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Alport syndrome is a genetic disorder [1] affecting around 1 in 5,000–10,000 children, [2] characterized by glomerulonephritis, end-stage kidney disease, and hearing loss. [3] Alport syndrome can also affect the eyes, though the changes do not usually affect vision, except when changes to the lens occur in later life. Blood in urine is universal.
Affected children have rapid decline in kidney function resulting in end-stage renal disease within the first years of life, and require treatment with dialysis and kidney transplantation. [ 1 ] [ 4 ] Most children live fairly normal life post-transplant but will spend significant time hospitalised pre-transplant and have numerous surgeries to ...
Nephritic syndrome is a syndrome comprising signs of nephritis, which is kidney disease involving inflammation. It often occurs in the glomerulus, where it is called glomerulonephritis. Glomerulonephritis is characterized by inflammation and thinning of the glomerular basement membrane and the occurrence of small pores in the podocytes of the ...
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 ...
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.
Medical specialty professional organizations recommend that physicians do not perform routine cancer screening in people with limited life expectancies due to ESKD because evidence does not show that such tests lead to improved outcomes. [95] [96] In children, growth failure is a common complication from CKD.
Loss of necessary protein due to nephritis can result in several life-threatening symptoms. The most serious complication of nephritis can occur if there is significant loss of the proteins that keep blood from clotting excessively. Loss of these proteins can result in blood clots, causing sudden stroke. [16]
The symptoms and the severity of these symptoms vary between patients where most patients experience nephritis in childhood and then progress to kidney failure in adolescence. [5] In macrothrombocytopenia platelet sizes can reach to approximately 6.6 um compared to a normal platelet size of 2.5 um where 30% platelets can reach the size of an ...