Nephrotic syndrome

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Nephrotic syndrome
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Edema, proteinuria, hypoalbuminemia, hyperlipidemia
Complications	Infection, thrombosis, acute kidney injury, chronic kidney disease
Onset	Any age, but often in children and young adults
Duration	Variable
Types	N/A
Causes	Minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, diabetic nephropathy, lupus nephritis
Risks	Diabetes mellitus, lupus, infections, medications
Diagnosis	Urinalysis, blood test, kidney biopsy
Differential diagnosis	Nephritic syndrome, acute kidney injury, chronic kidney disease
Prevention	N/A
Treatment	Corticosteroids, immunosuppressants, ACE inhibitors, diuretics, dietary modifications
Medication	N/A
Prognosis	Variable; depends on underlying cause and response to treatment
Frequency	3 per 100,000 children per year
Deaths	N/A

Nephrotic Syndrome (NS) is a clinical disorder characterized by marked proteinuria, hypoalbuminemia, hyperlipidemia, and edema. The syndrome is typically linked to damage to the clusters of tiny blood vessels in the kidneys that filter waste and excess water from the blood, known as the glomeruli. It is often associated with glomerulonephritis or can arise as a complication of various systemic diseases.

Facial edema

Glomerulus (PAS stain)

Diabetic glomerulosclerosis (PAM stain)

Diagram of the kidney

Urine sample

Ultrasonography of kidney with nephrotic syndrome

Pathophysiology of Nephrotic Syndrome

In Nephrotic Syndrome, the glomeruli are damaged, impairing their ability to filter blood properly. This leads to a loss of protein, particularly albumin, in the urine (proteinuria). As albumin helps keep fluid in the bloodstream, its deficiency results in fluid leaking into surrounding tissues, causing edema. Hyperlipidemia in NS is due to both increased production and decreased clearance of lipoproteins by the liver. The mechanisms behind these changes are complex and not fully understood.

Causes of Nephrotic Syndrome

Nephrotic Syndrome can result from various diseases that damage the kidneys, including:

• Glomerulonephritis: An inflammation of the glomeruli, often immune-related.
• Diabetic nephropathy: A common complication of diabetes and a leading cause of NS in adults.
• Lupus nephritis: An autoimmune disease where the body's immune system targets its own kidney cells.
• Amyloidosis: A disease that causes abnormal proteins to accumulate in organs and tissues.
• HIV-associated nephropathy (HIVAN): Kidney disease in patients with HIV infection.

Diagnosis of Nephrotic Syndrome

Diagnosis is typically based on clinical signs and symptoms, urinalysis demonstrating heavy proteinuria, and blood tests showing hypoalbuminemia and hyperlipidemia. Further investigation to determine the underlying cause may include renal ultrasound and kidney biopsy.

Management of Nephrotic Syndrome

Management aims to control symptoms, minimize complications, and treat the underlying cause:

• Edema Control: Diuretics are used to remove excess fluid.
• Proteinuria Reduction: Drugs such as ACE inhibitors or ARBs can decrease proteinuria.
• Hyperlipidemia Control: Statins may be used to lower cholesterol levels.
• Underlying Cause Treatment: Disease-specific treatments, such as immunosuppressive therapy for glomerulonephritis or tighter glucose control for diabetic nephropathy, can be instituted.

Prognosis and Complications

The prognosis of Nephrotic Syndrome varies widely depending on the underlying cause, age of the patient, response to treatment, and presence of other health conditions. Some patients may experience spontaneous remission, while others may progress to chronic kidney disease or end-stage renal disease requiring dialysis or kidney transplantation. Complications of Nephrotic Syndrome can include:

• Thromboembolism: The hypercoagulable state in Nephrotic Syndrome can increase the risk of developing blood clots.
• Infection: Loss of immunoglobulins in the urine can result in a weakened immune system, increasing susceptibility to infections.
• Acute kidney injury: This can occur due to severe fluid volume depletion.
• Malnutrition: Loss of proteins and other nutrients in the urine can lead to malnutrition.
• Cardiovascular disease: Hyperlipidemia can contribute to atherosclerosis and other heart diseases.

See also

• Glomerulonephritis
• Proteinuria
• Edema
• Hypoalbuminemia
• Hyperlipidemia

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