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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 ]
[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]
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 ...
FSGS accounts for 35% of all cases of nephrotic syndrome, making it one of the most common causes of nephrotic syndrome in the United States. [8] FSGS accounts for 2% of all cases of kidney failure. [4] African American patients have four times the likelihood of developing FSGS. Men are about two times as likely to develop FSGS compared to ...
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.
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]
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 .
Glomerulonephrosis is a non-inflammatory disease of the kidney presenting primarily in the glomerulus (a glomerulopathy) as nephrotic syndrome. The nephron is the functional unit of the kidney and it contains the glomerulus, which acts as a filter for blood to retain proteins and blood lipids.