Nephrotic Syndrome Nursing Care Planning & Management: Study Guide

Nephrotic syndrome is a constellation, not a single disease: heavy proteinuria, low serum albumin, high cholesterol and triglycerides, and edema. It runs with remissions and exacerbations over months. On the floor your work is managing edema and fluid balance, protecting fragile skin, preventing infection in an immunosuppressed child, and teaching families to monitor urine protein at home.

What is Nephrotic Syndrome?

Nephrotic syndrome (nephrosis) is defined by nephrotic-range proteinuria, edema, hyperlipidemia, and hypoalbuminemia. It can affect any age but is most common in children, especially between the ages of 2 and 6 years. Nephrotic-range proteinuria in adults means protein excretion of 3.5 g or more per day; in children it is protein excretion of more than 40 mg/m2/h or a first-morning urine protein/creatinine of 2-3 mg/mg creatinine or greater.

Pathophysiology

In a healthy person, less than 0.1% of plasma albumin crosses the glomerular filtration barrier; amounts above 500 mg/day signal glomerular disease. The glomerular capillaries are lined by a fenestrated endothelium on the glomerular basement membrane, covered by glomerular epithelium (podocytes) whose foot processes wrap the capillaries. Filtration slits sit between the foot processes, and these three structures (fenestrated endothelium, glomerular basement membrane, glomerular epithelium) form the filtration barrier. Plasma water and solutes filter extracellularly through the endothelial fenestrae and filtration slits.

Genetics shows the barrier's importance: in congenital nephrotic syndrome of the Finnish type, the gene for nephrin (a filtration-slit protein) is mutated, causing nephrotic syndrome in infancy; podocin, a podocyte protein, may be abnormal in children with steroid-resistant focal glomerulosclerosis. Proteinuria follows damage to the endothelial surface, the basement membrane, or the podocytes, and any type of nephrotic syndrome may involve one or more of these. Albuminuria alone may occur, or with greater injury all plasma proteins leak. Two hypotheses explain the edema: the underfill hypothesis blames albumin loss and lower plasma colloid pressure, while the overfill hypothesis blames primary renal sodium retention.

Statistics and Incidences

Nephrotic syndrome is present in as many as 7 children per 100,000 population younger than 9 years of age. Average age of onset is 2.5 years, with most cases between the ages of 2 and 6 years. The US annual incidence is 2-7 cases per 100,000 children younger than 16 years. In children younger than 8 years at onset, the male-to-female ratio runs from 2:1 to 3:2 across studies, and nephrotic syndrome may occur at a rate of 20 cases per million children. Because diabetes is a major cause, American Indians, Hispanics, and African Americans have a higher incidence than white persons. There is a male predominance, as with chronic kidney disease in general.

Causes

Causes are primary or secondary. Primary causes are kidney diseases such as minimal-change nephropathy, membranous nephropathy, and focal glomerulosclerosis. Secondary causes are systemic diseases such as diabetes mellitus, lupus erythematosus, and amyloidosis.

Clinical Manifestations

The first sign in children is usually facial swelling, followed by swelling of the whole body. Edema is the salient feature, starting around the eyes and legs and becoming generalized with weight gain, ascites, or pleural effusions. Malnutrition can become severe, but generalized edema masks the tissue loss, so the child looks chubby and may double their weight. Anorexia, irritability, and loss of appetite develop. These children are prone to infection, typically repeated acute respiratory illness, and a respiratory tract infection often immediately precedes onset. Albumin leaks from structural damage, producing proteinuria. About 30% of children with nephrotic syndrome have a history of allergy.

Assessment and Diagnostic Findings

Labs should confirm nephrotic-range proteinuria, hypoalbuminemia, and hyperlipidemia. First-morning urine protein/creatinine is easier than 24-hour collection, possibly more reliable, and excludes orthostatic proteinuria; a ratio over 2-3 mg/mg is consistent with nephrotic-range proteinuria. Serum albumin is generally less than 2.5 g/dL; serum sodium is low from hyperlipidemia (pseudohyponatremia) and dilution from water retention; on CBC, increased hemoglobin and hematocrit indicate hemoconcentration and intravascular volume depletion, and the platelet count is often increased. Infants and congenital cases should be tested for NPHS1 and WT1 mutations; if normal, consider testing NPHS2 and PLCE1.

Medical Management

Management is long, with frequent remissions and recurrences. Corticosteroid therapy is daily induction for 6 weeks, then alternate-day maintenance for another 6 weeks. Diuretics help symptomatic edema; loop diuretics such as furosemide (starting at 1-2 mg/kg/d) may improve edema but must be used carefully, since plasma volume contraction may already exist and hypovolemic shock has occurred with overly aggressive therapy. Home monitoring matters: train all patients and parents to check first-morning urine protein by dipstick, which also tracks response to steroids. Keep a sodium-restricted diet while edematous and until proteinuria remits; during severe edema, careful modest fluid restriction may be appropriate with close monitoring for excessive intravascular volume depletion. A normal activity plan is recommended.

Pharmacologic Management

Prednisone is first-line for children, with other immunosuppressives reserved for nonresponders or frequent relapsers. All glucocorticoids work, but prednisone or prednisolone is most common; the exact mechanism in nephrotic syndrome is unknown. Diuretics promote renal excretion of water and electrolytes and treat edema or ascites from sodium and water retention. Plasma protein supplements diuresis in edema by raising oncotic pressure and shifting fluid from interstitial tissues. Immunosuppressive agents are used for steroid-resistant or frequently relapsing disease.

Nursing Management

Nursing Assessment

Observe for edema on physical exam. Weigh the child and record abdominal measurements as a baseline. Obtain vital signs, including blood pressure. Note pitting edema and swelling around the eyes, ankles, and other dependent parts. Inspect the skin for pallor, irritation, or breakdown, and examine the scrotal area of the male child for swelling, redness, and irritation.

Nursing Diagnoses

Major diagnoses are excess fluid volume related to fluid accumulation in tissues and third spaces, risk for imbalanced nutrition (less than body requirements) related to anorexia, risk for impaired skin integrity related to edema, fatigue related to edema and disease process, risk for infection related to immunosuppression, deficient caregiver knowledge related to disease process and home care, and compromised family coping related to caring for a child with chronic illness.

Nursing Care Planning and Goals

Goals are relieving edema, improving nutritional status, maintaining skin integrity, conserving energy, and preventing infection.

Nursing Interventions

Monitor fluid intake and output accurately and document it; weigh the child at the same time daily on the same scale in the same clothing, and measure the abdomen daily at the umbilicus. Improve nutrition with a visually appealing, nutritious diet, asking the child and family which foods appeal; six small meals may raise total intake. Protect skin: inspect all surfaces regularly, turn and position every 2 hours, pad pressure points with pillows, separate overlapping skin surfaces with cotton gauze, bathe regularly, and use a light dusting of cornstarch for comfort. Conserve energy: bed rest is common during the edema stage, balance activity with rest, encourage rest when fatigued, and plan quiet age-appropriate activities. Prevent infection: shield the child from anyone with an infection (staff, family, visitors, other children), maintain handwashing and strict medical asepsis, and watch for early signs of infection.

Evaluation

Goals are met when edema is relieved, nutritional status improves, skin integrity is maintained, energy is conserved, and infection is prevented.

Documentation Guidelines

Document temperature and other assessment findings including vital signs, causative and contributing factors, the condition's impact on personal image and lifestyle, the plan of care and teaching plan, responses to interventions and teaching, attainment or progress toward outcomes, and modifications to the plan.