Current Treatment of Nephrotic Syndrome In Children

Nephrotic syndrome is a clinical condition with symptoms of massive proteinuria, hypoalbuminemia, edema, and hypercholesterolaemia. Sometimes symptoms are accompanied by hematuria, hypertension, and decreased renal function. The incidence rate varies between 2-7 per 100,000 children, and more in boys than in women with a ratio of 2: 1.image

Nephrotic syndrome is a clinical condition in the presence of massive proteinuria (3.5 g / day), hypoalbuminemia, edema, and hyperlipidaemia. Usually it is followed by some primary glomerulonephritis disease or systemic disorder with a secondary affected kidney. Examples of primary renal diseases that cause nephrotic syndrome are: minimal change glomerulonephritis (GN), membranous gN, focal glomerulosclerosis, mesangial proliferative GN and membranoproliferative GN. Examples of secondary diseases that cause nephrotic syndrome are: diabetes, SLE, amyloidosis, henochschonleinpurpura, drugs, immune complex disease.

Nephrotic syndrome can be distinguished into congenital nephrotic syndrome, primary nephrotic syndrome, and secondary nephrotic syndrome. In general, most (± 80%) primary nephrotic syndromes respond well to initial treatment with steroids, but approximately 50% of them relapse repeatedly and about 10% respond no more with steroid treatment.

CongenitalimageNephrotic Syndrome

    Congenital nephrotic syndrome (CNS) is a very rare renal disorder syndrome, usually characterized by severe proteinuria simtoma, hypoproteinemia and edema that can be observed immediately after delivery. In general, CNS is caused by a deficiency of the constituent components of the glomerular filtration barrier, especially nephrine and podosin. CNS therapy can be an albumin infusion to prevent edema that can take the soul of the patient, nutritional intake with very high calories and thyroxine
    First reported in Finland, so it is also called Finnish type SN. This disorder is passed down through the recessive gene. Usually the child is born premature (90%), the placenta is large (weighs approximately 40% of body weight). Symptoms of asphyxia are present in 75% of cases.
    The first symptoms of edema, ascites, usually appear at birth or in the first week. On laboratory tests found hypoproteinemia, proteinuria massif and hipercolestrolemia.
    Other clinical symptoms include congenital abnormalities on the face such as a small nose, the distance of both eyes wide, the ears lying lower than normal.
    Prognosis is ugly and died Karen secondary infection or kidney failure. One way to find the possibility of this abnormality early is to examine the level of alpha-feto amniotic fluid protein that is usually elevated.

Clinical Manifestations

    Common complaints are swelling in both eyelids, abdomen, legs, or whole body which may be accompanied by decreased urine levels.
    Other complaints can also be found such as cloudy urine or if there is reddish hematuri.

Physical examination

    On physical examination, edema can be found in both eyelids, limbs,
    Ascites and scrotum / labia edema; Sometimes found hypertension.

Supporting investigation

    In urinalysis found massive proteinuria (≥ 2+), urincreatinine albumin> 2 and may be accompanied by hematuria.
    In the blood test, hypoalbuminemia (dL), hypercholesterolaemia (> 200 mg / dl) and elevated sedimentary blood rate were obtained. The levels of urea and creatinine are generally normal unless there is a decrease in kidney function.
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    Treatment with prednisone was given at an initial dose of 60 mg / m2 / day or 2 mg / kg / day (maximum 80 mg / day) in divided doses, for 4 weeks, followed by 2/3 starting dose (40 mg / m2 / day , Maximum 60 mg / day) a single daily dose of morning interval (alternating dose) for 4-8 weeks (see appendix) (ISKDC 1982)
    When relapse occurs, it is given prednisone 60 mg / m2 / day until remission occurs (up to 4 weeks), followed by 2/3 starting dose (40 mg / m2 / day) alternately for 4 weeks. In steroid or steroid toxic steroid-resistant nephrotic syndrome, other immunosuppressant drugs such as cyclophosphamide orally with a dose of 2-3 mg / kgbb / day in a single dose are administered under the supervision of a child's nephrology doctor. The dose was calculated on the basis of body weight without edema (50th percentile body weight by height)

Supportive Therapy

    If there is anasarka edema required bed rest. In addition to the administration of corticosteroids or immunosuppressants, other supportive treatments are required, such as normal protein diet (1.5-2 g / kgb / day), low-salt diet (1-2 g / day) and diuretics. Furosemide diuretics 1-2 mg / kgbb / day, if necessary combined with spironolactone (aldosterone antagonist, potassium-sparing diuretics) 2-3 mg / kgb / day when there is anacaral or edema edema that interferes with activity. If there is hypertension can be added antihypertensive drugs.
    Giving of albumin 20-25% at a dose of 1 g / kgbb for 2-4 hours to draw fluid from the interstitial tissue and ending with intravenous furosemide 1-2 mg / kgbb was performed on indications such as refractory edema, shock, or albumin ≤1 gram / DL. Psychological therapy to patients and parents is needed because the disease can be repeated and is a chronic disease.
    - Dosage of albumin: serum albumin level 1-2 g / dL: given 0,5g / kgBB / day; Albumin levels - The initial SN treatment scheme according to ISKDC 1967

Indications for referring to a pediatric nephrology specialist: image

    Onset of nephrotic syndrome under 1 year, history of nephrotic syndrome in the family
    Nephrotic syndrome with hypertension, persistent persistenthaematuria, impaired renal function, or accompanied by extrarenal symptoms, such as artitis, serositis, or skin lesions
    Nephrotic syndrome with complications of refractory edema, thrombosis, severe infection, toxic steroids
    - Steroid resistant nephrotic syndrome
    - Nephrotic syndrome often relapsed or steroid-dependent
    - Needed kidney biopsy

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    With the administration of prednisone or other immunosuppressants in the long term, it is necessary to monitor the possibility of drug side effects. Prednisone may cause hypertension or other side effects and cyclophosphamide may cause bone marrow depression and other side effects. Blood pressure checks need to be done routinely.
    On the use of cyclophosphamide peripheral blood examination is required every week.
    In the event of hypertension, prednisone is discontinued and replaced with other immunosuppressants, hypertension is treated with antihypertensive drugs. J
    If bone marrow depression occurs (leukocytes <3000 / uL) then the drug is suspended and continued again if the leukocyte is ≥5.000 / uL.
    Growth and development
    Developmental growth disorders may occur as a result of the disease of nephrotic syndrome alone or side effects of repeated prednisone administration in the long term.
    In addition, the disease is an immunocompromised state so it is very susceptible to infection. Recurrent infections can disrupt the growth of the patient.

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