Kidney disease in children – Nephrotic Syndrome

What is nephrotic syndrome?

Nephrotic syndrome as the name suggests is a disease of the kidney which affects the filtering system of the kidney known as the nephrons, which are the small functioning structures within the kidney. These nephrons are damaged due to various causes which results in a set of symptoms, with which the patient usually presents. Hence this condition is a known as nephrotic syndrome. The characteristic symptoms of nephrotic syndrome include:

• Large amounts of protein present in the urine which results from leakage of protein through the damaged nephrons which usually prevent protein leak.
• Low levels of protein in the blood, which results from the protein leaking out into the urine.
• Edema or tissue swelling in all areas of the body, which is the result of reduce 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.
• Increased levels of cholesterol in the blood.
• Reduced frequency of passage of urine as well as volume of urine.
• Increased body weight due to retention of water within the body

There are different types of nephrotic syndromes which can result in children as a result of the different causes behind them. But the most common type of nephrotic syndrome which occurs in children belongs to a group called Idiopathic Nephrotic Syndrome which means that there is no identified cause behind the development of these symptoms, in comparison to the other types. And even amongst the idiopathic type, children develop what is known as Nephrotic Syndrome with Minimal change also called Minimal Change Nephrotic Syndrome (MNS). This is because on histological examination of the kidney tissue, in this case, there is very little change or damage that can be identified, and yet the child will present with full blown symptoms. MNS is commoner in boys than girls. And when children develop this condition, it is very common for them to experience relapses, meaning that they can develop these symptoms from time to time. But these episode can be managed well, and the prognosis for the condition is good.

There is a rare form of nephrotic syndrome known as Congenital Nephrotic syndrome, where the symptoms will develop as early as the first week of life, and the prognosis for this is very poor. It is usually inherited from the parents, where both parents have been carriers.

The symptoms of nephrotic syndrome:

The characteristic symptoms of the disease 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

These symptoms may also be present as a result of other medical illnesses and you must always consult your doctor for the correct diagnosis, if your child develops any of the above mentioned symptoms.

Diagnosing nephrotic syndrome:

A diagnosis of nephrotic syndrome is made based on investigation findings, these investigations include:

• Urine full report and other urine tests to check for the amount of protein present in the urine
• Blood tests, which will check the amount of protein called albumin present in the blood and the level of cholesterol in the blood
• Ultrasound scan of the kidneys, which will help to visualize the kidneys and determine its shape and size, as well as other abnormalities such as presence of masses, stones or instruction
• Renal biopsy, where a small needle will be inserted into the abdomen in order to draw out a tissue sample from the kidney, so that it can be visualized under a microscope to look doe damage at the cellular level

Treatment of nephrotic syndrome:

There a variety of treatment methods which are employed for nephrotic syndrome, and this choice depends on a variety of factors such as, your child’s age, past medical history, overall health status, the ability of the child to tolerate certain medications, the extent to which the disease has progressed at the time of presentation, how we, as medical professionals expect the disease to progress, and your opinion as well.

Taking into consideration all of these factors, the medications which are used include:

• Corticosteroids
• Diuretics to bring down the edema
• Immuno- suppressive therapy
• Low salt diet with other restrictions
• IV albumin, which helps to replace the protein in the blood

During the initial few episodes your child will need hospital admission, because the severity of the disease has to be monitored. Your child should not receive any live vaccine during an acute episode of nephrotic syndrome because the condition weakens the immune system. The prognosis for this condition is good, because even though relapses may occur during childhood, once your child reaches puberty and moves into adulthood, the disease tends to stay under control and in remission.