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The Inaugural MTI Humanitarian Award Celebration Honoring Alonzo Mourning

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Our Research - Kidney & Pancreas Transplantation


PROJECT 2: The recurrence of proteinuria with focal segmental glomerulosclerosis in children and young adults

PROJECT SUMMARY:

We are actively investigating the recurrence of proteinuria, specifically in children and young adults with focal segmental glomerulosclerosis (FSGS).  This is an unusual disease where immediately after kidney transplantation the recipient often begins to spill protein in the urine.  From January 2000-December 2003, we observed a rate of recurrence of 80% (similar to published literature) in our pediatric population with FSGS subsequent to kidney transplantation.  Since January 2004, we have treated these patients with a novel induction regimen, including rituximab, a monoclonal antibody.  We reasoned that rituximab may play a role in stabilization of the podocyte (a key cellular element of the kidney glomerulus) at the time of transplantation.  Since January 2004, we have used this protocol on approximately 25 patients and have observed a remarkable decrease in the incidence of proteinuia after transplant as well as the need for plasmapheresis after transplant.  We are currently investigating the mechanisms for this and are pursuing ultimate therapies for this difficult and challenging clinical problem.  We have a series of abstracts and are currently working on federal grants and publications. 

What is Focal Segmental Glomerulosclerosis (FSGS)?

Hyperglycemia, or high blood glucose (sugar), is a serious health problem for those with diabetes.  Hyperglycemia develops when there is too much sugar in the blood.  In people with diabetes, there are two specific types of hyperglycemia that occur.

"Focal" means that some of the glomeruli become scarred, while others remain normal. "Segmental" means that only part of an individual glomerulus is damaged.

The cause of focal segmental glomerulosclerosis is usually unknown.  A small number of cases result from reflux nephropathy.  The condition affects both children and adults. Males are affected slightly more often than females, and it also occurs more frequently in African-Americans.

Focal segmental glomerulosclerosis causes about 10 - 15% of all cases of nephrotic syndrome.

GOAL:

Ultimately, we hope that this research will translate to a cure for children with this unusual disease before they develop renal failure and need to be transplanted.