Nephrotic syndrome

The nephrotic syndrome (nephrosis) is a clinically defined state characterized by proteinuria, hypoalbuminemia, hyperlipidemia and edema. It is sometimes accompanied by hematuria, hypertension and reduced glomerular filtration rate (GFR).

The nephrotic syndrome is the clinical manifestation of a large number of glomerular disorders. When associated with primary glomerular diseases, it is termed primary nephrotic syndrome. When the nephrotic syndrome occurs as part of a recognized systemic disease or result from some evident cause, it is termed secondary nephrotic syndrome. These may include systemic collagen vascular disorders, infections, drugs and toxins, allergies, cardiovascular disorders and malignancies.

Congenital nephrotic syndrome, a hereditary form of nephrotic syndrome, is present in infancy. Proteinuria and edema are significant early findings, often immediately after birth. The disease does not respond well to therapy. Death usually occurs before the age of 2 years.

Pathophysiology

The children affected with nephrotic syndrome, 75 to 80 percent cause is minimal change nephrotic syndrome (MCNS).  The cause of MCNS remain unclear. 

Causes of nephrotic syndrome

A. Primary renal causes:

• Minimal change nephropathy
• Mesangial proliferation
• Focal glomerulosclerosis
• Immune complex glomerulonephritis:- Membranoproliferative glomerulonephritis, Acute poststreptococcal glomerulonephritis, and Membranous nephrosis.
• Congenital nephrosis

B. Systemic Causes:

• Infections
• Toxins: Mercurials, Bismuth, Gold, Penicillamine, Gold etc.
• Allergies: Bee sting, Inhaled pollens, Food allergy, Poison oak, and Serum sickness.
• Malignancies: Hodgkin disease, Leukemia.
• Cardiovascular: Renal vein thrombosis, Sickle cell disease, and Passive congestive heart failure etc.
• Others: Multiple myeloma, Anaphylactoid purpura, Amyloidosis and Systemic lupus erythematosus etc.

Clinical features 

• Child gains weight
• Periorbital and facial edema most prominent in morning.
• Leg, ankle, labial or scrotal edema.
• Urine output decreases; urine dark and frothy
• Massive proteinuria
• Blood pressure normal or slightly decreased
• Hypoproteinemia and elevated serum lipid level
• Lethargy, anorexia and pallor
• Ascites: Fluid in abdominal cavity
• Some children may be admitted with massive edema (anasarca), with respiratory distress and diarrhea due to edema of the intestinal mucosa.
  

Diagnosis

The child with nephrosis should have urine analysis, complete blood count and evaluation of serum electrolytes, calcium, phosphorus, blood urea nitrogen, creatinine, total protein, albumin, globulin, cholesterol, triglycerides.

Proteinuria is reflected by urinary excretion of protein. Dipstick result are often 4+. The urine specific gravity may be high and proportionate to the amount of protein concentration.

The serum cholesterol may be elevated and serum sodium is generally low. A renal biopsy my be indicated if the child does not respond to initial steroid therapy.

Treatment

• The goal of medical management is reduction of protein excretion. Prednisone is the drug of choice. Proteinuria should disappear within first weeks of therapy and negative dipsticks testing for 2 consecutive days is considered response to treatment.
• A regular diet without added salt may be prescribed if the child is in remission; sodium is restricted during periods of massive edema.
• Water restriction may be indicated if decreasing sodium does not control marked edema.
• Diuretics and salt-poor albumin may be prescribed in severe edema.
• Nurse closely monitor vital signs, intake and output and daily weights of child.
• Monitor for edema.
• Providing emotional support and education for all family members.
• Nurse prevent skin breakdown due to edema.
• Monitor the side effects of medications.