new_n_lost
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A 13-year-old girl is brought to the doctor’s office by her anxious parents. They are very concerned about the change in her urine color; in fact, it seems that there is blood in it. The child has been in good health except for a recent respiratory tract infection. Physical examination reveals mild edema in the lower extremities and increased blood pressure. Examination of the skin is normal. Serum creatinine and blood urea nitrogen are also normal. Examination of the urine reveals gross hematuria and elevated protein. Antistreptolysin O assays are negative. Histologic examination demonstrates mesangial proliferation. Immunostaining is positive for deposition of IgA, C3 and properdin.
Which of the following is the most likely diagnosis?
(A) Acute post streptococcal glomerulonephritis
(B) Alport syndrome
(C) Berger disease
(D) Henoch-Schönlein purpura
(E) Minimal change disease
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| doc179
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| unique1
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Berger disease
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| new_n_lost
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Choice A: Post streptococcal glomerulonephritis is a distinct disease from Berger disease. It is mediated by the formation of IgG antibody complexes deposited in the glomeruli. Serum tests reveal antibodies to several streptococcal proteins. These patients only rarely progress to chronic renal failure. <?xml:namespace prefix = o ns = "urn:schemas-microsoft-com:office:office" />
Choice B: Alport syndrome is an X-linked disorder in the alpha-5 chain of the collagen type IV molecule. This disorder is associated with deafness.
Choice C: CORRECT: Deposits of IgA, C3, and properdin in the mesangium occurs in IgA nephropathy, also known as Berger disease. Berger disease initially presents in children, most commonly, following a respiratory tract infection. It can, however, occur following urinary or gastrointestinal tract infections. Patients may have a mild nephritic syndrome with edema and some proteinuria. The proteinuria is not to the level seen in nephritic syndrome. The gross hematuria only lasts a few days but often recurs a few months later. Although initial serum creatinine can be normal, chronic renal disease can occur in up to half of the patients after several decades.
Choice D: IgA deposits are seen in Henoch-Schönlein purpura; there are, however, purpuric lesions.
Choice E: Minimal change disease is the most common cause of nephrotic syndrome in children. It is not associated with deposition of IgA.
___________________
FORUM RULES-- Those who believe in telekinesis, raise my hand. I get enough exercise just by pushing my luck --P4U World.." The pure and simple truth is rarely pure and never simple."
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