How to Deal With Nephrotic Syndrome in Children

Nephrotic syndrome or nephrosis is a rare condition that can affect both children and adults. Childhood nephrotic syndrome is usually diagnosed in children between the age of two to fifteen. It can be life-changing for the child and the parent. A clear understanding of its causes, symptoms, treatment, and prognosis can greatly help in the management of the disease. Continue reading to know more.

What Is Nephrotic Syndrome?

Kidneys are one of the primary excretory organs of the body, and they function by separating out unwanted wastes and toxins from the body through urine. Tiny structures called glomeruli act as filtering machinery inside the kidney. When these glomeruli fail to function effectively, the filtering mechanism fails.

Nephrotic syndrome refers to a condition in which the kidneys (glomeruli) fail to preserve the proteins from the blood and excrete it in the urine. The term ‘nephrotic syndrome’ is used to indicate the condition in which the proteins in the urine are above a certain permissible range. Many medical conditions can lead to this phenomenon, and a deeper examination is usually required to ascertain the cause.

Types of Nephrotic Syndrome in Children

Nephrotic syndrome in children is classified as primary childhood nephrotic syndrome, secondary childhood nephrotic syndrome, and congenital nephrotic syndrome.

• Primary Nephrotic Syndrome: This type of nephrosis is also called idiopathic nephrotic syndrome,  and many times, the reason behind its occurrence is unknown. Minimal Change Disease, focal segmental glomerulosis, and membranoproliferative glomerulonephritis (MPGN) come under this type of nephrotic syndrome.
• Secondary Nephrotic Syndrome: This subtype of nephrosis, is caused by some kind of infection, the overdose of certain medications, or an underpinning disease in the body. Diseases like diabetes, hepatitis, lupus, HIV, malaria, streptococcal infection and Henoch-Schönlein purpura are some diseases that are associated with the secondary nephrotic syndrome.
• Congenital (Finnish-Type) nephrotic syndrome: This is the hereditary form of nephrotic syndrome that is transmitted from one generation to the other through their DNA. Primarily, a genetic variation in the gene NPHS1, coding for a key transmembrane protein called nephrin, causes this syndrome. It is quite rare and manifests as early as three months of age. Other genes, whose mutations are found to cause the syndrome, are NPHS2-Pododsin gene and WT1- Wilms tumour suppressor gene.

Causes of Nephrotic Syndrome in Children

The causes of childhood nephrotic syndrome are many, as the syndrome is just a manifestation of an underlying condition. The various causes of each type are as follows:

1. Causes of Primary Nephrotic Syndrome

The causes of Primary Nephrotic Syndrome are:

• Minimal change nephrotic syndrome (MCNS): MCNS is characterised by minute damages to the glomeruli in the kidney. These damages are so tiny that they are visible only in an electron microscope. This sub-type of nephrotic syndrome contributes to nearly 50% of primary nephrotic syndrome cases. The cause of MCNS is unknown.
• Membranoproliferative glomerulonephritis (MPGN): In this condition, there is a build-up of antibodies over the minute structures of glomeruli, causing it to become thick. The causes of MPGN can be an autoimmune disease, carcinoma or some kind of infection.
• Focal segmental glomerulosclerosis (FSGS): FSGS, as the name suggests, is sclerosis or scarring of a focused individual part of the glomeruli. This can cause irreversible damage to the kidneys.
• Idiopathic crescentic glomerulonephritis: This condition occurs when the glomerulus is visible as crescent shapes under a microscope. The cause of this type of idiopathic glomerulonephritis is unknown.

2. Causes of Secondary Nephrotic Syndrome

The causes of Secondary Nephrotic Syndrome are:

• Systemic diseases like diabetes (diabetic nephropathy), renal vein thrombosis, lupus erythematosus, vasculitis, Poststreptococcal glomerulonephritis, and lymphoma.
• Infections like congenital toxoplasmosis, syphilis, rubella and cytomegalovirus, HIV AIDS, Hepatitis B, hepatitis C, and malaria.
• Overuse of some drugs also can cause secondary nephrotic syndromes like usage of Penicillamine, NSAIDs (Non-steroidal anti-inflammatory drugs), heroin, mercury, lithium and Pamidronate.

3. Causes of Congenital Nephrotic Syndrome

This type is found associated with rare diseases caused by genetic mutations. These mutations have autosomal recessive inheritance and can be passed down in generations. If a child has a family history of this disease, then there are high chances of him acquiring the faulty gene from the parents. Some of the rare diseases associated with nephrotic syndrome are:

• Finnish-type congenital nephrotic syndrome
• Galloway-Mowat syndrome
• Oculocerebrorenal (Lowe) syndrome
• Nail-patella syndrome
• Pierson syndrome
• Denys-Drash syndrome
• Frasier syndrome

Symptoms of Nephrotic Syndrome in Children

Childhood nephrotic syndrome is characterised by one or many of the following symptoms:

• Oedema: Oedema is the accumulation of water and subsequent swelling in selected parts of the body. In the case of nephrotic syndrome, oedema is found in legs, feet, ankles, face, around eyes and hands.
• Albuminuria: Due to improper functioning of the kidneys, the albumin (major form of body protein) that needs to be retained in the blood, gets excreted in the urine. This phenomenon is clinically measured and termed as Albuminuria. An increased level of albumin in the urine indicates a problem in the kidneys.
• Hypoalbuminemia: A low level of albumin in the blood, known as hypoalbuminemia is also a measure of the faulted excretory system.
• Infections: Loss of important proteins, that play a role in immune functions, in the body makes them more prone to infections.
• Dyslipidemia and clotting issues: These Children have a tendency of having high lipid fractions and very high cholesterol level. The loss of certain blood-thinning proteins also makes them more vulnerable to blood clotting and stroke-like episodes.
• Growth issues: As proteins are essential building blocks for growth and development, a loss of proteins in the urine affects the growth in children. Repeated infections, use of steroids in treatment, malabsorption, etc. are contributing factors.

Diagnosis of Childhood Nephrotic Syndrome

For children who present with the classic symptoms of childhood nephrotic syndrome, the doctor usually prescribes the following diagnostic tests:

• Urine Dipstick Test: It is a simple procedure that is performed with the first urine of the day. A dipstick is used to check the urine for the presence of proteins. If the protein concentration is high, it changes the colour of the dye in the dipstick. The concentration of the protein is quantified with the help of a colour-coded chart.
• Urine Test: To gain further clarity on the concentration of excreted protein in the urine, the doctor would prescribe a routine urine test to measure the albumin levels. It is called the protein-creatinine ratio, and although it is not specific, a high ratio of albumin and creatinine in the urine assists in the diagnosis of the condition.
• Blood Test: As a converse, the protein levels in the blood are checked to estimate the extent to which the proteins are lost from the blood. A blood test is also used to decipher the underlying cause of the leakage of essential proteins.
• Physical Exam: The doctor also checks for any visible signs of nephrotic syndrome in the child, like patchy skin, swelling in the body, fever, fluid collection in the abdomen, etc.
• Family History: Due to genetic disposition, a thorough understanding of any family history of nephrotic syndrome is essential for the doctor to arrive at a correct diagnosis. Genetic testing is also prescribed in recent times to find out the exact cause of the syndrome.
• Ultrasound of the Kidney: If the urine or blood tests are suggestive of a nephrotic syndrome, the doctor carries out an ultrasound examination of the kidneys to rule out other causes leading to nephrotic syndrome-like conditions.
• Biopsy: In some rare cases, doctors recommend a biopsy test of the kidney tissue to understand the morphology of the kidney cells. It is usually not performed on very young children and is indicated only for very specific listed conditions.

What Category of Kids Are At the Risk of Developing Nephrotic Syndrome?

Children with idiopathic or primary nephrotic syndrome do not have a clear-cut reason behind their condition. Research is still ongoing in deciphering the exact risk factors for it. However, secondary nephrotic syndrome usually affects children with diseases that cause harm to the kidneys, or infections that elicit an immune response.

Children who carry mutations in the genes encoding some important proteins that are involved in the proper functioning of the kidneys can suffer from congenital nephrotic syndrome. Those children who have a known history of the nephrotic disease are at a greater risk of the disease compared to other kids. Additionally, according to the available data, boys have a higher chance of developing nephrotic syndrome than girls.

Risks and Complications

Children suffering from nephrotic syndrome primarily lose essential proteins in the urine due to the lack of an efficient excretory mechanism. When these essential proteins are lost, it impacts the growth and development of the child.

The proteins that prevent blood clotting are lost in children with the syndrome, making them more prone to blood clots. Children with this condition are also highly prone to various infections, and doctors prescribe medications to counter them.

Dyslipidemia (increased cholesterol) and decrease in calcium levels in the body are some more associated risk factors leading to other issues.

Further complications that are likely to be associated with this syndrome are:

• Anaemia
• Fluid retention
• Heart disease
• Blood pressure
• Kidney failure

Treatment of Nephrotic Syndrome in Children

The treatment options for nephrotic syndrome differ for each subtype of the syndrome. Some of the standard treatment options for each type of nephrotic syndrome are listed below:

1. Treatment of Primary Nephrotic Syndrome

Primary nephrotic syndrome is treated with different types of medications, which are given to control the blood pressure, correct oedema, or to lower the immune response.

1. Steroids: Many cases of idiopathic nephrotic syndrome are caused by the circulating autoantibodies against their own glomerular cells. In order to reduce this aberrant immune response, corticosteroids like prednisone are prescribed. These steroids not only act as immunosuppressant but also prevent the loss of valuable proteins in the urine. This is usually the first-line therapy that is prescribed for about 4-6 weeks. The dosage is then tapered gradually and given for consumption on alternate days, for four weeks. A period of remission, where the affected children do not show any visible symptoms of the syndrome, is achieved through this medication. However, in some children, there is a relapse of symptoms after some time. In such cases, the doctor usually prescribes other classes of immunosuppressant along with the corticosteroids.
   Although considered one of the effective first-line treatments for childhood nephrotic syndrome, prednisone has a couple of side effects like excessive weight gain, increased appetite, mood swings, hyperactive behaviour, brittleness of bones, etc. These side effects usually fade out after the dosage is stopped. Consumption of other immunosuppressants can also expose the child to the risk of catching infections, like chickenpox, soon. Hence immunisations prior to starting them are very important.
2. Lowering Blood Pressure: Medications that lower the blood pressure like ACE inhibitors or beta-blockers are prescribed for children. The elevation of blood pressure is observed, and such medications are essential to keep it under control to avoid further complications.
3. Diuretics: Oedema is a very common symptom of childhood nephrotic syndrome, and it is treated with diuretics. Diuretics work by excreting the excess fluid in the body. They are only given in certain conditions.
4. Albumin Infusion: To restore the albumin lost from the body, doctors usually advise intravenous infusion of albumin into the blood. This method is generally used if the loss of albumin is severe.
5. Kidney Transplant: This is rarely required in MCNS or primary type of Nephrotic Syndrome. However, if the nephrotic syndrome is severe, the child may require a kidney transplant. Until a suitable match is found, dialysis is performed to sustain the excretion process.

2. Treatment of Secondary Nephrotic Syndrome

Secondary nephrotic syndrome is caused by certain infections, systemic diseases or overdose of certain medications. Apart from the drugs used to treat primary nephrotic syndrome, doctors also prescribe antibiotics to treat infections, causing the nephritis. Additionally, if any systemic diseases are suspected, then medications to treat those diseases are also given to the children.

3. Treatment of Congenital Nephrotic Syndrome

Congenital nephrotic syndrome is caused due to certain mutations in key transmembrane proteins. Children with congenital problems usually are treated with medications that reduce the symptoms of nephritis. The only available cure is kidney transplantation, wherein a healthy kidney from a suitable donor is used to replace the diseased one. Dialysis is performed to sustain the excretory function until a donor with a match is found.

Home Remedies

Although there are no home remedies that cure nephrotic syndrome completely, a restricted diet plan for children with nephrotic syndrome can greatly help reduce the stress on the kidneys. The diet for such children should be planned, keeping the following points in mind:

• Low-salt foods in order to keep a check on oedema.
• Low cholesterol diet.
• Limited consumption of water, juice, etc.
• Low saturated fat.
• Diet rich in proteins in the recovery period.
• Diet rich in calcium and other multivitamins.

A healthy balanced diet can be given to the child. Proper management at home involves continuous monitoring of the protein levels in the urine with a dipstick, and diligently following the medications given by the doctor.

Tips to Prevent Nephrotic Syndrome in Kids

If the nephrotic syndrome is congenital or idiopathic, prevention is not possible. However, secondary nephrotic syndrome can be prevented by following these steps.

• Keeping Infections Away: Maintaining good health and hygiene can be very important in protecting children from unwanted infections. Excessive hygiene and not letting children get exposed to any microorganisms in growing age can lead to aberrant immune response. However, this is still debated. So, a balance in their day-to-day lives, activities, and unnecessary overprotection from benign infections should be advocated.
• Exercise: Exercise is a crucial part of well-being. Children who are prone to a higher risk of nephrotic syndrome should participate in activities like walking, jogging, swimming, etc. Mild exercises that do not exert the body can be very helpful in preventing the relapse of the syndrome, during the remission period.

FAQs

1. Does nephrotic syndrome relapse?

Yes, in some children nephrotic syndrome relapses. Relapses are often triggered by common infections, and they can be brought under control in the initial period. It is crucial for parents and caregivers to observe these signs and bring them to the doctor’s notice. Regular monitoring of the urine for proteins, using a dipstick, is a good way to identify them. A prompt visit to the doctor should be made when the urine dipstick shows 1+ reading for three subsequent days. During an episode, the doctor usually prescribes a regimen of steroid or other types of immunosuppressants to bring the relapse under control.

2. Does it ever get cured?

Usually, childhood nephrotic syndrome has a good prognosis after an episode of medications. By the time the children reach teenage, the disease is completely cured without any damage to the kidneys. Any relapses during this period can also be corrected with medications. In the case of a congenital nephrotic syndrome, a transplant is necessary to completely relieve the child of the disease.

Childhood nephrotic syndrome can be managed effectively with medications and lifestyle changes if diagnosed at an early stage. It can be a challenging phase for the child to cope with the disease and the side effects of the medications. Parents and caregivers can greatly help in the recovery process by lending a helping hand in following the medications accurately and preparing a safe and healthy diet.

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