Nephrotic Syndrome in Children

Have you ever noticed swelling around your child’s eyes or body, or observed that their urine is frothy? Have you ever wondered what it is, whether it’s dangerous, or if it can be treated?

Children that present with the symptoms mentioned above should be brought to see a doctor promptly for diagnosis and given the correct care. In general, the doctor will determine the causes of the swelling, which can be categorized into these larger groups:

• Heart failure
• Liver failure
• Kidney disease
• Anaphylaxis
• Protein malnutrition

After doctors have differentiated between these conditions, they can investigate further to confirm the diagnosis and treat the causes of the condition, providing immediate treatment to prevent any additional complications.

Nephrotic syndrome or the conditions causing protein to leak into the urine (known as proteinuria), is one cause of kidney disease. The patient usually presents with swelling on the upper eyelids, legs, abdomen, or the whole body, along with frothy urine. The symptoms can be arranged in groups, consisting of:

• A history of swelling around the eyes, legs, feet, ankles, abdomen, or over the whole body
• A certain amount of protein leaking into the urine
• Low blood-protein levels
• High cholesterol levels

Symptoms are usually found in children ages one to eight years old, but can occur across all age groups and have different causes. If we look into the kidney’s filtration unit, we can find inflammation and damage to the blood vessels in what’s called the glomerulus. This inflammation and damage to the blood vessels in the glomerulus causes a protein called albumin to leak out into the urine. The loss of albumin, an essential body protein, can lead to the conditions mentioned above, and result in subsequent complications.

• In children, most of the time the of causes of this inflammation are not entirely clear; but, very small abnormalities can be found in the kidney filtration unit, called minimal change disease. The remainder may be due to abnormalities in parts of the membrane, or a thickening of the kidney filtration unit’s wall. There are only a small number of known causes, such as SLE, viral infections, or complications from using medicines.
• In adults, 30% of the causes are from congenital abnormalities present in the patient, such as diabetes and high blood pressure, the remainder are mostly known conditions, like infections or autoimmune conditions (e.g., SLE), medications, or heavy metals. There are only a few unknown causes, unlike those found in children.

• Swelling of the body, abdomen, and sexual organs
• Abnormally fast weight gain
• Fluid in the lungs or abdominal cavity
• Prolonged dyslipidemia that is unresponsive to treatment and can affect the coronary arteries
• Hypercoagulation and risk of blood clots
• Susceptibility to repeated and severe infections
• Low blood pressure
• Less urine/no urine/kidney failure

These complications are considered quite dangerous and their occurrence must be monitored closely, while the patients themselves must also take the correct steps to prevent complications occurring in the first place.

• In children, the causes are largely unknown. However, you do not need to worry about the treatment regardless of whether the cause is known or not. Eighty percent of patients respond well to treatments, while just 20% of this group (only) respond slowly, or poorly.
  • Steroids are the standard form of treatment and the first-line treatment considered for treatment. The treatment period lasts for several months, and is adjusted according to the patient’s response to the treatment and prescribed standards. The patient must also be monitored to prevent complications.
  • Immunosuppressants is a group of drugs that would be administered if the patient does not respond to steroid treatments within the prescribed time or if there is a poor response to steroids or the patient relapses to the point where steroids can no longer be used, or if there are significant side effects from the steroids. There are many types of immunosuppressants and they are quite costly. Furthermore, a kidney biopsy must be performed before each type of immunosuppressant is considered for use.
  • Diuretics can be administered orally or via injection. Whether they are used depends on the severity of the condition, although they are usually considered for patients with substantial swelling.
  • Albumin infusions into the vein, in addition to diuretics, are administered to patients with severe symptoms, a lot of swelling, excess fluid in the body, and at risk of serious complications, to ensure patients are in a safe condition and able to continue with the next oral drug treatment.
  • In the case of known causes, treatment will proceed accordingly.
• In adults, where causes are largely known, treatment will proceed accordingly, and any congenital conditions will be managed and treated. Other medications that may be considered include drugs to reduce protein in the urine, reduce cholesterol, anticoagulants to prevent clots or an embolism, and steroids also may be used as indicated.

As for whether the condition can be cured, in cases where the causes are known, it depends on how well the symptoms can be managed, whether the causes are curable, or if effective support can be given in accordance with each type of condition and cause. In cases where the causes are unknown, as in the majority of children’s cases, the goal is to ‘manage’ the symptoms, but there will be periodic relapses. In this group, 50% will see a relapse occur in the first year, as is characteristic of the condition, and 80% can experience at least two to three relapses. Once past this phase of frequent relapsing and into adolescence, the frequency of relapses decreases and often stops.

Factors that typically cause relapses in children:

• Infections. The most common infections include upper respiratory tract infections, followed by tooth decay, and parasitic gastrointestinal tract infections. Conditions should be monitored in order to prevent the aforementioned infections, and patients should promptly visit their doctor for repeat urine tests when infections are found.
• Failing to take medication consistently as prescribed by the doctor can lead to the condition being managed poorly, resulting in relapses, or making managing the condition difficult.
• Natural relapses of the condition are not a huge worry but other causes must be investigated to determine what is causing the relapses, as well as treating them, administering medications, and strictly monitoring the symptoms as advised by the doctor.

Care must be taken to prevent infections as advised by your doctor, as well as continuing to take any prescribed medications and to regularly attend appointments with your doctor.

Photo Credit: Nathan Put-Fernandez Flickr via Compfight cc