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Chapter 207: Nephrotic syndrome
207
Chapter 207 Nephrotic syndrome

DEFINITION
Nephrotic syndrome is a renal disorder characterized by high

urine protein excretion (3.5g/1.73 m3/24 hr), peripheral
edema, and metabolic abnormalities (hypoalbuminemia, hypercholesterolemia).
PHYSICAL FINDINGS AND CLINICAL PRESENTATION
Periorbital edema (Fig. 2071)

Peripheral edema (Fig. 2072)
Ascites, anasarca
Hypertension
Pleural effusion
Xanthelasma (Fig. 2073)
Nails with Muehrckes lines (Fig. 2074)
Typically, patients present with severe peripheral edema,
exertional dyspnea, and abdominal fullness secondary to
ascites. There is a signicant amount of weight gain in most
patients.
CAUSE
Membranous glomerulonephritis is the most common

cause of nephrotic syndrome.
Fig 2071
Fig 2073
Periorbital edema in the early morning in a nephrotic child. The edema

resolves during the day under the inuence of gravity.
Xanthelasma in nephrotic syndrome. These prominent xanthelasma

developed within a period of two months in a patient with recent onset
of severe nephrotic syndrome and serum cholesterol 550 mg/dL (14.2
mmol/L).
(From Johnson RJ, Feehally J: Comprehensive Clinical Nephrology, 3rd ed.

St. Louis, Mosby, 2007.)

Fig 2072
Fig 2074
Nephrotic edema. Severe peripheral edema in nephrotic syndrome;

note the blisters caused by intradermal uid.
Muehrckes bands in nephrotic syndrome. The white band grew

during a transient period of hypoalbuminemia caused by the nephrotic
syndrome.


707
207
Section 8: Kidneys
Idiopathic (may be secondary to the following glomerular

diseases: minimal change disease [nail disease, lipoid nephrosis], focal segmental glomerular sclerosis, membranous nephropathy, membranoproliferative glomerular
nephropathy)
Associated with systemic diseases (diabetes mellitus, SLE,
amyloidosis). Amyloidosis and dysproteinemias should be
considered in patients older than 40 years.
Majority of children with nephrotic syndrome have minimal change disease (this form also associated with allergy,
nonsteroidals, and Hodgkins disease)
Focal glomerular disease: can be associated with HIV infection, heroin abuse. A more severe form of nephrotic syndrome associated with rapid progression to end-stage renal
failure within months can also occur in HIV seropositive
patients and is known as collapsing glomerulopathy.
Membranous nephropathy: can occur with Hodgkins lymphoma, carcinomas, SLE, gold therapy
Membranoproliferative glomerulonephropathy: often associated with upper respiratory infections
DIFFERENTIAL DIAGNOSIS
Other edema states (CHF, cirrhosis)

Primary renal disease (e.g., focal glomerulonephritis,
membranoproliferative glomerulonephritis)
Carcinoma, infections
Malignant hypertension
Polyarteritis nodosa
Serum sickness
Toxemia of pregnancy
LABORATORY TESTS
Urinalysis reveals proteinuria. The presence of hematuria,

cellular casts, and pyuria is suggestive of nephritic syndrome. Oval fat bodies (tubular epithelial cells with cholesterol esters) (Fig. 2075) are also found in the urine in patients with nephrotic syndrome.
24-hour urine protein excretion is 3.5 g/1.73 m3/24 hr.
Abnormalities of blood chemistries include serum albumin
3 g/dL, decreased total protein, elevated serum cholesterol, glucose, and azotemia.
Additional tests in patients with nephrotic syndromes depending on the history and physical examination are ANA,
serum and urine immunoelectrophoresis, C3, C4, CH50,
lactate dehydrogenase (LDH), liver enzymes, alkaline phosphatase, hepatitis B and C screening, and HIV.
IMAGING STUDIES
Ultrasound of kidneys
Chest x-ray
TREATMENT
Bed rest as tolerated, avoidance of nephrotoxic drugs, lowfat diet, uid restriction in hyponatremic patients; normal
protein intake unless urinary protein loss exceeds 10 g/24 hr
(some patients may require additional dietary protein to
prevent negative nitrogen balance and signicant protein
malnutrition).
Improved urinary protein excretion and serum lipid changes
have been observed with a low-fat soy protein diet providing 0.7 g of protein/kg/day. However, because of increased
risk of malnutrition, many nephrologists recommend normal protein intake.
Strict sodium restriction to help manage peripheral edema
Close monitoring of patients for development of peripheral
venous thrombosis and renal vein thrombosis because of
hypercoagulable state secondary to loss of antithrombin III
and other proteins involved in the clotting mechanism
Furosemide is useful for severe edema.
Use of ACE inhibitors to reduce proteinuria is generally indicated, even in normotensive patients.
Anticoagulant therapy should be administered as long
as patients have nephrotic proteinuria, an albumin level
20 g/L, or both.
The mainstay of therapy is treatment of the underlying disorder:
Minimal change disease generally responds to prednisone. Relapses can occur when corticosteroids are discontinued. In these individuals, cyclophosphamide and chlorambucil may be useful.
Focal and segmental glomerulosclerosis: corticosteroid
therapy is also recommended. However, response rate is approximately 35% to 40%, and most patients progress to
end-stage renal disease within 3 years.
Membranous glomerulonephritis: prednisone, may be
useful in inducing remission. Cytotoxic agents can be added
if there is poor response to prednisone.
Membranoproliferative glomerulonephritis: most patients are treated with steroid therapy and antiplatelet drugs.
Despite treatment, the majority of patients will progress to
end-stage renal disease within 5 years.
Fig 2075
Fat in the urine. A hyaline cast containing oval fat bodies, which are
tubular epithelial cells full of fat. Oval fat bodies often appear brown
in color.
(From Johnson RJ, Feehally J: Comprehensive Clinical Nephrology,
3rd ed. St. Louis, Mosby, 2007.)
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Chapter 207: Nephrotic syndrome

Chapter 207 Nephrotic syndrome

Nephrotic syndrome is a renal disorder characterized by high

PHYSICAL FINDINGS AND CLINICAL PRESENTATION

Periorbital edema (Fig. 2071)

Membranous glomerulonephritis is the most common

Periorbital edema in the early morning in a nephrotic child. The edema

Xanthelasma in nephrotic syndrome. These prominent xanthelasma

(From Johnson RJ, Feehally J: Comprehensive Clinical Nephrology, 3rd ed.

(From Johnson RJ, Feehally J: Comprehensive Clinical Nephrology, 3rd ed.

Nephrotic edema. Severe peripheral edema in nephrotic syndrome;

Muehrckes bands in nephrotic syndrome. The white band grew

(From Johnson RJ, Feehally J: Comprehensive Clinical Nephrology, 3rd ed.

(From Johnson RJ, Feehally J: Comprehensive Clinical Nephrology, 3rd ed.

Idiopathic (may be secondary to the following glomerular

Other edema states (CHF, cirrhosis)

Urinalysis reveals proteinuria. The presence of hematuria,

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