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Nephrotic Syndrome

Updated : March 11, 2023





Background

Nephrotic syndrome is a kidney disorder characterized by high levels of protein in the urine, low levels of protein in the blood, and swelling of parts of the body due to fluid retention. It is usually caused by damage to the tiny blood vessels in the kidneys, which filter waste out of the blood and pass it out of the body in urine.

It is usually caused by damage to the tiny blood vessels in the kidneys, which filter waste out of the blood and pass it out of the body in urine. It is caused by increased permeability of the damaged basement membrane in the renal glomerulus due to an intrinsic renal disease or an underlying condition such as, systemic lupus, diabetes, neoplasia, or certain drug use.

Epidemiology

It is estimated that approximately 3 out of every 100,000 people in the United States have Nephrotic Syndrome. The prevalence of Nephrotic Syndrome is highest in children under the age of 10, with an estimated incidence of 20-40 cases per 100,000 children under the age of 5.

The mortality rate of varies depending on the underlying cause and severity of the condition. Approximately half of all Nephrotic syndrome patients reach complete remission, and long-term survival is possible. However, those with severe or end-stage kidney disease may have a poorer prognosis.

Anatomy

Pathophysiology

Nephrotic syndrome is caused by damage to the glomeruli. This damage leads to the leakage of proteins in the urine, which can cause proteinuria, edema, and hypoalbuminemia. The exact cause of glomerular damage is not known, but it is thought to be caused by autoimmune disorders, infections. The primary pathophysiology of nephrotic syndrome involves the loss of proteins, most notably albumin, in the urine. Enhanced glomerular permeability causes albuminuria, ultimately leading to hypoalbuminemia.

This reduction in plasma colloid osmotic pressure then causes an increase in transcapillary fluid filtration, leading to edema. The progress of edema is due to a decrease in intravascular oncotic pressure and an increase in hydrostatic capillary pressure, which results in an excessive amount of fluid being filtered from the vascular compartment into the interstitium. This excess fluid exceeds the maximal lymphatic flow, leading to a decrease in plasma volume and an increase in water and sodium retention in the kidneys.

Due to the damage to the glomeruli, they cannot retain proteins, causing them to be filtered out into the urine. As a result, the body cannot maintain the fluid balance in the bloodstream, leading to edema. Additionally, the loss of albumin in the urine causes hypoalbuminemia, leading to complications such as anemia and thrombosis. Long-term complications of nephrotic syndrome can include high cholesterol, kidney failure, and an increased risk of infection.

Etiology

Nephrotic syndrome is primarily caused by intrinsic kidney diseases, such as minimal-change nephropathy, membranous nephropathy, and focal glomerulosclerosis, as well as systemic diseases, such as diabetes mellitus, amyloidosis, and lupus erythematosus.

In some cases, it may be caused by genetic mutations in podocyte proteins, such as nephrin, podocin, or the cation channel 6 protein. Additionally, nephrotic syndrome can sometimes be triggered by an episode of infectious diseases of the upper respiratory tract, allergic reaction, insect bite, vaccination, or drugs of abuse, such as heroin.

Genetics

Prognostic Factors

The prognosis of the nephrotic syndrome depends on the underlying cause. In general, nephrotic syndrome can be managed with medications and lifestyle changes, and most people with the condition can lead a normal life. However, some underlying causes can cause permanent kidney damage and lead to end-stage renal disease.

Clinical History

Clinical History

The first sign in children is typically facial swelling. This can then be followed by swelling of the entire body. Adult patients may present with dependent edema. Other potential symptoms include frothy urine, tiredness, loss of appetite, and deep venous thrombosis (DVT) of the calf veins or a pulmonary embolus.

Depending on the cause of the nephrotic syndrome, additional features may be present in the patient’s history. For example, the recent initiation of NSAID therapy could suggest such drugs as the cause, while a more than ten year history of diabetes mellitus with symptomatic neuropathy could indicate diabetic nephropathy.

Physical Examination

Physical Examination

Edema is the hallmark symptom of nephrotic syndrome and is often first noted around the eyes and legs, eventually becoming generalized and leading to increased weight and the development of ascites or pleural effusions.

While hematuria and hypertension are not always found in those with nephrotic syndrome, they are more commonly seen in nephritic syndrome.

Depending upon the underlying cause of the nephrotic syndrome, there may be other physical findings such as diabetic retinopathy in those with longstanding diabetes mellitus or anemia, hypertension, or both in those with impaired renal function.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Differential Diagnoses

  • Hepatocellular cirrhosis
  • Budd-Chiari Syndrome
  • Hereditary angioneurotic edema
  • Diabetic nephropathy
  • Iga nephropathy
  • Minimal change disease

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

 
 

prednisolone 

The first four weeks of treatment is 60 mg/m²/day or 2 mg/kg/day taken orally, divided into doses every 8 hours until urine is protein-free for three consecutive days. The treatment should not exceed 28 days, and the maximum daily dose should not exceed 80 mg
Subsequent four weeks: 40 mg/m²/day or 1-1.5 mg/kg/day orally every alternate day



 

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK470444/

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Nephrotic Syndrome

Updated : March 11, 2023




Nephrotic syndrome is a kidney disorder characterized by high levels of protein in the urine, low levels of protein in the blood, and swelling of parts of the body due to fluid retention. It is usually caused by damage to the tiny blood vessels in the kidneys, which filter waste out of the blood and pass it out of the body in urine.

It is usually caused by damage to the tiny blood vessels in the kidneys, which filter waste out of the blood and pass it out of the body in urine. It is caused by increased permeability of the damaged basement membrane in the renal glomerulus due to an intrinsic renal disease or an underlying condition such as, systemic lupus, diabetes, neoplasia, or certain drug use.

It is estimated that approximately 3 out of every 100,000 people in the United States have Nephrotic Syndrome. The prevalence of Nephrotic Syndrome is highest in children under the age of 10, with an estimated incidence of 20-40 cases per 100,000 children under the age of 5.

The mortality rate of varies depending on the underlying cause and severity of the condition. Approximately half of all Nephrotic syndrome patients reach complete remission, and long-term survival is possible. However, those with severe or end-stage kidney disease may have a poorer prognosis.

Nephrotic syndrome is caused by damage to the glomeruli. This damage leads to the leakage of proteins in the urine, which can cause proteinuria, edema, and hypoalbuminemia. The exact cause of glomerular damage is not known, but it is thought to be caused by autoimmune disorders, infections. The primary pathophysiology of nephrotic syndrome involves the loss of proteins, most notably albumin, in the urine. Enhanced glomerular permeability causes albuminuria, ultimately leading to hypoalbuminemia.

This reduction in plasma colloid osmotic pressure then causes an increase in transcapillary fluid filtration, leading to edema. The progress of edema is due to a decrease in intravascular oncotic pressure and an increase in hydrostatic capillary pressure, which results in an excessive amount of fluid being filtered from the vascular compartment into the interstitium. This excess fluid exceeds the maximal lymphatic flow, leading to a decrease in plasma volume and an increase in water and sodium retention in the kidneys.

Due to the damage to the glomeruli, they cannot retain proteins, causing them to be filtered out into the urine. As a result, the body cannot maintain the fluid balance in the bloodstream, leading to edema. Additionally, the loss of albumin in the urine causes hypoalbuminemia, leading to complications such as anemia and thrombosis. Long-term complications of nephrotic syndrome can include high cholesterol, kidney failure, and an increased risk of infection.

Nephrotic syndrome is primarily caused by intrinsic kidney diseases, such as minimal-change nephropathy, membranous nephropathy, and focal glomerulosclerosis, as well as systemic diseases, such as diabetes mellitus, amyloidosis, and lupus erythematosus.

In some cases, it may be caused by genetic mutations in podocyte proteins, such as nephrin, podocin, or the cation channel 6 protein. Additionally, nephrotic syndrome can sometimes be triggered by an episode of infectious diseases of the upper respiratory tract, allergic reaction, insect bite, vaccination, or drugs of abuse, such as heroin.

The prognosis of the nephrotic syndrome depends on the underlying cause. In general, nephrotic syndrome can be managed with medications and lifestyle changes, and most people with the condition can lead a normal life. However, some underlying causes can cause permanent kidney damage and lead to end-stage renal disease.

Clinical History

The first sign in children is typically facial swelling. This can then be followed by swelling of the entire body. Adult patients may present with dependent edema. Other potential symptoms include frothy urine, tiredness, loss of appetite, and deep venous thrombosis (DVT) of the calf veins or a pulmonary embolus.

Depending on the cause of the nephrotic syndrome, additional features may be present in the patient’s history. For example, the recent initiation of NSAID therapy could suggest such drugs as the cause, while a more than ten year history of diabetes mellitus with symptomatic neuropathy could indicate diabetic nephropathy.

Physical Examination

Edema is the hallmark symptom of nephrotic syndrome and is often first noted around the eyes and legs, eventually becoming generalized and leading to increased weight and the development of ascites or pleural effusions.

While hematuria and hypertension are not always found in those with nephrotic syndrome, they are more commonly seen in nephritic syndrome.

Depending upon the underlying cause of the nephrotic syndrome, there may be other physical findings such as diabetic retinopathy in those with longstanding diabetes mellitus or anemia, hypertension, or both in those with impaired renal function.

Differential Diagnoses

  • Hepatocellular cirrhosis
  • Budd-Chiari Syndrome
  • Hereditary angioneurotic edema
  • Diabetic nephropathy
  • Iga nephropathy
  • Minimal change disease

prednisolone 

The first four weeks of treatment is 60 mg/m²/day or 2 mg/kg/day taken orally, divided into doses every 8 hours until urine is protein-free for three consecutive days. The treatment should not exceed 28 days, and the maximum daily dose should not exceed 80 mg
Subsequent four weeks: 40 mg/m²/day or 1-1.5 mg/kg/day orally every alternate day



https://www.ncbi.nlm.nih.gov/books/NBK470444/

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