About Nephrotic Syndrome in Children

Overview

Nephrotic syndrome is a common kidney disorder in children characterized by specific symptoms, including:

  • Severe edema (swelling) due to fluid retention
  • Significant proteinuria (excessive protein in the urine)
  • Hypoalbuminemia (low levels of albumin, a protein in the blood)
  • Hyperlipidemia (high levels of lipids, such as cholesterol and triglycerides, in the blood)

It is important to note that nephrotic syndrome can have different forms based on the age of onset, such as congenital, infantile, and childhood-onset. Most children with nephrotic syndrome respond well to steroid treatment. However, about 20-30% of children may not respond to steroids and may progress to renal failure. In some cases, nephrotic syndrome can be caused by genetic mutations. It is important to work with a healthcare professional for proper diagnosis and management of nephrotic syndrome in children.

Causes and Risk Factors

The pathophysiologic causes of Nephrotic Syndrome in children include:

  • Primary nephrotic syndrome: Four types of kidney disease can cause primary nephrotic syndrome in children, including minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), and membranous nephropathy (MN). MCD is the most common cause in young children, while FSGS can be caused by genetic variants. MN is an autoimmune disease that affects the kidneys' glomerular basement membrane.
  • Secondary nephrotic syndrome: This can be caused by systemic diseases like IgA vasculitis and lupus, infections such as hepatitis B and C, HIV, and malaria, blood diseases like leukemia and lymphoma, and certain medications.
  • Congenital nephrotic syndrome: This occurs in newborns and infants due to genetic variants or infections present at or before birth.

Non-modifiable risk factors for Nephrotic Syndrome in children are factors that cannot be changed. These include:

  • Genetic factors: Certain genetic variants can increase the risk of developing Nephrotic Syndrome, especially in cases of congenital nephrotic syndrome.

Modifiable risk factors are factors that can be influenced or changed. For Nephrotic Syndrome in children these are not well-defined. However, some factors that may contribute to kidney disease development include:

  • Infections: Some infections can lead to secondary nephrotic syndrome. Preventing and treating infections promptly may help reduce the risk.
  • Medications: Certain medications have been associated with secondary nephrotic syndrome. It's important to follow proper medication guidelines and discuss any concerns with a healthcare professional.

Remember, these are general descriptions, and individual cases may vary. It's essential to consult with your healthcare professional for personalized advice and guidance.

Symptoms

The most common early symptoms of Nephrotic Syndrome in children include:

  • Swelling around the eyes, which is usually more noticeable in the morning and can be mistaken for seasonal allergies.
  • Swelling in the lower legs, feet, abdomen, hands, face, or other parts of the body.
  • Foamy urine
  • Fatigue

As Nephrotic Syndrome progresses or becomes more severe, additional symptoms may occur. These can include:

  • Blood in the urine
  • Loss of appetite
  • Muscle cramps
  • Diarrhea or nausea

It's important to note that some children with Nephrotic Syndrome may also experience high blood pressure and an increased susceptibility to infections. Additionally, high blood cholesterol levels are commonly observed in children with this condition.

Diagnosis

To diagnose nephrotic syndrome in children, health care professionals commonly perform the following examinations, tests, and procedures:

  • Medical and family history: Gathering information about the child's medical background and family history.
  • Physical exam: A thorough examination of the child's body to assess symptoms and physical signs.
  • Urine tests: Analyzing urine samples to check for elevated levels of protein.
  • Blood tests: Measuring levels of albumin, cholesterol, and other blood components to assess kidney function and identify underlying diseases.
  • Ultrasound of the kidney: Using sound waves to create images of the kidneys and evaluate their structure.

Additional examinations, tests, and procedures to determine the stage or severity of nephrotic syndrome in children may include:

  • Kidney biopsy: Taking a small sample of kidney tissue to examine it under a microscope for microscopic changes.
  • Genetic testing: Assessing genetic factors that may contribute to nephrotic syndrome.

It's important to note that not all children with nephrotic syndrome will require a kidney biopsy. This procedure is typically reserved for those with complex disease, low kidney function, or who are 12 years old or older. The child's response to treatment with steroids is often used as an indicator of overall prognosis.

Remember, always consult with a health care professional for personalized advice and guidance regarding examinations, tests, and procedures for diagnosing and determining the severity of nephrotic syndrome in children.

Treatment Options

The goals of treatment for Nephrotic Syndrome in children are to:

  • Reduce proteinuria: Medications called corticosteroids are commonly used to reduce the amount of protein passed into the urine. These medicines suppress the immune system and decrease swelling, leading to a decrease in proteinuria. Medications such as angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) can also help reduce protein loss in the urine, which is a hallmark of Nephrotic Syndrome.
  • Control edema: Corticosteroids also help reduce swelling by decreasing fluid retention in the body. Diuretics, also known as water pills, can help the kidneys remove excess fluid from the body, reducing swelling.
  • Achieve remission: The primary goal is to achieve remission, where symptoms improve or disappear completely. Corticosteroids are effective in inducing remission in most children with primary Nephrotic Syndrome. If symptoms return (relapse), a shorter course of corticosteroids may be prescribed until remission is achieved again.
  • Manage complications: Health care professionals may prescribe medications to manage specific complications associated with Nephrotic Syndrome, such as antibiotics for infections or other medicines to treat underlying causes.
  • Minimize side effects: Long-term use of corticosteroids can cause side effects, such as impaired growth, obesity, high blood pressure, eye problems, and bone loss. Health care professionals aim to minimize these side effects by using the lowest effective dose for the shortest duration possible.

It's important to note that treatment approaches may vary depending on the type and cause of Nephrotic Syndrome (primary, secondary, or congenital). Health care professionals may also recommend health behavior changes, such as a low-sodium diet and regular exercise, to support overall kidney health.

Remember, treatment decisions should always be made in consultation with a health care professional who can provide personalized advice based on the individual's condition and needs.

Medication dosing may be affected by many factors. Check with your health care professional about dosing for your individual situation. Other side effects can occur. Check with your health care professional or read the information provided with your medication for additional side effect information.