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Idiopathic Nephrotic Syndrome in Children: An Insight

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Idiopathic nephrotic syndrome is a rare disorder that involves excessive protein loss in urine.

Written by

Dr. Pallavi. C

Medically reviewed by

Dr. Yash Kathuria

Published At January 31, 2024
Reviewed AtJanuary 31, 2024

Introduction

Idiopathic nephrotic syndrome is an uncommon kidney disease that affects around five out of every 100,000 children globally. The disease is characterized by symptoms such as proteinuria (high protein levels in the urine), hypoalbuminemia (low albumin levels in the blood), and edema (swelling). Most kids with idiopathic nephrotic syndrome recover from their condition and do not have any long-term kidney issues.

What Is Idiopathic Nephrotic Syndrome?

Idiopathic nephrotic syndrome is characterized by an excessive amount of albumin, a protein, released into the urine by the body. It indicates damage to one or both kidneys. Numerous small blood vessel coils can be seen inside the kidneys. These are referred to as glomerulus. Urine is filtered by glomeruli, which remove particles from the blood. Nephrotic syndrome is the result of abnormal glomerular function.

A kid suffering from nephrotic syndrome might have:

  • Very high albumin (protein) levels in the urine.

  • Blood protein levels that are low.

  • Body-wide tissue edema, particularly in the abdomen (ascites).

  • Gaining weight due to drinking too much liquid.

  • Elevated blood cholesterol level.

  • Reduced urination.

The most prevalent type of nephrotic syndrome is minimal change nephrotic syndrome (MCNS). A child with MCNS may experience episodes of worsening symptoms or relapses. However, with time, the disease can be controlled. Rarely, a youngster may require dialysis due to renal failure.

What Are the Causes of Idiopathic Nephrotic Syndrome?

The main cause of idiopathic nephrotic syndrome is unknown. Hence, the term idiopathic.

In rare instances, during the first week of life, congenital nephrotic syndrome may develop. A recessive autosomal gene inherits it. Boys and girls are, therefore equally impacted. Each carrier parent passes on one copy of the gene to their offspring. With every pregnancy, carrier parents have a 1 in 4 risk of giving birth to a kid who has this condition. This kind of nephrotic syndrome has an extremely bad prognosis.

What Are the Signs and Symptoms of Idiopathic Nephrotic Syndrome in Children?

Children may experience symptoms in different ways.

They may consist of:

  • Excessive fatigue.

  • An all-around uncomfortable feeling.

  • Reduced desire to eat.

  • Gaining weight and edema in the face.

  • Discomfort or swelling in the belly.

  • Urine with foam.

  • Body accumulation of fluid (edema).

  • Accumulation of fluid in the abdomen (ascites).

  • Light-colored nail beds.

  • Dull-looking hair.

  • Cartilage in the ears is less rigid.

  • Sensitivities or intolerances to certain foods.

How to Diagnose Idiopathic Nephrotic Syndrome?

The medical professional will inquire about the symptoms and medical background of the child. The health history of the family may also be questioned by him or her. The physician will examine the child physically. Additionally, the child could undergo certain investigations:

  • Urine Examination: This is carried out to measure the amount of protein.

  • Blood Examinations: These test the child's blood for other blood components, such as albumin, blood protein, and cholesterol.

  • Renal Sonography, or Ultrasonography: This non-invasive examination generates pictures of bodily tissues using sound waves and a computer. A transducer used in this test is moved over the abdomen in the kidney region by the medical professional. This projects an image of the kidney onto a monitor. The kidney's size and form are visible to the medical professional. In addition, a growth, kidney stone, cyst, or other issue may also be visible to evaluate the kidney's health.

  • Biopsy of the Kidneys: A little sample of kidney tissue is taken by a medical professional. This is accomplished during surgery or by passing a needle through the skin. A microscope is used to examine the sample.

How Is Idiopathic Nephrotic Syndrome Treated in Children?

The child's age, overall health, and symptoms will all affect the course of treatment. It will also depend on the severity of the ailment.

If the kid has nephrotic syndrome, they might need to stay in the hospital during the initial episode. Parents may need to keep an eye on them if the kid develops significant edema, low blood pressure, or breathing issues. Initial symptoms, as well as relapses, can be managed with medications.

These might consist of:

  • Corticosteroids.

  • Immunosuppressive drugs.

  • Diuretics to lessen inflammation.

The immune system is weakened by the medications used to treat nephrotic syndrome. Hence, live vaccinations should not be administered to the child. The child could require a vaccination if they have not received the chickenpox vaccination and are exposed to the virus. Discuss every medication's advantages, disadvantages, and potential adverse effects with the physician and medical professionals.

Nephrotic syndrome can cause children to struggle with controlling their body's water balance. Fluid retention from this may result in swelling (edema). A child with nephrotic syndrome may need to consume less salt and liquids in their diet. This might assist in controlling the child's fluid balance. Any liquid food at room temperature, such as ice cream and popsicles, is considered a fluid. Body swelling is impacted by salt. Avoid adding salt to food at the table and prevent letting the kids eat salty snacks. The amount of salt and water the child should consume daily will be discussed by the child's healthcare professional.

What Are the Potential Side Effects of Idiopathic Nephrotic Syndrome?

Rarely, a youngster may require dialysis due to renal failure. Dialysis is a process used to remove surplus fluid and waste from the blood. Normally, the kidneys handle this.

What Precautions To Be Taken by Parents for Idiopathic Nephrotic Syndrome?

To keep the child healthy, parents may do the following:

  • Assist in shielding the child from those sick with the common cold or other respiratory ailments. A viral respiratory disease may be the reason for a recurrence.

  • Check for protein in the child's urine daily. Finding the early indicators of a relapse will be aided by this.

  • Routine vaccinations should wait until the kid has been off steroids and in remission for at least three months. Speak with the child's medical professional.

Both on its own and with medication, nephrotic syndrome is treatable. In addition, it could worsen despite therapy. A youngster could have phases of progress and regression. An essential component of a child's well-being is family support. When a kid enters adolescence, the condition often goes into remission. This indicates that the symptoms lessen or disappear. Most of the time, symptoms disappear in adults.

Conclusion

In conclusion, idiopathic nephrotic syndrome is still a complicated and multidimensional illness marked by increased protein loss through the urine that has no known etiology. The main goals of treatment plans are to reduce proteinuria, manage symptoms, and avoid complications. The parents should be cautious about the symptoms. Proper care and diagnosis help physicians prevent complications and promote early management.

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Dr. Yash Kathuria
Dr. Yash Kathuria

Family Physician

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