Bruce Langberg and his younger brother Gabriel Langberg were both diagnosed with Focal Segmental Glomerulosclerosis (FSGS) in 2002. The foundation was created by Bruce Langberg in 2007 in order to fundraise for the following causes:
Supporting FSGS research for a CURE!
- Educating kidney patients about medical resources, and local support networks.
VIDEO ON FSGS
ABOUT THE PROBLEM:
"Focal segmental glomerulosclerosis (FSGS) describes scarring in scattered regions of the kidney, typically limited to one part of the glomerulus and to a minority of glomeruli in the affected region. FSGS may result from a systemic disorder or it may develop as an idiopathic kidney disease, without a known cause. Proteinuria is the most common symptom of FSGS, but, since proteinuria is associated with several other kidney conditions, the doctor cannot diagnose FSGS on the basis of proteinuria alone. Biopsy may confirm the presence of glomerular scarring if the tissue is taken from the affected section of the kidney. But finding the affected section is a matter of chance, especially early in the disease process, when lesions may be scattered.
Confirming a diagnosis of FSGS may require repeat kidney biopsies. Arriving at a diagnosis of idiopathic FSGS requires the identification of focal scarring and the elimination of possible systemic causes such as diabetes or an immune response to infection. Since idiopathic FSGS is, by definition, of unknown cause, it is difficult to treat. No universal remedy has been found, and most patients with FSGS progress to total kidney failure over 5 to 20 years. Some patients with an aggressive form of FSGS reach total kidney failure in 2 to 3 years. Treatments involving steroids or other immunosuppressive drugs appear to help some patients by decreasing proteinuria and improving kidney function. But these treatments are beneficial to only a minority of those in whom they are tried, and some patients experience even poorer kidney function as a result. ACE inhibitors and ARBs may also be used in FSGS to decrease proteinuria. Treatment should focus on controlling blood pressure and blood cholesterol levels, factors that may contribute to kidney scarring." **
Pathology report for Bruce Langberg in 2002:
This 23 year old male has a history of recently discovered nephritic range proteinuria without other significant medical history. His family history is positive for multiple family members with diabetes and multiple paternal family members with renal disease, including a great-grandfather who died of renal disease and grandfather who was diagnosed with focal segmental glomerulosclerosis on a previous renal biopsy. The clinical history of nephritic range proteinuria (5.4 grams per 24 hours) with relatively well preserved renal function (serum creatine = 1.2 mg/dl) coupled with the current biopsy findings are best explained by focal segmental glomerulosclerosis (FSGS).
FSGS is characterized by focal glomerular lesions of segmental tuft collapse with GBM wrinkling; adhesions; mesangial matrix expansion (sclerosis); widespread glomerular foot process effacement; segmental obsolescent-type IgM and C3 deposits; and usually both glomerular and arteriolar hyalinosis. This case demonstrates all of these histologic findings. The absence of significant longstanding hypertension, hyperfiltration or other progressive glomerulopathy in this case suggests primary idiopathic FSGS that may be familial in origin. Currently, chronicity is judged to be mild based on the degree of focal interstitial scarring and the overall level of glomerular obsolescence (15-20%). None of this chronic damage can be attributed to the chronic affects of HTN.
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** http://kidney.niddk.nih.gov/kudiseases/pubs/glomerular/
The Paradise, CA kidney foundation is A private foundation exempt under section 501(c)(3) currently in the application process