Nephrotic Syndrome
About a million filtering units called nephrons make up kidneys. Every nephron has filters, known as a tubule and a glomerulus. The blood is filtered by the glomerulus, and waste and excess water are taken out of your blood by the tubule, which then becomes urine. An excessive amount of protein can leak from the blood into urine due to inflammation of the glomeruli, which is the cause of nephrotic syndrome.
Nephrotic syndrome is defined by clinical characteristics that arise from alterations to the glomerular capillary wall, which results from increased permeability, particularly from thrombo-embolic or pathogenic agents. The compromised membrane of the renal glomerulus causes hypoalbuminemia(less than 30 g/L) and severe proteinuria(above 40 mg/m^2 per hour), commonly linked to edema, generalised hyperlipidemia, and other complications. Primarily, it is caused by intrinsic renal disease and secondarily caused by congenital infections, diabetes, systemic lupus erythematosus, neoplasia, or certain medications.
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Symptoms of Nephrotic Syndrome
Facial edema is typically the initial symptom in youngsters. It may manifest in its usual form or along with nephritic syndrome. The latter indicates inflammation of the glomerulus, which results in hematuria and reduced kidney function. Children with nephrotic syndrome initially exhibit facial edema, which later spreads throughout the body.
The following are a few common symptoms:-
- Foamy urine
- Appetite loss
- Abdominal pain
- Fatigue & exhaustion
- Swollen legs, feet or ankles
- Weight gain due to fluid retention
- Low albumin levels in the blood(hypoalbuminemia)
- High cholesterol levels in the blood(hyperlipidemia)
Nephrotic Syndrome in Children
In rare circumstances, a newborn may have congenital nephrotic syndrome(inherited by a recessive autosomal gene). From each carrier parent, a kid receives one copy of the gene. Every time they get pregnant, carrier parents run the 1 in 4 risk of giving birth to a kid with this condition. However, this kind of nephrotic syndrome does not have an established prognosis. Nephrotic syndrome can cause children to experience difficulties controlling their body's fluid balance, which may result in edema or swelling due to fluid retention. Restricting salt and liquids may be part of a child's diet if they have nephrotic syndrome, as they assist in maintaining the fluid balance.
Causes of Nephrotic Syndrome
Glomerular disease, characterised by kidney scarring (sclerosis), is called FSGS. Generally, a few per cent of glomeruli are initially injured, and FSGS scarring only occurs in small areas of each glomerulus (filter). Twenty per cent of cases in children and forty per cent of cases in adults with nephrotic syndrome are caused by focal segmental glomerular sclerosis (FSGS).
It is characterised by increased renal membrane permeability and protein loss due to glomerular filtration barrier damage and is a significant cause of concern for nephrotic syndrome in children. The symptoms appear earlier in MCD patients than in patients with other glomerular illnesses. It is responsible for 70–90% of cases in children(older than one year), compared to 10-15% in adults.
Among the glomerular disorders that cause nephrotic syndrome is membraneous nephropathy (MN), often called membraneous glomerulopathy. In addition to peripheral edema, hypertension, frothy urine, and symptoms associated with thromboembolic complications, it is characterised by severe proteinuria (>3.5 g/day). Urine microscopy shows that it differs from nephritic syndrome due to the absence of red cell casts, hematuria, and active sediments.
Nephrotic syndrome may manifest as primary (idiopathic) or secondary. Ninety per cent of cases in children are primary, with the other instances being secondary. Secondary nephrotic syndrome causes include infections(Hepatitis B, C, Human immunodeficiency virus, Malaria, Toxoplasmosis, Syphilis), drugs, and malignancies(Lymphoma, Leukemia).
Other significant causes include:
- Infections
- Amyloidosis
- Hereditary Causes
- Certain Medications
- Diabetic Nephropathy
- Congenital Nephrotic Syndrome
- Systemic Lupus Erythematosus (SLE)
- Membranoproliferative Glomerulonephritis
Complications Associated With Nephrotic Syndrome
Lungs, the brain, and peripheral arteries can all be impacted by thromboembolic implications. Thromboembolic events occur in around 3% of overall cases. These occurrences result in a hypercoagulable condition and are brought on by a decrease in antithrombin III and protein S in the urine and an increase in fibrinogen concentration.
Antibodies directed against membrane anionic phospholipids (e.g., anticardiolipin antibody, antiphosphatidylserine) or their related plasma proteins, primarily beta-2 glycoprotein I (apolipoprotein H), are chronically increased in this illness.
Nephrotic syndrome may result in infections and has adverse side effects. The susceptibility to infection is influenced by factors such as decreased IgG production, poor T-cell function, and urine loss of factor B (which aids in the opsonisation of bacteria). Peritonitis is the most prevalent infection.
Patients with nephrotic syndrome have a higher risk of developing cardiovascular disease due to endothelial dysfunction, hyperlipidemia, and enhanced thrombogenesis. The degree of hypoalbuminemia is closely correlated with hypercholesterolemia, and cardiovascular disease can be exacerbated by chronic proteinuria or renal insufficiency.
Acute renal failure (ARF) is a rare side-effect of nephrotic syndrome. Reduced circulating volume in the plasma due to severe proteinuria(protein in the urine) and significantly low albumin levels result in circulatory collapse or pre-renal uremia, which is moderate in severity.
Children with nephrotic syndrome and low tissue pressure are frequently seen to have edema. While pericardial effusion is uncommon until heart function is impaired, ascites and pleural effusions are common. Reduced plasma oncotic pressure and functional hypovolemia are repercussions of edema, which is brought on by increased glomerular permeability and hypoalbuminemia.
Diagnostic Tests
Diagnosis for nephrotic syndrome includes the following tests.
Urine Analysis
Nephrotic kidney disease is typically identified by the presence of protein in the urine by your doctor. If your urine test yields positive findings for protein, then in that case, you may have this illness.
Blood Test
The doctor assesses the kidney’s waste filtration capacity based on the blood test results. If they are not filtering waste like they should, nephrotic syndrome may be the cause of it.
Renal Biopsy
The doctor will study a small sample of your kidneys under a microscope during a kidney biopsy to check for damage and illness.
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