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Immunoglobulin A Nephropathy

Immunoglobulin A nephropathy is the most common chronic glomerulonephritis. It is seen in older children and young adults, but no age has been spared immunoglobulin A nephropathy. There have been descriptions of babies with IgA nephropathy and old people with IgA nephropathy, but in our population it will be most likely an older child or young adult. Sixteen-year-old boy with a runny nose and a cough told his mother that when he went to the bathroom he saw that his urine was red. This was apparently ignored, and a few months later the boy was seen by his pediatrician because of a sore throat. During the exam he told the doctor that now he has red urine again. The blood pressure was 138/83, no edema, no rash or joint findings. The urine was tea-colored with too numerous to count red blood cells, and 3+ protein. On blood test, complements were normal, ANA was negative, creatinine 1, and BUN 25. So this IgA nephropathy has no low complements. Complements are normal. No serology really, no serologic test that diagnoses IgA, and creatinine at diagnosis can be normal.

The biopsy; it is more prevalent in males, but not Ö some studies show 15:1, but really itís not that high in most studies. The most important feature is the significant hematuria; the finding of hematuria during the time of the upper respiratory infection. Thatís the most important thing to remember.

The diagnosis is by finding of mesangial deposition of IgA in the biopsy. So the biopsy is the sine quo non of this disease. You cannot make it by serological testing or any other way, other than elimination of other things and definitively by a biopsy. There is no known treatment that we know will treat this, and there is a big study ongoing right now trying to determine which is better, but weíve tried lots of things and currently we use steroids, sometimes another immune suppression to spare the steroid side effects, and some people have now shown that fish oil.

In terms of predictive features that predict a worse outcome, are males, older age at onset, heavy proteinuria at the time of diagnosis, hypertension, and obviously the worse the biopsy looks, the worse the disease is.

Systemic Lupus Erythematosus. In terms of renal manifestations, I already mentioned that again it is nephrotic/nephritic urine. Both nephrotic range proteinuria or non-nephrotic range proteinuria, but also hematuria with red blood cells and red blood cell casts. Thatís called nephrotic/nephritic or nephritic/nephrotic. They have hypertension and decreased renal function because itís a chronic progressive glomerulonephritis as well. The pathology ranges between nothing, normal pathology.

The treatments are different, and I wonít go into details about treatment, but the findings in urine and blood are pretty self-evident. Weíve already mentioned them several times. In blood tests there will be the findings of lupus, anemia, leukopenia and thrombocytopenia with a positive VDRL, which is really a false positive.

You of course will have most of the time, in first screening, anti-nuclear antibodies and then get these more specific ones, and hypo-complementemia. Both C-3 and C-4 in lupus are low in the active disease. They can go back up to normal when the disease is more in remission. Circulating immune complexes and sometimes there are

In terms of treatment, again, there are different treatments to the different pathologies. The worst pathology, diffuse proliferative glomerulonephritis, requires high dose IV prednisone or Solu-Medrol and Cytoxan.

In terms of prognosis, before we even had steroids, which none of us I think were in that era, there was a dismal prognosis with a five-year survival less than 10%. Nowadays, thankfully, the lupus patients donít die of renal failure.

Alportís has an association with deafness and the familial hematuria, leading to renal failure. And remember the HSP, which is what we see in kids, as another spectrum. The HSP, which is IgA in terms of pathology, but with a rash and the joint involvement. So those would be things that are more Ö that would be reasonable to be in the test.

Urinary Tract Infections. In the neonatal age, the predominance of males have UTIís. Thatís an important feature that then changes when they get older, to much much higher predominance in girls. But in the neonatal age, more boys than girls have UTIís. For instance, the most common cause - no matter what age - is by far E. coli. So there is no hesitation about the answer to whatís the most common etiology. It doesnít matter the age or the gender.