Nephrotic syndrome is what are some disease states that can cause nephrotic syndrome how do we treat nephrotic syndrome and ultimately what are some of the health consequences of having nephrotic syndrome so to begin nephrotic syndrome is actually a glomerular inflammatory syndrome involving five different aspects the first is excess proteinuria the second is hypoalbuminemia the third is edema the fourth is hyperlipidemia and the fifth is hypercoagulability.
We’re gonna talk about hownephrotic syndrome and different diseases can affect different parts of this glomerular apparatus and nephrotic syndrome exists on a spectrum on one end of the spectrum we have high proteinuria with little hematuria and that’s known as nephrotic syndrome on the opposite end of the spectrum we have high levels of hematuria with very little proteinuria and that is a nephritic syndrome and different diseases exist on different parts of this spectrum at some point when we have high levels of protein in our urine or proteinuria.
We consider that in a nephrotic syndrome and I’ll tell you what that number is in the next slide so how does nephrotic syndrome present itself clinically? I like to use the mnemonic help to help remember the clinical presentation of nephrotic syndrome help stands for hypoalbuminemia and hypercoagulability so a patient with nephrotic syndrome excretes excess amounts of protein in their urine and these proteins can include albumin, protein C, protein s, and antithrombin 3 because they’re excreting so much albumin in their urine.
They can become hypoalbuminemia and because they’re excreting protein C, protein s, and antithrombin 3 they can actually have an increased risk of hypercoagulability. The next one is edema and edema is due to that state of low albumin. If we have low levels of albumin we have decreased oncotic pressure, we have a decreased ability to reabsorb some of that interstitial fluid, leading to peripheral edema. The next one is lipid abnormalities and generally, patients with nephrotic syndrome have increased LDL cholesterol and they also have lipiduria or oval fat bodies in their urine.
The last one is P for proteinuria and this is the hallmark of nephrotic syndrome, and generally, a level of 3.5 grams or greater is necessary to consider a nephrotic syndrome. So, greater than 3.5grams per 1.73 meters squared per day is the level of protein excretion in the urine necessary for the definition of the nephrotic syndrome, and anything below this with hematuria we would generally consider it a nephritic syndrome. So, again to remember the clinical presentation remember.
Hypoalbuminemia and hypercoagulability, edema, lipid abnormalities, and proteinuria. So, nephrotic syndrome is not a disease state in itself, but it can be caused by other diseases, and there are about three to four different diseases that can cause nephrotic syndrome. The first one is minimal change disease. Minimal change disease is named minimal change disease because when we look at a glomerulus under a light microscope there are minimal chances that are observed.
Looks generally like a normal glomerulus – there are very few changes if at all any when we look at it under light microscopy. Minimal change disease primarily affects podocytes and what I want you to remember is that a majority of cases of nephrotic syndrome in children are due to minimal change disease, especially children under the age of 10 years old. Other causes of minimal change diseases include drug use – so NSAIDs, lithium and ampicillin, Hodgkin’s lymphoma, and atopic individuals also have a higher risk of minimal change disease as well.
The second disease is focal segmental glomerulosclerosis or FSGS. Like minimal change disease, it primarily affects podocytes, but when we look at it microscopically we actually read do significant changes – and in this image, we read that there are collapses of capillaries within the glomerulus. It is among one of the most common causes of idiopathic nephrotic syndrome in adults and generally, it’s considered to be about 35% of nephrotic syndrome. Some of the etiologies of FSGS include obesity, infections with HIV, and hepatitis.
B.Having sickle cell disease also increases the risk for FSGS and heroin use. And the third disease is membranous-glomerulonephropathy and this involves glomerulus basement membrane thickening and autoantibodies to phospholipase A2 on podocytes. Here are an immunofluorescence image ofIgG antibodies to those podocytes. So some of the etiologies of membranous glomerulonephropathy include hepatitis b virus infection, lupus, and thyroiditis. Some drugs can also lead to membranous glomerulonephropathy as well as exposure to gold.
Some of the other causes of nephrotic syndrome include amyloidosis so there can be primary and secondary amyloidosis that can lead to renal amyloidosis and secondary amyloidosis is often associated with chronic inflammatory diseases such as rheumatoid arthritis and osteomyelitis. And other causes of the nephrotic syndrome – although these are more likely to be an intermediate nephrotic syndrome, so they are kind of the middle of that spectrum that I showed you earlier.
There’s a mixture of some nephrotic and nephritic components proteinuria and hematuria have membranoproliferative glomerulonephritis. and So these can be related to hepatitis C infections and monoclonal gammopathies And nodular glomerulosclerosis secondary to diabetic nephropathy so how do we treat nephrotic syndrome? Treatment depends on the type of glomerulopathy if we have minimal change disease we use steroids if we have membranous-glomerulonephropathy we often want to reduce blood pressure and use steroids.
Risk of infection the mechanism by which this does this is not well known but generally, a patient with nephrotic syndrome has an increased risk of pneumococcal infections so having a pneumococcal vaccination is important for these patients the fourth is protein malnutrition their excreting lots of lots of protein so they can actually be malnourished with regard to protein in the last is hypovolemia so if they’re reducing a lot of their albumin they become hypoalbuminemia hypo album anemic they can have an increased diuresis and they can actually lose some of their this systemic volume becominghypovolemic and again the 5 health consequences of nephrotic syndrome areas thromboembolism infection protein malnutrition and hypovolemia.