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Childhood-onset nephrotic syndrome is a kidney disorder characterized by severe proteinuria (excess proteins in the urine), hypoalbuminemia (low albumin levels in the blood), and edema (swelling) that affects approximately 2-7 per 100,000 children under 18 years of age annually.
[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 ...
Nephrotic syndrome can affect any age, although it is mainly found in adults with a ratio of adults to children of 26 to 1. [ 62 ] The syndrome presents in different ways in the two groups: the most frequent glomerulopathy in children is minimal change disease (66% of cases), followed by focal segmental glomerulosclerosis (8%) and ...
Patients with nephrotic-range (>3.5 g/day) proteinuria have over a 50% rate of progression to end-stage kidney disease at 10 years. [6] Only 15% of patients with sub-nephrotic ranges of proteinuria progress to end-stage renal failure at 10 years. [6] Initial response to therapy also dictates long-term outcomes.
Primary forms of glomerulonephrosis can occur at any age, although it is found in adults more commonly than children. The most common cause of glomerulonephrosis in children is Minimal Change Disease where they make up a majority of cases. There are 10-50 cases per 100,000 children, with male cases being twice as common as female cases. [16]
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.
The term childhood disease refers to disease that is contracted or becomes symptomatic before the age of 18 or 21 years old. Many of these diseases can also be contracted by adults. Some childhood diseases include: