Nephrotic syndrome is disease of the kidneys where the filtering system made of functional units called the nephrons, gets damaged. This is the name given to the collection of findings which develop as a result of such a damage. When the filtering system gets damaged, the kidney tends to filter substances which it usually doesn’t, and filter less of substances which it usually does. All of these issues give rise to some characteristic findings, which include:

Large amounts of protein present in the urine which results from leakage of protein through the damaged nephrons which usually prevent protein leak. This is known as proteinuria in medical terms and is calculated according to your child’s age and body weight.
Low levels of protein in the blood, which results from the protein leaking out into the urine, and is called Hypoalbuminemia.
Edema or tissue swelling in all areas of the body, which is the result of reduced levels of protein in the blood. This swelling is especially evident around the eyes, in the face, and in the abdomen which is known as ascites. The swelling results because the protein in the blood actually acts like a sponge which helps to keep most of the fluid in the blood, and when there is a reduced amount of proteins in the blood, the water tends to leak out from the blood into the tissues, resulting in swelling.
Increased levels of cholesterol in the blood, which results from reduced levels of protein in the blood which is a signal for the body to increase its production of certain types of fat.
The symptoms and signs of nephrotic syndrome:
The signs and symptoms that children often present with include:

Malaise and fatigue
Abdominal pain and swelling
Frothy urine
Increased body weight and facial edema
Loss of appetite
Paleness of the nail bed
Dull hair
Development of intolerance to certain food or allergies
The cartilage of the ears may feel less firm
The different types of nephrotic syndrome:
There are different types of nephrotic syndromes in existence as a result of the different causes. Some of these causes include, diabetes, hepatitis B and C, medications such as corticosteroids, and conditions like systemic lupus erythematosis, and cancer of the kidneys. The most common type of nephrotic syndrome to affect children is one that is known as idiopathic nephrotic syndrome, where there is no identifiable underlying cause.

Nephrotic syndrome can also be categorized according to the way the kidney tissue appears when it is viewed under a microscope following a biopsy. According to this tow forms have been recognized, Minimal Change Disease (MCD) and Focal Glomerular Sclerosis (FSGS).

Minimal Change Disease (MSD):
This is most common form to affect children and you will see that the renal cells look almost normal when seen under the microscope. It responds well to steroids, and has a good outcome in most cases. This form doesn’t have any lasting damage on kidney function, and relapses less and less frequently as the child ages, and is usually absent in adulthood. It is very rare for a child affected by minimal change disease to require dialysis or kidney transplant.

Focal Glomerular Sclerosis (FSGS):
This form of disease is present only in about 10% of the children with nephrotic syndrome. It requires biopsies in order to be diagnosed and doesn’t usually respond to steroid therapy, and is an aggressive form, which more often than not requires the child to undergo dialysis or a kidney transplant.

What is congenital nephrotic syndrome?
As the word suggests this form of nephrotic syndrome is present at birth, but it is most likely that the condition will go unnoticed at the time of birth and be discovered only when the baby is few months old. It is a very rare condition and is caused by a genetic predisposition where both parents have to be carriers of the condition. It is a very aggressive and severe form of nephrotic syndrome where the affected baby will require a kidney transplant as soon as possible, ideally within the first few years of life. And till a transplant becomes possible, the baby will require transfusions of blood protein almost every day, because the protein leaks out from the kidneys at a rapid rate. They will also require frequent boosting of their immune system and hormones in order to keep them healthy till the transplant can be done, and till they reach the appropriate weight with which a transplant becomes possible, which is about 15 pounds when the child is roughly around one year.

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Testing for and diagnosing nephrotic syndrome in children

Testing For And Diagnosing Nephrotic Syndrome In Children
Nephrotic syndrome explained:
Nephrotic syndrome is a condition which affects the kidneys and the miniscule filtering system within it, called the nephrons. There is damage to these structures as a result of various reasons, and mostly in children the cause remains unknown, also known as idiopathic nephrotic syndrome. As a result of this damage, the kidney starts to filter less of the substances that it usually helps our body get rid of, and it also starts to filter in excess certain substances which are useful to the body. Therefore an affected child usually presents with certain characteristic symptoms, including, swelling of the tissues, especially in the face and abdomen.

Diagnosing the condition:
It is when a child presents with this characteristic symptom of swelling in most areas of the body, that your doctor/pediatrician will suspect nephrotic syndrome. It is very important that the diagnosis is made correctly, because only then can prompt treatment be started in order to effectively manage the condition. Nephrotic syndrome is a condition which can be accurately diagnosed using a few tests, because the condition has some telltale signs which can easily be detected.

The investigations used to diagnose nephrotic syndrome:
The diagnostic tests which are carried out when a doctor suspects nephrotic syndrome in your child include:

Urine test – to look for the levels of protein in the urine, because nephrotic syndrome results in increased leakage of protein form the kidney and high levels of protein in the urine. The urine is also tested under the microscope in order to look for signs of inflammation of the kidney and kidney damage, which may indicate the type of nephrotic syndrome your child is suffering from.
Blood test – in order to look for the level of protein in the blood, because the increased leakage of protein in the urine, will result in reduced levels of protein in the blood, which is the reason why the child develops swelling within the body tissues.
Renal biopsy – Children usually do not require kidney biopsy at the onset. Which is a test where a small sample of kidney tissue is drawn out using a needle, and viewed under a microscope, in order to see the changes which have occurred in the kidney tissue. Some children may require additional immunosuppression if they frequently relapse or are steroid dependent.
Nephrotic syndrome can present in two forms. The first known as Minimal Change Disease is the most common form to affect children, and is a form that doesn’t show a lot of damage or change in the urine test, and even in the biopsy, there will not be any identifiable damage to the filtering system of the kidney. The second form known as Focal Glomerular Sclerosis, is a more aggressive form of the disease, and shows extensive damage in the urine test as well as renal biopsy.

On certain occasions, if your child is very symptomatic, your doctor will begin treatment straight away without waiting for the tests to confirm which form of nephrotic syndrome your child is suffering from. The treatment for nephrotic syndrome is a course of steroids, which are administered daily, till your child becomes symptom free. Once the treatment has commenced if it is minimal change disease, it will respond well to the treatment and immediate signs of improvement can be seen in your child. But if it Focal Glomerular Sclerosis, then it will not respond well to treatment with steroids.

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Our Team Published A Very Important Review On ‘Multisystem Inflammatory Syndrome In Children
Our team published a very important review on ‘Multisystem Inflammatory Syndrome in Children with COVID- A Pediatric Nephrologist’s Perspective’ in journal ‘Clinical Kidney Journal’. This review is very important for all physicians, Pediatricians and Nephrologists taking care of children with COVID and AKI.

About Dr.
Dr. Sidharth Kumar Sethi
Kidney & Urology Institute
He was trained as a Fellow (International Pediatric Nephrology Association Fellowship) and Senior Resident in Pediatric Nephrology at All India Institute of Medical Sciences and Division of Pediatric Nephrology and Transplant Immunology, Cedars Sinai Medical Centre, Los Angeles, California. He has been actively involved in the care of children with all kinds of complex renal disorders, including nephrotic syndrome, tubular disorders, urinary tract infections, hypertension, chronic kidney disease, and renal transplantation. He has been a part of 8-member writing committee for the guidelines of Steroid Sensitive Nephrotic Syndrome and Expert committee involved in the formulation of guidelines of Pediatric Renal Disorders including Steroid Resistant Nephrotic Syndrome and urinary tract infections. He has more than 30 indexed publications in Pediatric Nephrology and chapters in reputed textbooks including Essential Pediatrics (Editors O.P. Ghai) and “Pediatric Nephrology” (Editors A Bagga, RN Srivastava). He is a part of Editorial Board of “World Journal of Nephrology” and “eAJKD- Web version of American Journal of Kidney Diseases”. He is a reviewer for Pediatric Nephrology related content for various Pediatric and Nephrology journals.

For more information = http://www.pediatricnephrologyindia.com/