Nephrotic Syndrome

Nephrotic syndrome is a collection of symptoms because of damage to the blood vessels or glomeruli of the kidneys. It is a group of symptoms that occurs when your kidneys are not working properly. The clinical features that characterise the nephrotic syndrome consist of heavy proteinuria and hypoalbuminemia that result from alteration of the glomerular capillary wall. It is often associated with oedema and generalised hyperlipidemia.

 

Nephrotic syndrome is generally caused by damage to the clusters of tiny blood vessels or glomeruli of your kidneys. Healthy glomeruli retain the blood protein which is required to maintain an adequate amount of fluid in your body, whereas damaged glomeruli allow an unacceptable amount of protein to leave your body. There are several diseases and conditions which cause glomerular damage.

1. One of the most common causes of nephrotic syndrome in adults is focal segmental glomerulosclerosis.
2. Diabetes is also among the common causes of nephrotic syndrome. It can lead to kidney damage which is also known as diabetic nephropathy.
3. Membranous nephropathy is the result of thick membranes within the glomeruli.
4. Chronic inflammatory disease, for example, systemic lupus erythematosus, can lead to kidney damage.
5. In children, the most common cause of the nephrotic syndrome is minimal change disease. In this disease, an electron microscope is the only tool through which lesions are visible. Pronounced proteinuria is another symptom of this disease

Symptoms

1. Puffiness around the eyes, particularly in the morning.
2. Weight gain due to fluid retention
3. Foamy or frothy urine due to excess protein
4. Loss of appetite
5. Tiredness
6. Oedema is seen mostly in the feet and ankles.

Signs

1. Excess fluid in the body due to serum hypoalbuminemia
2. Fluid in the pleural cavity causes pleural effusion
3. Increase in the liver synthesis of cholesterol
4. An increase in the filtration of lipoprotein causes lipiduria or loss of lipids.

The diagnostic evaluation focuses on the identification of the underlying cause and on the role of renal biopsy. The initial investigation includes history, physical examination, and serum chemistry panel.

1. Patient history: 
   1. Identify any medication or exposure to toxins; any risk for viral infections such as hepatitis, or HIV, and any symptoms suggesting causes of oedema.
   2. Previous history of systemic lupus erythematosus, diabetes, or other systemic illness.
2. Urine-based tests: Creatinine ratio/Urine protein to be checked.
3. Blood-based tests:
   1. Serum creatinine: To rule out any acute renal failure or to assess glomerular filtration rate.
   2. Serum albumin: Assess the degree of hypoalbuminemia.
   3. Identify HIV by HIV screening test.
   4. Hepatitis serology panel: For identifying hepatitis B or hepatitis C.
   5. Undergoing serum or urine protein electrophoresis suggests amyloidosis or multiple myeloma.
4. Lipid-based tests: Lipid panel: Assess the degree of hyperlipidemia.
5. Others:
   1. To assess a person with nephrotic syndrome, imaging studies are usually not very helpful.
   2. Renal ultrasonography is prescribed in order to detect renal venous thrombosis. Renal venous thrombosis is characterized by haematuria or flank pain.
   3. Renal biopsy is often recommended for individuals with nephrotic syndrome. This is done to establish the underlying pathology, and assess/confirm the diagnosis of diseases, such as amyloidosis or systemic lupus erythematosus.
6. Clinical evaluation:
   1. Chest examination: Pleuritic friction and pericardial rubs may be a sign of uraemia.
   2. Abdominal examination: Visible fullness of the upper abdomen is an unusual finding of kidney disease.
   3. Skin examination: Pallor due to anaemia, ecchymoses due to platelet dysfunction.

Treatment for early stages

1. Medications such as diuretics can help maintain the balance of fluids in your body, hence relieving the swelling.
2. Supplements of the hormone erythropoietin, added with iron, help in producing more red blood cells that might relieve tiredness and weakness associated with anaemia.
3. Medications called statins might be recommended to lower your cholesterol levels.
4. Supplements like vitamin D and calcium can help prevent weak bones and lower your risk of fracture.
5. Your doctor might recommend medications to lower blood pressure, commonly angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers.
6. A dietitian can suggest ways to lower your protein intake while still eating a healthy diet.

Treatment for end-stage kidney disease

1. Dialysis. Dialysis is an artificial way of removing the extra fluid and waste products from your blood when the kidneys are damaged and can longer do this. 
2. In Hemodialysis, a machine filters the excess fluids and waste from your blood.
3. During Peritoneal Dialysis, a thin tube is inserted into your abdomen, and it fills your abdominal cavity with a dialysis solution that absorbs the excess fluids and waste. After some time, the dialysis solution drains from your body, carrying the waste with it. 
4. Kidney transplant. A kidney transplant involves a surgical procedure in which your doctor will place a healthy kidney from a donor into your body. Transplanted kidneys can come from either living or deceased ones.

Diseases and conditions that increase the risk of nephrotic syndrome are: 

1. Diabetes
2. Amyloidosis
3. Lupus systemic erythematosus
4. Reflux nephropathy
5. HIV, the virus's antigens, provoke an obstruction in the glomeruli capillary's lumen that alters the normal kidney function.
6. Hepatitis B, Hepatitis C
7. Malignancy, especially Hodgkin's lymphoma
8. Drugs such as (corticosteroids, NSAIDs, gold salts)
9. Protozoal infections like malaria
10. Bacterial infections, e.g., syphilis and leprosy
11. Hypertension
12. Obesity
13. Kidney disease
14. Congenital nephrotic syndrome, a genetic disorder
15. Sjogren's syndrome

Disease progression

Stage 1 : Kidney function/GFR - >90%

               Description: Normal or high function

Stage 2 : Kidney function/GFR - 60-89%

               Description: Mildly decreased function

Stage 3 : Kidney function/GFR - 30-59%

               Description: Mild to moderately decreased function

Stage 4 : Kidney function/GFR - 15-29%

                Description: Severely decreased function

Stage 5 : Kidney function/GFR - <15%

                Description: Kidney failure

If you experience symptoms like:

1. Puffy eyes or face, especially in the morning.
2. Swelling in your legs or ankles, feet.
3. Foamy or bubbly urine
4. Unexpected weight gain
5. Excess levels of cholesterol and fats in your blood.
6. Generalized oedema
7. Changes in urination (discomfort or infrequency)
8. Additional symptoms like headache, cough, fever, etc.