Nephrotic Syndrome

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Chapter 53 Nephrotic Syndrome

PATHOPHYSIOLOGY

Nephrotic syndrome is the clinical state in which the glomerular membrane has an increased permeability to plasma proteins. This leads to severe edema, proteinuria, and hypoalbuminemia. The loss of protein from the vascular space causes decreased plasma osmotic pressure and increased hydrostatic pressure, resulting in the accumulation of fluids in interstitial spaces and the abdominal cavity. The decrease in vascular fluid volume stimulates the renin-angiotensin system, resulting in secretion of antidiuretic hormone (ADH) and aldosterone. Tubular resorption of sodium (Na⁺) and water is increased, expanding the intravascular volume. This fluid retention leads to increased edema as retained fluid shifts into the interstitial space. Coagulation and venous thrombosis may occur as a result of decreased vascular volume, which causes hemoconcentration and urinary loss of coagulation proteins. Loss of immunoglobulins through the glomerular membrane can lead to increased susceptibility to infection. Hyperlipidemia is believed to occur because of increased synthesis of lipoproteins in response to low plasma oncotic pressure.

Nephrotic syndrome is the pathologic outcome of various factors that alter glomerular permeability. The causes of nephrotic syndrome can be categorized into primary (idiopathic) and secondary (Box 53-1). Primary nephrotic syndrome is divided into three histologic groups: minimal-change nephrotic syndrome (MCNS), focal segmental glomerulosclerosis (FSGS), and membranous neuropathy (rare in children). Based on clinical classification, the syndrome types differ according to the course of the disease, treatment, and prognosis. It is considered a chronic illness because of the occurrence of relapses. Many children will have five or more relapses over the course of the disease. A child is considered to have frequently relapsing nephrotic syndrome if there are two or more relapses within the first 6 months and/or four or more relapses within a 12-month period. The frequency of relapse decreases over time and becomes relatively rare by adolescence.

INCIDENCE

1. The prevalence of idiopathic nephrotic syndrome is approximately 16 per 100,000.

2. Idiopathic nephrotic syndrome can occur at any age. Most often, it first manifests in children between ages 2 and 6 years.

3. Of all cases of nephrotic syndrome in children, 80% are MCNS. Incidence is slightly greater in males.

4. The mortality and prognosis of children with nephrotic syndrome vary with the disorder’s etiology and severity, the extent of renal damage, the child’s age and underlying condition, and response to treatment.

5. Nephrotic syndrome mortality and prognosis can range from complete recovery to causing end-stage renal disease (ESRD).

6. Children with MCNS tend to have a good prognosis.

7. Prognosis is more likely to be poor in children who do not respond to treatment and/or with some of the less common types of nephrotic syndrome such as FSGS.

COMPLICATIONS

1. Fluid/electrolyte balance: intravascular volume depletion (hypovolemia), respiratory compromise (related to fluid retention and abdominal distention), skin breakdown (from severe edema, poor healing)

2. Cardiovascular: hypercoagulability (venous thrombosis)

3. Immune: infection (especially cellulitis, peritonitis, pneumonia, septicemia)

4. Multiple body systems: untoward side effects of medications, growth failure, and muscle wasting (long-term)

LABORATORY AND DIAGNOSTIC TESTS

Nephrotic syndrome is generally diagnosed based on clinical presentation and laboratory test results.

Refer to Appendix D for laboratory and diagnostic values.

1. Urinalysis, urine dipstick—to detect protein and blood in the urine.

a. Proteinuria (urinary protein loss)

b. Hematuria (may be microscopic)

2. Urinary protein/creatinine ratio—calculated to monitor persistent proteinuria; best if first void of the morning is used; more accurate than 24-hour urine protein collection

a. Normal value <0.5

b. Values of >2 mg protein/mg creatinine are associated with nephrotic syndrome

3. Serum chemistry, lipid panel—to assess electrolytes, serum protein, renal function, lipids

a. Serum electrolytes—vary with individual disease states and treatments

b. Blood urea nitrogen and creatinine—may be increased if renal impairment has occurred

c. Serum albumin—decreased, related to protein loss

d. Serum cholesterol and triglycerides—increased and related to increased lipoprotein synthesis

4. Complete blood count—to monitor for hemoconcentration and infection

a. Hemoglobin, hematocrit, and platelets—increased (related to hemoconcentration)

b. White blood cell count—may be increased if infection is present

5. Renal biopsy—is done to determine the glomerular status, type of nephrotic syndrome, response to medical management, and disease course, most often used for patients who are steroid resistant and/or steroid dependent. Microscopic evaluation shows abnormal appearance of basement membranes.

MEDICAL MANAGEMENT

Medical management will vary according to the type of nephrotic syndrome and as new data are available regarding the efficacy of various treatments. Corticosteroids (prednisone, prednisolone) are traditionally administered daily until remission is achieved (cessation of urinary protein loss). Corticosteroids are usually tapered off by administering on alternate days and/or with decreasing doses. Children with MCNS tend to respond quickly to steroid therapy. Approximately 75% achieve remission by 2 weeks and 95% by 8 weeks. Only about 20% of children with FSGS achieve remission by 8 weeks. Relapses may be treated with additional courses of corticosteroids. For children who fail to achieve remission after 8 weeks of steroids, they are often deemed “steroid resistant.” Immunosuppressive therapy (alkylating agents [cyclophosphamide, chlorambucil], cyclosporine, or levamisole) may be used to stimulate remission in children with steroid-resistant nephrotic syndrome and/or to decrease the frequency of relapses. Dietary sodium restriction (no added salt) is used to reduce edema. A high protein diet and/or intravenous albumin infusion are used for protein replacement and to restore fluid balance.

Diuretic therapy may be needed to treat severe edema (i.e., when severe fluid retention interferes with respiration and/or causes skin breakdown). Diuretics should be used with caution to prevent intravascular volume depletion, thrombus formation, and/or electrolyte imbalances. Electrolyte replacement is administered as needed. Antibiotics may be administered to prevent or treat infection. Pain (related to edema and invasive therapy) may be treated with medications and nonpharmacologic approaches (see Appendix I). Anticoagulants may be used to prevent or treat thrombosis. Antihypertensives (i.e., angiotensin-converting enzyme [ACE] inhibitors) may be used as needed to control blood pressure and decrease urinary protein losses.

NURSING ASSESSMENT

1. Assess for signs and symptoms of fluid volume excess.

a. Local edema: periorbital, facial, external genitalia, and/or extremities

b. Abdominal edema (ascites): clothing that feels or appears to be “too tight,” increased abdominal girth, abdominal distention with taut and shiny skin (severe edema)

c. Anasarca (severe, generalized edema)

e. Decreased urine output

f. Dark, frothy urine

g. Pulmonary congestion, increased respiratory effort, pleural effusions, pulmonary edema

2. Assess for signs of electrolyte imbalance.

a. Assess for signs of hypokalemia.

i. Cardiovascular: arrhythmias, flattened T waves, decreased ST segment, widened QRS, increased PR interval, gallop rhythm, increased or decreased heart rate, hypotension

ii. Central nervous system (CNS) and musculoskeletal: apathy, drowsiness, muscle weakness, muscle cramping, hyporeflexia

b. Assess for signs of hyponatremia (related to diuresis).

i. CNS: apathy, weakness, dizziness, lethargy, encephalopathy, seizures

ii. Cardiovascular: hypotension

iii. Gastrointestinal (GI): nausea, abdominal cramping

c. Assess for signs of hypernatremia (related to hemoconcentration)

i. CNS: disorientation, muscle twitching, lethargy, irritability

ii. GI: intense thirst, dry membranes, nausea, and vomiting

iii. Other: dry, flushed skin; increased temperature; oliguria

3. Assess protein loss and nutritional status.

a. Monitor serum protein and urine lab tests.

b. Assess appetite and nutritional intake.

c. Assess nails for signs of prolonged hypoalbuminemia (white [Muehrcke’s] lines parallel to the lanula).

d. Assess for pallor.

e. Assess for irritability, weakness, and fatigue.

4. Assess for side effects from medication administration.

a. Steroids: cushingoid features, hyperglycemia, infection, hypertension, obesity, GI bleeding, growth retardation, bone demineralization, cataracts

b. Alkylating or cytotoxic agents: leukopenia, infection, GI discomfort, GI bleeding, alopecia, impaired growth, gonadal dysfunction and/or sterility (long-term effect)

c. Diuretics: intravascular volume depletion, thrombus formation, electrolyte imbalance

d. Antihypertensives: hypotension, dizziness

e. Anticoagulants: bleeding (GI, nose bleeds, oozing from puncture sites, etc.)

5. Assess for signs of decreased cardiovascular functioning (hypotension, hypertension, shock, congestive heart failure, cardiac arrhythmias, fluid volume deficit).

a. Blood pressure (hypotension or hypertension)

b. Heart rate and rhythm (tachycardia, arrhythmias)

c. Distal perfusion (pulses, capillary refill, temperature, color)

d. Left ventricular hypertrophy (arrhythmias, increased heart size, decreased output)

6. Assess for signs of orthopnea, pulmonary congestion, and/or pulmonary infection.

a. Respiratory rate and pattern (tachypnea, irregular pattern)

b. Use of accessory muscles (retractions, shoulder shrugging) and nasal flaring

c. Need to sit upright or to have the head of the bed elevated

d. Abnormal breath sounds (rales, ronchi, decreased breath sounds in lower lobes)

e. Abnormal chest x-ray

f. Cyanosis, decreased oxygen saturations

g. Respiratory acidosis

7. Assess for signs of infection.

b. Increased white blood cell count

c. Positive cultures (pulmonary secretions, urine, blood, other body fluids)

d. Signs of cellulitis: local swelling, redness, tenderness

e. Signs of pneumonia (see above)

f. Signs of peritonitis: red, tender abdomen

g. Septicemia, septic shock

8. Assess for skin breakdown from severe edema.

9. Assess child’s comfort level and ability to tolerate activity. Address child’s and family’s concerns and fears related to disease and altered body image.

10. Assess child’s and family’s coping response to illness.

a. Assess family functioning related to child’s irritability and mood swings.

b. Assess coping related to altered body image from severe edema and pallor.

c. Assess child’s and family’s response to bed rest and activity limitation.

NURSING INTERVENTIONS

1. Monitor and maintain fluid balance.

a. Assess hydration status frequently.

b. Monitor ascites by monitoring abdominal girth.

c. Closely monitor edematous areas; report changes as indicated.

d. Measure and record daily weights; report changes as indicated.

e. Record accurate input and output.

f. Administer diuretics if ordered; assess effectiveness; closely monitor for side effects.

g. Replace fluids lost as a result of interstitial fluid shifts.

h. Administer albumin if ordered to increase vascular volume and serum protein.

i. Monitor type of fluids and administration rate to avoid fluid overload and cerebral edema while maintaining adequate vascular volume.

j. Promote bed rest as needed during periods of severe edema and periods of rapid weight loss during periods of diuresis.

2. Monitor electrolytes on an ongoing basis. Administer measures to correct electrolyte imbalance as indicated.

3. Encourage and support nutritional intake and proper nutritional status.

a. Continually monitor appetite and nutritional intake.

b. Complete calorie counts and nutritional screens as indicated.

c. Provide diet high in calories and protein.

d. Decrease sodium intake (avoid high-sodium foods, no added salt diet).

e. Avoid extreme salt restrictions or extremely high-protein foods since these may be undesirable to children and/or lead to paradoxic problems.

f. Allow the child as many dietary choices as possible; provide child’s favorite foods as allowed.

g. Offer food in small quantities and in an attractive manner.

h. Support family in providing a calm, relaxed atmosphere during mealtimes (avoid high-pressure feeding or disruptive events).

i. Consider intravenous (IV), nasogastric, or nasojejunal feedings if patient is unable to maintain proper nutritional status.

j. Collaborate with clinical nutritionist as indicated.

4. Assess adequacy of ventilation and promptly implement airway stabilization methods as indicated; encourage patient to cough and deep breathe (refer to Nursing Assessment in this chapter for signs of respiratory distress).

5. Maintain skin integrity and prevent infection.

a. Practice good handwashing, use of alcohol based agents, and aseptic technique.

b. Provide skin care, eye care, and perianal care on an ongoing basis.

c. Reposition and turn patient frequently; prevent pressure ulceration with pressure relief/reduction surfaces; avoid hard objects (tubing, cables) under patient.

d. Avoid restrictive clothing, armbands, and tape.

e. Place on pressure-relief surface as needed if there is severe edema and/or skin breakdown.

f. Maintain sterility of all invasive lines, and perform dressing changes and site care as needed and on schedule.

g. Monitor for signs of infection and implement appropriate interventions as indicated.

6. Monitor for pain and provide pain relief measures as needed (see Appendix I).

a. Assist child to find comfortable positions; reposition frequently.

b. Administer analgesics as needed.

c. Use nonpharmacologic pain relief methods as appropriate.

d. Continually reevaluate the effectiveness of pain relief measures.

7. Provide emotional support to child and family (see Appendix F).

a. Coping and managing the disease

b. Dealing with changes with bodily appearance and function

ANNA Pediatric Nephrology Special Interest Group. Pediatric nephrotic syndrome fact sheet. (website) www.annanurse.org/download/reference/practice/pns.fact.pdf Accessed February 7, 2007

Broome L. Treating pediatric nephrotic syndrome: A clinical challenge. Nephrol Nurs J. 2003;30(6):662.

Eddy A, Symons JM. Nephrotic syndrome in childhood. Lancet. 2003;362(9):384.

Luxner KL. Delmar’s pediatric nursing care plans, ed 3. Clifton Park, NY: Delmar Thompson Learning, 2005.

Moses S. Family practice notebook: Urine protein to creatinine ratio. (website) www.fpnotebook.com/URO72.htm Accessed May 4, 2006

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