Pediatrics Case 31: Nephrotic Syndrome

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Conjunctivitis

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is inflammation of the conjunctiva. The conjunctiva becomes red and swollen, with some mild lid edema. There is also associated watery or purulent drainage depending on the type of conjunctivitis.Conjunctivitis can be caused by allergies, chemical irritation or infection.

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Viral vs. Bacterial conjuctivitis:

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Children younger than 6 are more likely to have a bacterial than a viral infection. Both produce conjunctival redness and itching, but bacterial conjunctivitis is more likely to be bilateral and to cause a purulent exudate with eyelids crusted closed upon waking. Both viral and bacterial tend to begin in one eye, but both may spread to the other eye. Adenovirus conjunctivitis frequently becomes bilateral and may be associated with pharyngitis and pre-auricular lymphadenopathy.

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The most common diagnoses to explain bilateral periorbital swelling are...

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conjunctivitis, URI and sinusitis. Allergic rhinitis is possible, but is a less likely cause.

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anasarca:

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medical condition characterised by widespread swelling of the skin due to effusion of fluid into the extracellular space.[1]

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An S3 gallop

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if present, would indicate on overloaded left ventricle, and would suggest congestive heart failure.

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Post-streptococcal Glomerulonephritis

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may occur after a streptococcal infection of either the throat or the skin.Treating strep throat with antibiotics will prevent the complication of Acute Rheumatic Fever, but it will not prevent the occurrence of PSGN. When a patient presents with tea-colored urine, it is important to ask about recent throat and skin infections. Diagnosis of PSGN can be supported with elevated Antistreptolysin O (ASO) titer, Streptozyme, anti-DNase B, or low C3.

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interstitial nephritis

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In children, interstitial nephritis is rare and usually caused by a medication such as Methicillin (no longer available in the United States), NSAIDs, penicillins, cephalosporins, rifampin, sulfonamides, or infection.

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Why is there diffuse edema in people with nephrotic syndrome?

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The fall in serum albumin concentration due to proteinuria results in a lowering of plasma colloid osmotic pressure. Therefore, fluid moves out of the vascular compartment into the interstitial space, and hypovolemia develops. Intravascular hypovolemia activates volume sensors and extrarenal neurohumoral and hemodynamic mechanisms that signal the kidney to retain salt and water through increased renin-angiotensin-aldosterone, catecholamines, antidiuretic hormone and depressed natriuretic factor leading to edema.

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An increase in interstitial fluid causing anasarca will occur if there is:

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- An increase in capillary hydrostatic pressure (as in congestive heart failure);
- A decrease in plasma protein concentration (as in nephrotic syndrome);
- Increased vascular permeability to proteins (as occurs in allergic reactions);
- Impaired lymphatic flow

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What does a normal CBC mean for a patient with nephrotic syndrome?

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However, even with a normal CBC, patients who have nephrotic syndrome are predisposed to venous thrombosis involving any of the large veins of the body, including the sagittal sinus and pulmonary arteries. Renal venous thrombosis is a relatively frequent complication in adults. Factors predisposing to venous thrombosis include urinary loss of proteins that inhibit coagulation (antithrombin III) or increased fibrinolysis, destabilization of platelets by hyperlipidemia, increased fibrinogen levels, and increased blood viscosity due to a rise in the hematocrit (especially if diuretics are used without albumin replacement). Intense, prolonged therapeutic use of corticosteroids, which are themselves thrombogenic, adds to the risk of thrombosis for a patient.

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Complement levels, especially C3 and C4,

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are useful to determine if there is a secondary cause for nephrotic syndrome such as a collagen vascular disease (systemic lupus erythematosis (SLE), a primary renal disease other than minimal change disease (membranoproliferative glomerulonephritisor) or an infectious etiology such as a recent group A strep infection (post streptococcal glomerulonephritis). Levels will be decreased in secondary glomerulonephritis or nephrotic syndrome due to these etiologies.

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Treatment for Nephrotic syndrome:

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However, treatment with IV furosemide alone is very dangerous in nephrotic syndrome because it could lower intravascular volume to dangerously low levels, precipitating shock and increasing risk for venous thrombosis. It should be preceded by an albumin infusion. The albumin infusion should be followed with 1 to 2 mg/kg of furosemide to promote diuresis. Katie also should be started on corticosteroids. Current guidelines recommend sodium restriction to a level of 1500-2000 mg daily

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focal segmental glomerulonephritis (FSGS)

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On biopsy most of the glomeruli appear normal or show mesangial proliferation, while others, especially juxtamedullar glomeruli, show segmental scarring in one or more lobules. The disease frequently leads to involvement of all glomeruli, and to end-stage renal failure in most patients. Only about 20% of patients respond to steroid or cyclophosphamide therapy. Unfortunately even with transplantation the disease may recur in the transplanted kidney.

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Infectious complications of patients with primary nephrotic syndrome:

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During a nephrotic syndrome relapse with steroid use, the pt. is at high risk for infection. This increased risk of bacterial infection may be due to the following: decreased immunoglobulin levels, edema fluid acting as a culture medium, protein deficiency, decreased leukocyte bactericidal activity, immunosuppression from steroids, decreased splenic perfusion due to hypovolemia, and urinary loss of a complement factor (properdin factor B) that opsonizes certain bacteria.
Spontaneous peritonitis is one of the most frequent complications, with an insidious presentation of fever and minimal other findings. Untreated it can lead to overwhelming sepsis and death, especially while the patient is on steroids.
Streptococcus pneumoniae is the most common pathogen causing peritonitis, but gram-negative bacteria can also be found. Pneumonia, cellulitis, and urinary tract infections are other common infectious complications of nephrotic syndrome.
A high index of suspicion, prompt evaluation (including cultures of blood and peritoneal fluid), and early treatment with broad spectrum antibiotics to cover gram-positive and gram-negative organisms are critical to preventing life-threatening illness. When in remission, The pt. should receive polyvalent pneumococcal vaccine. If not already immunized she should receive two doses of varicella vaccine, and she should get an influenza vaccine annually.