone-resistant nephrotic syndrome in a child is based 12-17 mg/kg intravenously.
Chlorambucil is necessary to give the child per os in a dose of 0.15–0.2 mg/kg per day, the course of 8-10 weeks. Today less used azathioprine and methotrexate, which are antimetabolites.
The cytotoxic drugs can cause such side effects:
- nausea and vomiting
- failure of the gonads
- hemorrhagic cystitis
- pulmonary fibrosis
- hepatopathy, etc.
Apply these drugs cyclosporine A:
- Sandimmun Neoral
Doctors before prescribing these drugs must appoint a kidney biopsy. The constant monitoring of creatinine levels in the blood and the concentration of the drug.
For the treatment can be used such drugs:
- of mycophenolate mofetil (CellCept)
They are new selective immunosuppressants. In identifying steroidreceptor spend?.
Principles of treatment of primary FSGS
Initially, the treatment of FSGS is carried out as a therapy for idiopathic NS. With appropriate treatment PZ remission occurs, which is a good prognostic sign. Approximately 95 percent of patients lives saved over 10 years. When it is possible to achieve stable remission of the NS therapy is performed as in NCMI. But if you set hormonereplacement, a need for a biopsy. And only at this stage, treatment in children actually FSGS, because earlier therapy was subjected idiopathic NS (without morphological refinement).
Therapy FSGS has as its objective the achievement of remission and a better prognosis. If this cannot be achieved, the only way to slow the progress of CKD is the reduction of proteinuria.
If indicators of proteinuria < 2 grams/day, 90 possible 10-year survival rate of patients, whereas the level of 2,5–5,0 – only about 45. If the protein level is equal to or greater than 14 g/day, without substitution therapy patients can accommodate a maximum of 6 years.
When treating children, because prednisolone was administered prior to biopsy and was proven hormonereplacement, therapy is carried out with alkylating agents, particularly cyclophosphamide, alternating mode is prednisolone. Perhaps the introduction of the "pulse" cyclophosphamide or methylprednisolone.
The best results gives compound in the therapy of FSGS cytotoxic (cytotoxic) drugs and glucocorticoids. In this case, the 10-year renal survival of patients an average of 80, whereas only in the treatment with GK – about 40, and using a CA – only 20. Achieving remission in adult patients with FSGS associated with the cumulative dose of GC. If the course of taking the medications for at least 8-9 months, the likelihood of remission is almost a 100-th.
Principles of treatment mezangioproliferativnom glomerulonephritis (Spgn)
Initially therapy Spgn, manifested in the form NS, similar to the treatment of idiopathic NS.
There is no single cure Spgn (IgA nephropathy). Because patients are often resistant, often the doctor prescribes cytotoxic agents (in particular, cyclophosphamide) and prednisone, applying the same scheme. And that in therapy of FSGS.
General recommendations for the treatment of patients with Spgn
1. The most important condition for proper and effective treatment – the implementation of a kidney biopsy.
2. In a rapidly developing renal failure, progressive nephrotic syndrome, vasculitis and nephritis with observed need treatment with corticosteroids without drugs or in conjunction with them.
3. The effectiveness of treatment Spgn increases in redevelopment of foci of chronic infection. Their destruction allows you to make the recurrence of the disease more rare. In addition, it is desirable aglyutenovaya diet, which helps to reduce and even reverse the development of symptoms in celiac disease.
4. To have a sick child as long as possible developed kidney failure, requires strict control of hypertension. Shown the renal protective treatment with blockers of angiotensin II receptors of the first type in combination with diuretics and angiotensin converting enzyme inhibitors.
Principles of treatment of MH in children
First of all, sick children with normal kidneys and in the absence avtoritetnogo syndrome immunosuppressive therapy your doctor is not assigned. But they should be constantly observed by a doctor.
If you develop hypertension and proteinuria detected more than 1 g/day, their correction is performed using angiotensin converting enzyme inhibitors. To normalize hyperlipidemia shown diet.
If MH manifests in the form of the NA or the disease has reached a late stage of development, therapy similar to the treatment of idiopathic NS. If there are frequent relapses avtoritetnogo syndrome, a sick child hormone-dependent or resistant, therapy is enhanced with the help of the MP, chlorambucil or Tzia (scheme Ponticelli C., de Santo, etc.). The use of CA, and combinations of corticosteroids in the future leads to more rare to relapse. However, GC monotherapy is as effective in achieving a decrease in symptoms as the combined intake of these drugs.
MH, according to researcher C. Ponticelli, is a potentially curable disease. And this must be considered when choosing the ways of treatment of the disease.
As you can see, immunosuppressive therapy is the basis of pathogenetic treatment of Ann. But not least, diet regime, in Addition to immunosuppressive therapy, no less important are diet and regime activities, rehabilitation of foci of infection, treatment of side ailments, eliminating possible allergens, etc. But the crucial condition for achieving persistent long-term remission, which improve the prognosis of the disease, early diagnosis, purpose, dose and therapy with glucocorticoids.
Prevention of Nephrotic syndrome in children:
For the prevention of nephrotic syndrome should be time to treat infectious diseases, not to delay going to the doctor. All recommendations of your doctor need to be followed. If some of you incomprehensible or obscure the reason of their appointment, check back with the doctor and not to self-cancel drugs or reducing the dose.
Dress appropriately for the weather that was not superheating and hypothermia. Be sure to buy a thermometer and hang out the window to know the temperature outside, and not rely on information on the weather sites. During exacerbation of seasonal allergies in time give your child antihistamines (before taking you need to consult to the doctor). If possible, insulate your child from allergens.
Which doctors should be consulted if You have Nephrotic syndrome in children: