DEFINITION

Nephrotic syndrome is a primary glomerular disease characterized by proteinuria, hypoproteinemic

Defined as

– protein excretion of > 40 mg/m2/hr

– First morning protein : creatinine ratio of > 2-3 : 1

INCIDENCE
2 – 7 cases per 100,000 children
per year
Higher in underdeveloped
countries ( South east Asia )
Occurs at all ages but is
most prevalent in children
between the ages 1.5-6
years.
It affects more boys than girls, 2:1
ratio
TYPES

1. Idiopathic NS: In childhood, the vast majority belongs to category it is regarded as a sort of
autoimmune phenomenon, especially since it responds well to immunosuppressive therapy. It is two
types: a. Minimal change NS – this predominant type, seen 86% of the cases. b. Significant change
NS – this is infrequent. Mesangial proliferation is seen in 5% cases and focal sclerosis in 10% of cases.

2. Secondary NS: • It occurs in children (about 10%) of all cases. • This condition may occur due to
some form of chronic glomerulonephritis, or due to diabetes mellitus, SLE, malaria, malignant
hypertension, hepatitis B, infective endocarditic, HIV/AIDS, drug toxicity, lymphomas syphilis etc.

3. Congenital NS: • It is rare but a serious and fetal problem usually associated with other congenital
anomalies of kidney. • It is inherited as autosomal recessive disease. • Severe renal insufficiency &
urinary infections along with this condition result is poor prognosis.

4. Infantile NS: • The term is applied to NS occurring in infants between 4 – 12months of age. Its
major causes are: A. NPHS2 B. Diffuse mesengial sclerosis (DMS)

ETIOLOGY

1. Primary renal cause • Minimal change nephropathy • Glomerulosclerosis • Acute post

2. Systemic cause • Infections • Toxins – mercury, bismuth, gold • Allergic – bee sting, inhaled
pollen, food allergy • Cardiovascular – sickle cell disease, renal vein thrombosis, congestive heart
failure • Malignancies – leukemia • Others – systemic lupus erythematous, anaphylactic purpura

PATHOPHYSIOLOGY

Alteration in glomerular basement membrane

Decreased colloidal osmotic pressure

Decreased vascular volume

Decreased renal blood flow

Increased loss of protein in urine

Altered glomerular protein permeability

Increased secretion of aldosterone

Edema

Tubular Na and H2O reabsorption

CLINICAL MANIFESTATION
Four main symptoms of nephritic symptoms:• Protein urea • Hypoalbuminemia • Hyperlipidemia •
Edema

• Edema

– Mild to start with – peri orbital puffiness, lower extremities

– Progression to generalized edema, ascites, pleural effusion, genital edema

OTHERS:

SOB (Shortness of breath)

Decreased urine output

Anorexia, Irritability, Abdominal pain and diarrhoea

Absence of

Hypertension

Gross hematuria

Mild headache

Fever,

rash,

joint pain

Weakness

Malaise

Weight gain

Irritability

Anemia due to loss of RBCs

Flank pain

Fatigue.

DIAGNOSIS

PALPATION: Due to edema and ascites kidney cannot be palpable.

Urine analysis

Haematuria

24 hour urinary total protein estimation – urine sample shows proteinuria (>3.5 g per liter per 24
hours)

Blood test

BUN S.creatinine S.protein Desreases

Lipid profile shows high level of S. cholesterol 200mg.

– Spot UPC ratio > 2.0

– UPE > 40 mg/m2/hr

• Serum Creatinine – normal or elevated

 Needle biopsy of kidney

INDICATIONS

• Age below 12 months

• Gross or persistent microscopic hematuria

• Low blood

• Hypertension

• Impaired renal Function

• Failure of steroid therapy

 ECG

 KUB – X.ray

 Renal ultrasound

 Renal scan

 Intravenous urogram (IVU).

MANAGEMENT MEDICAL MANAGEMENT:

The goal of medical management is reduction of protein excretion

If causative agent is streptococcal then treated with penicillin antibiotics

If significant edema – diuretics Aldosterone antagonist ( Fursemide, spironolactone )

Corticosteroid therapy with Prednisolone or prednisone

Prednisolone is the drug of choice

( 2mg/kg per day for 6 weeks followed by

1.5 mg/kg single morning dose on alternate days for 6 weeks )

Proteinuria disappears within the first week of therapy and negative dipstick test for 2 consecutive
days shows positive response to treatment. Management of Relapse
.

Management of Relapse

• Parent Education

• Symptomatic therapy for infections in case of low grade proteinuria

• Persistent proteinuria ( 3 - 4+ ) –

– Prednisolone

( 2mg/kg/day until protein is negative for 3 days )

1.5 mg/kg on alternate days for 4 weeks )

Frequent Relapses

• Alternate Day prednisolone

• Steroid sparing agents

– Levamisole ( 2 – 2.5 mg/kg )

– Cyclophosphamide ( 2 – 2.5 mg/kg/day)

– Mycophenolate Mofetil ( 20 – 25 mg/kg/day )

– Cyclosporin ( 4 – 5 mg/kg/day )

– Tacrolimus (0.1 – 0.2 mg/kg/day )

– Rituximab ( 375mg/m2 IV once a week )

• Diuretic and salt poor albumin may be indicated in presence of severe edema.

Frusamide (1-44 mg/kg/day in 2 divided doses) may be prescribed.

DIETARY MANAGEMENT

• High protein diet

• Salt moderation

• Treatment of infections

Children should take a well-balanced diet rich in protein.

Sodium is restricted when marked edema is present

. Provide high protein and high carbohydrates diet to patient.

If disease in advance stage then avoid protein intake because it is affected to kidney.

Water restriction may be indicated if decreasing salt intake does not control edema.
NURSING MANAGEMENT

The major goals for the child with nephrotic syndrome are relieving edema, improving nutritional
status, maintaining skin integrity, conserving energy, and preventing infection.

Design the nursing care plan to include all these goals.

NURSING DIAGNOSIS:

• Risk for infection related to immunosuppressive drugs.

• Fluid and electrolyte imbalanced related to edema.

• Impaired skin integrity related to disease process.

• Altered nutrition related to Anorexia.

• Altered kidney function related to disease condition.

• Knowledge deficit related to disease process.

Care during hospitalization:

Child is hospitalized from initial therapy. Patient may not understand importance of hospitalization
because initially the child is symptomless. During hospitalization parents should be involved in child
care and goal setting.

Nurses should regularly monitor the vital signs and check the Childs daily weight.

Monitor signs of infection and edema.

Detailed chatting of intake/output most be done to monitor child’s response to medical therapy.

Daily urine examination for albumin is required.

Administer the prescribed medications:

Children with nephritic syndrome are receiving steroids so the nurse most be aware of the side
effects of these drugs.

Patient should be observed for gastrointestinal bleeding, gastro intestinal ulcers, hyperglycemia and
cataract.

Steroid is continued till the child is protein free, thereafter the drug dose in decreased gradually.

MAINTAIN FLUID AND ELECTROLYTE BALANCE:

Accurately monitor and document intake and output.

The abdomen may be greatly enlarged with ascites (edema in scale in the same clothing. Measure
the child’s abdomen daily at the level of the umbilicus the peritoneal cavity). The abdomen can even
become marked with striae (stretch marks).

Test the urine regularly for albumin and specific gravity. Albumin can be tested with reagent strips
dipped into the urine and read by comparison with a color chart on the container.

• Nurses should monitor serum sodium level of the child.

• Fluid intake either oral or I/V should be strictly monitored.

• Child is assessed for venous stasis, ascites and pulmonary edema.

IMPROVING NUTRITIONAL INTAKE

• Daily weight of child is accurately documented. Although the child may look plump, underneath
the edema is a thin, possibly malnourished child. The child’s appetite is poor for several reasons

: • The ascites diminishes the appetite because of the full feeling in the abdomen.

• The child may be lethargic, apathetic, and simply not interested in eating. • Ano-added-salt or low
salt diet may be unappealing to the child

. • Corticosteroid therapy may decrease the appetite. Offer a visually appealing and nutritious diet.
Consult the child and the family to learn which foods are appealing to the child.

Cater to the child’s wishes as much as possible to perk up a lagging appetite.

A dietitian can help to plan appealing meals for the child.

Serving six small meals may help increase the child’s total intake better than the customary three
meals a day.

PROMOTING SKIN INTEGRITY

The child’s skin is stretched with edema and becomes thin and fragile.

Inspect all skin surfaces regularly for breakdown.

Because the child is lethargic, turn and position the child every 2 hours.

Protect skin surfaces from pressure by means of pillows and padding.

Protect overlapping skin surfaces from rubbing by careful placement of cotton gauze.

Bathe the child regularly.

Thoroughly wash the skin surfaces that touch each other with soap and water and dry them
completely. A sheer dusting of cornstarch may be soothing.

If the scrotum is edematous, use a soft cotton support to provide comfort

The child is on corticosteroid therapy (immunosuppressant) and there is loss of immunoglobulin in

Strict aseptic technique should be used during invasive procedures.

Monitor vital signs for early signs of infection.

Isolate the child as he is on immunosuppressive therapy

The child with nephrotic syndrome is especially at risk for respiratory infections because the edema
and the corticosteroid therapy lower the body’s defenses.

Protect the child from anyone with an infection: staff, family, visitors, and other children.
Handwashing and strict medical asepsis are essential. Monitor vital signs every 4 hours and observe
for any early signs of infection.

In a few children, the persistence of abnormal urinary findings after diuresis presents a less hopeful
outlook. A child who has frequent relapses lasting into adolescence or adulthood may develop renal
failure and eventually be a candidate for a kidney transplant.

PROMOTE REST

Promoting Energy Conservation Bed rest is common during the edema stage of the condition.

The child rarely protests because of his or her fatigue.

The sheer bulk of the edema makes movement difficult.

When diuresis occurs several days after beginning prednisone, the child may be allowed more
activity, but balance the activity with rest periods and encourage the child to rest when fatigued.

Plan quiet, age-appropriate activities that interest the child. Most children love having someone read
to them. Coloring books, dominoes, puzzles, and some kinds of computer and board games are quiet
activities that many children enjoy.

Provide passive play to the child as tolerated e.g, watching TV, reading story books, etc.

Allow a period of rest after activities.

Limit visitors during acute phase of illness.

Involve the family in providing some of these activities. Avoid using television excessively as a
diversion

PROVIDE EMOTIONAL SUPPORT

Explain parents about the disease and its treatment

Allow the patients and child to express their feelings.

Encourage child to wear own clothes rather than hospital clothes as this make the child feel good.

DISCHARGE PLAN

Providing Family Teaching and Support Children with nephrotic syndrome are usually hospitalized
for diagnosis, thorough evaluation of their general health and specific condition, and institution of
therapy.

. Provide a written plan to help family caregivers follow the program successfully

Explain to patients about treatment programmed, follow up and risk of relapse.

Encourage patients to measure child’s weight weekly in order to identify early fluid retention.

Tell then to contact doctor if any unusual symptoms appear.

Increase intake of fruits and vegetables. No potassium and phosphorus restriction in necessary.

Explain about the medications to be continued at home and their side effects like cushingoid
appearance, gastrointestinal bleeding and sodium retention

. If the child is on corticosteroid therapy for very long time, fundus checkup should be done because
prednisolone causes cataract. o

Dietary modifications should be explained to the parents.

Ask them to avoid saturated fats such as butter, cheese, fried foods, and fatty cuts of red meat
and egg yolks and increase unsaturated fat intake including olive oil, canola oil, peanut butter, and
nuts. The child can eat low fat desserts.

COMPLICATIONS

• Edema

• Infections

• Thrombotic complications

• Hypovolaemia and Acute renal Failure

• Steroid Toxicity