Adult_MCD[1] - Rash ADULT ONSET MINIMAL CHANGE DISEASE...

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ADULT ONSET MINIMAL CHANGE DISEASE Nephrology Grand Rounds Aditya Mattoo, MD October 20 th , 2009
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Outline Background Pathophysiology Etiologies IgA and MCD Clinical Findings Treatment Prognosis
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BACKGROUND
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Background inimal change disease (MCD) is also known as nil disease. inimal change disease is defined by nephrotic syndrome with normal appearing light microscopy with foot process effacement on electron microscopy in the absence of cellular infiltrates or immune deposits.
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Background ow levels of mesangial IgM and/or C3 without ultrastructural evidence for electron dense deposits is acceptable for a diagnosis of minimal change glomerulopathy. gA mesangial deposition is a rare occurrence and whether or not it represents a pathological or a coincidental finding is uncertain.
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Electron Microscopy A. Normal podocyte foot processes B. MCD with podocyte foot process effacement
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Background ost common form of nephrotic syndrome in children. n children younger than 10 years, MCD makes up to 90% of all cases of nephrotic syndrome. n adolescents above the age of 10, MCD accounts for 50% of nephrotic cases. hile in adults, MCD accounts for 10-15% of primary
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Background n children, MCD is found twice as frequently in boys than in girls. he frequency is the approximately the same between the sexes in adults. he incidence peaks in children at age with approximately 80% being younger than 6 years at the time of diagnosis. Waldman et al. CJ ASN 2: p445, 2007.
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PATHOPHYSIOLO GY
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Pathophysiology he underlying cause of MCD is still uncertain, however, evidence points to T-cell dysfunction as a major player. irst postulated by Shalhoub in 1974, this theory (also known as the Shalhoub hypothesis) is supported by the following observations: emissions of MCD occur in the setting of a measles infection where viral associated immunosuppression occurs. CD occurs more frequently in patient’s with lymphoma.
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Pathophysiology he following observations support the possibility of a circulating “permeability factor” of immune origin which alters glomerular podocyte permeability causing proteinuria: T-cell hybridoma made from patient with MCD released a substance that when injected into rats, induced proteinuria and foot process effacement. wo kidneys of a young donor with presumptive MCD (never biopsied) were transplanted into two recipients without baseline proteinuria. Proteinuria diminished rapidly in both recipients and was absent by week six. Koyama A et al. KI 40: p453, 1991. Ali AA et al. Transplantation 58: p849, 1994.
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Permeability Factor – IL-13 ne of the leading permeability factor suspects is IL- 13. L-13 is known to be an autocrine growth factor for
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Adult_MCD[1] - Rash ADULT ONSET MINIMAL CHANGE DISEASE...

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