Make you a better understanding of IgA nephropathy

IgA nephropathy

Blood in the urine (hematuria) occurs in 10% of children on occasion. It may be microscopic, detected only with a dipstick or high magnification. It may be gross, with urine that looks like cola, red wine, or cherry Kool Aid. Sometimes kids have microscopic hematuria with intermittent episodes of gross blood. These children often have IgA nephropathy (IgAN).

There are a lot of different ways IgA Nephropathy can present. Here are some of them:

1. Asymptomatic hematuria: a lot of cases are picked up with routine urinalyses. Often these cases have little to no progression of renal disease over several decades.

2. Synpharyngitic hematuria: the "classic" presentation of IgA Nephropathy occurs within 1-3 days of a mucosal infection such as a URI.

3. Acute kidney injury: occasionally, IgA Nephropathy will first manifest as AKI. This can take 2 basic forms, and distinguishing between the two of them is critical:
A) The ATN Form: patients may become oliguric as a result of an acute worsening of hematuria, which can result in tubular toxicity, but will almost always recover renal full renal function afterwards.
B) The crescentic Form: patients with crescents on the background of mesangial IgA deposits on renal biopsy have a very poor prognosis and will often progress to permanent renal damage even despite aggressive therapy.

4. Nephrotic Syndrome: as mentioned above, IgA Nephropathy accounts for about 14% of all nephrotic syndrome in whites. This can also take two different forms:
A) "Minimal Change Disease" superimposed on IgA Nephropathy, which tends to be very steroid responsive.
B) "Structural Damage"--severe, prolonged damage to mesangial cells can result in secondary podocyte injury that is generally not reversible and can be thought of as a secondary nephrotic syndrome.

The course of IgAN varies from limited progression (or even some episodes of spontaneous remission) to rapidly progressive kidney failure. Kidney function measurements guide management:

Estimated glomerular filtration rate (eGFR)
Blood pressure (BP)
Protein:Creatinine ratio (P:C)
Nephrologists reserve biopsy, an invasive procedure, for children with abnormalities of at least one of these measures of kidney function, or children with multiple episodes of gross hematuria. Usually patients need no treatment if all measures of kidney function are normal, other than annual observation.

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