Nephrotic syndrome - causes, symptoms, treatment
Nephrotic syndrome - causes, symptoms, treatment
Nephrotic syndrome is a kidney disorder characterised by high levels of protein in the urine and swelling of body tissue. People of any age can be affected by nephrotic syndrome, although children aged between 18 months and four years are at increased risk.
Long-term nephrotic syndrome can lead to irreparable kidney damage leading to kidney failure, necessitating treatment with dialysis or, eventually, kidney transplant.
Long-term nephrotic syndrome can lead to irreparable kidney damage leading to kidney failure, necessitating treatment with dialysis or, eventually, kidney transplant.
General information
Nephrotic syndrome produces a collection of symptoms and signs that occur because tiny blood vessels (the glomeruli) in the kidneys do not function correctly. The syndrome is characterised by abnormally high levels of protein in the urine (particularly one called albumin) and abnormally low protein levels in the blood. This often leads to fluid retention in the body tissues causing swelling (oedema) and can be associated with high blood cholesterol levels and high blood pressure.
The glomeruli act as filters to remove waste products from the blood, which are then excreted from the body in the urine. In nephrotic syndrome these filters become defective and leak, allowing large quantities of protein to be lost in the urine. Protein in the blood acts to prevent water entering the body’s cells. Reduced blood protein levels cause water to leak into body tissue, causing swelling (oedema).
The glomeruli act as filters to remove waste products from the blood, which are then excreted from the body in the urine. In nephrotic syndrome these filters become defective and leak, allowing large quantities of protein to be lost in the urine. Protein in the blood acts to prevent water entering the body’s cells. Reduced blood protein levels cause water to leak into body tissue, causing swelling (oedema).
Causes
In Bangladesh, the most common cause of the nephrotic syndrome is glomerulonephritis - a group of kidney diseases where the glomeruli become inflamed. Malfunction of the immune system is thought to be the underlying cause of glomerulonephritis. For some reason the immune system attacks and damages the glomeruli. The three most common forms of glomerulonephritis causing nephrotic syndrome are:
- Minimal change nephropathy (also called minimal change disease).
- Membranous glomerulonephritis.
- Focal segmental glomerulonephritis.
There is also a form of the condition called congenital nephritic syndrome. This very rare form of the condition is genetic in origin and is present at birth. A wide range of other disorders (including diabetes mellitus and systemic lupus erythematosus) and factors such as toxins are less common causes of the nephrotic syndrome.
In children, nephrotic syndrome is normally detected between the age of 3 and 4 years old. The majority of children have the form of glomerulonephritis called minimal change nephropathy. This is where minimal or no glomerular abnormalities are noted by a microscope. This form of nephritic syndrome affects approximately 1 in 50,000 children. It can usually be successfully treated. In adults, membranous glomerulonephritis and focal segmental glomerulonephritis are the most common causes of the nephrotic syndrome.
In children, nephrotic syndrome is normally detected between the age of 3 and 4 years old. The majority of children have the form of glomerulonephritis called minimal change nephropathy. This is where minimal or no glomerular abnormalities are noted by a microscope. This form of nephritic syndrome affects approximately 1 in 50,000 children. It can usually be successfully treated. In adults, membranous glomerulonephritis and focal segmental glomerulonephritis are the most common causes of the nephrotic syndrome.
Signs and symptoms
Signs and symptoms include:
- Foamy urine
- Fatigue and weakness
- Swelling (oedema) around the eyes, hands, feet, and abdomen
- Loss of appetite
- Increased weight from fluid retention
- High protein levels in the urine and low protein levels in the blood
As the condition progresses there may be:
- Susceptibility to infectious diseases due to an impaired immune system
- An increased risk of blood clots (thrombosis)
- Muscle wasting (due to the loss of protein)
- Malnourishment
- Weakening of the bones
- Abnormally low or abnormally high blood pressure
- High blood cholesterol levels
- Kidney failure.
Diagnosis
A referral to a nephrologist (kidney specialist) may be required in order for a diagnosis to be made. The doctor will take samples of the blood and urine. If results show large amounts of protein in the urine and decreased amounts of protein in the blood, the doctor may recommend a kidney biopsy.
A kidney biopsy is where a tiny piece of kidney is removed by a needle and studied under a microscope. This procedure usually involves a one-day stay in hospital and is performed under a light general anesthetic or a local anaesthetic. Ultrasound scanning of the kidneys may also be performed.
A kidney biopsy is where a tiny piece of kidney is removed by a needle and studied under a microscope. This procedure usually involves a one-day stay in hospital and is performed under a light general anesthetic or a local anaesthetic. Ultrasound scanning of the kidneys may also be performed.
Treatment
Treatment will depend on the underlying cause of the condition and is aimed at alleviating symptoms and preventing complications.
Medications that reduce the effect of the immune system on the glomeruli are the mainstay of treatment. Medications to achieve this include steroids (eg: prednisone) and potent immune suppressant medications such as cyclosporine, cyclophosphamide, mycophenolate and rituximab. Medications to decrease high blood pressure and high blood cholesterol levels may also be prescribed.
The doctor may also recommend restricting fluid intake or may prescribe medications that increase the amount of urine produced (diuretics). These, along with bed rest, will help to reduce swelling. The doctor or a dietitian may recommend a special diet aimed at controlling the amount of protein and salt in the body. Overall, it is important to maintain good health and nutrition and to ensure other medical conditions are treated promptly.
Medications that reduce the effect of the immune system on the glomeruli are the mainstay of treatment. Medications to achieve this include steroids (eg: prednisone) and potent immune suppressant medications such as cyclosporine, cyclophosphamide, mycophenolate and rituximab. Medications to decrease high blood pressure and high blood cholesterol levels may also be prescribed.
The doctor may also recommend restricting fluid intake or may prescribe medications that increase the amount of urine produced (diuretics). These, along with bed rest, will help to reduce swelling. The doctor or a dietitian may recommend a special diet aimed at controlling the amount of protein and salt in the body. Overall, it is important to maintain good health and nutrition and to ensure other medical conditions are treated promptly.
