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Childhood-onset nephrotic syndrome differs from the adult nephrotic syndrome in that the former often has a single common cause that typically responds well to steroid treatment. In adults, there are numerous potential causes, which makes an early kidney biopsy necessary to determine the correct diagnosis and treatment plan. [ 4 ]
In nephrotic syndrome, protein loss can be as great as 3.5 grams over 24 hours, much of which is albumin, itself leading to hypoalbuminemia. [3] In children, nephrotic syndrome is commonly a primary disease process that is largely idiopathic, although more genetic causes are being identified with the cost and accessibility of whole exome ...
Minimal change disease (MCD), also known as lipoid nephrosis or nil disease, among others, is a disease affecting the kidneys which causes nephrotic syndrome. [1] Nephrotic syndrome leads to the loss of significant amounts of protein to the urine (proteinuria), which causes the widespread edema (soft tissue swelling) and impaired kidney function commonly experienced by those affected by the ...
Primary causes of nephrotic syndrome are usually described by their histology: [21] Minimal change disease (MCD): is the most common cause of nephrotic syndrome in children. It owes its name to the fact that the nephrons appear normal when viewed with an optical microscope as the lesions are only visible using an electron microscope .
[1] [3] NPHS1 mutations are the most common cause of primary congenital nephrotic syndrome, accounting for 40-80% of cases. [1] NPHS2: This gene encodes for the protein podocin. [1] Patients with this genetic mutation develop nephrotic syndrome in the first few weeks of infancy, but can also manifest symptoms later in life. [3]
Presentation of nephrotic syndrome in the context of mesangial proliferative glomerulonephritis have been treated with immunosuppressants, such as steroids and cyclophosphamide. [2] Presentation with nephrotic syndrome can resolve with treatment, but can also progress. [10]
By contrast, nephrotic syndrome is characterized by proteinuria and a constellation of other symptoms that specifically do not include hematuria. [6] Nephritic syndrome, like nephrotic syndrome, may involve low level of albumin in the blood due to the protein albumin moving from the blood to the urine. [7]
MPGN accounts for approximately 4% of primary renal causes of nephrotic syndrome in children and 7% in adults. [3] It should not be confused with membranous glomerulonephritis, a condition in which the basement membrane is thickened, but the mesangium is not.