Hildebrandt

Kaskel

The Genetics of FSGS.   
Friederich Hildebrandt MD, and Frederick J. Kaskel, MD, PhD.
(ASN Renal Week Clinical Nephrology Conference, November, 2007.)
Hildebrandt: What are the most common point mutations that cause FSGS and childhood nephrotic syndrome. How and when do they present? To what extent do they respond to treatment? Are any of them steroid responsive? Are they associated with recurrence post-transplantation? Where can one turn for gene analysis when an inherited mutation is suspected in a child with nephrotic syndrome? What are the characteristics of PLCE1 mutations (phospholipase C epsilon), and what should the approach to therapy of these patients be? Kaskel: What are predictors of outcome in FSGS? What are the general treatment approaches? How should steroids be used? Does cyclophosphamide or chlorambucil have a role? What do randomized trials show in terms of cyclosporine? What is the experience with MMF? With rapamycin? Pulse dexamethasone? What is the design of the ongoing NIH/NIDDK Study and when will we have results? When is plamapheresis indicated? What do preliminary results with rituximab show? (more...)


Columbia University

Biopsy Conference: A middle-aged male with new onset nephrotic syndrome
(Columbia University Nephrology and Pathology Faculty; January, 2008)

A middle-aged male had hyperlipidemia, heavy smoking history, hypovitaminosis D, no DM, no HTN, no family history of renal disease. Taking furosemide, simvastatin, lisinopril, vitamin D. Presents with pedal edema and 13g/day proteinuria. Serum albumin is 2.2 g/dL. Complements and serologies are normal. Is it membranous GN? FSGS? Minimal change disease? Amyloidosis? Discussion focus: Thin basement nephropathy and membranous nephropathy.
(Edited by Jai Radhakrishnan, MD). (more...)


Updated: November 27, 2008 (N27)
N27 JASN early release: Imatinib suppresses cryoglobulin production in mice.
N04 NK-22 cells - they're in the gut, they make IL-22, and are involved in mucosal immunity.
N04 1-alpha-25OH-D hydroxylase active in the lung; may be involved in immune defense.
N04 Strange, complex pharmacokinetics of methotrexate in RBCs have implications for clinical treatment.
O29 Patients with lupus being treated with hydroxychloroquine may have lower rate of kidney damage in observational study.
O23 European watchdog agency suggests not using diet drug rimonabant due to depression side effects.
O22 Low-levels of gut bacteria that secrete anti-inflammatory compounds may underlie or worsen Crohn's disease.
O17 New Mycobacterium species identified that can cause bone infections in the immunocompromised.
O07 NRAA: Advance copy of ESRD program interpretive guidelines. (pdf)
O07 Gut bacteria may protect mice against type 1 diabetes, other autoimmune disorders.
O03 DNA of commensal, good bacteria augment the intestinal response to infection.
S19 African American women have low white blood cell count.
S15 MYH9 gene variants linked to FSGS and diabetic kidney disease in African Americans.
S11 Rituximab prescribing info revised to reflect risk of progressive multifocal leukoencephalopathy.
S10 Molecular mechanism of steroid-induced fatty liver determined.
General Advances Emphasis
archive
Membranoproliferative Glomerulonephritis Pathology Update (Walker) ASN 2005 Renal Week
Membranoproliferative Glomerulonephritis: Clinical Spectrum (Glassock) ASN 2005 Renal Week
The Role of Podocytes in Proteinuria and Progressive Renal Disease (Kriz, Salant) ASN 2005 Renal Week (audiofiles)
New Insights in IgA Nephropathy (Haas, Monteiro, Feehally, Hsu) ASN 2005 Renal Week (audiofiles)
Antibody Mediated Responses in Allo and Autoimmunity (Wasowska) ASN 2005 Renal Week (audiofiles)
Renal Biopsy: Clinical Correlations (Walker, Smith, Chander, Barisoni) ASN 2005 Renal Week (audiofiles)
Non-Amyloid Dysproteinemias and the Kidney (Ronco, Korbet, Madore) ASN 2005 Renal Week (audiofiles)
Issues in Lupus Nephritis (Rifkin, Levine, Austin Appel) ASN 2005 Renal Week (audiofiles)
Management Issues in Systemic Lupus Erythematosus (Austin, Parfrey, Cohen) ASN 2005 Renal Week (audiofiles)
Renal Amyloidosis: Pathogenesis, Diagnosis, and Therapy (Benson, Herrera, Seldin) ASN 2005 Renal Week (audiofiles)
Progression and Treatment of Fibrosis (Bissell) ASN 2005 Renal Week (audiofiles)
Collapsing Glomerulopathy - A Distinct Pathogenic Entity (Barisoni, Kopp, Nelson) ASN 2005 Renal Week (audiofiles)
Basic Science for Clinical Nephrologists - Mechanisms of Injury in Glomerulonephritis: A Review and Update (Julian, VanderWoude, Botto) ASN 2005 Renal Week (audiofiles)
Why is TNF a Good Therapeutic Target? (Feldman) ASN 2005 Renal Week (audiofiles)
Glomerulonephritis Update: Diagnosis and Therapy (Part 1 and 2) (Appel, Rennke, Cattran, Falk, Korbet, Glassock) ASN 2005 Renal Week (audiofiles)
Glomerulonephritis Update: Diagnosis and Therapy (Part 3 and 4) (Falk, Pusey, Appel, Korbet, Kupin, Charytan) ASN 2005 Renal Week (audiofiles)
Infectious Diseases After Immunosuppression in the Non-Transplant Kidney Patient Population (Brennan) ASN 2004 Renal Week
Non-Standard Therapy in Membranous Nephropathy (Remuzzi) ASN 2004 Renal Week
Therapy of ANCA-Associated Vasculitis and EUVAS Trials (Jayne) ASN 2004 Renal Week
ANCA-associated RPGN: Role of Plasmapheresis (Pusey) ASN 2004 Renal Week
Indications for Immunosuppression in Membranous Nephropathy (Cattran) ASN 2004 Renal Week
Advances in the Treatment of Focal Glomerulosclerosis (FSGS) (Kaskel) ASN 2004 Renal Week

Idiopathic membranous nephropathy: diagnosis and treatment Clin J Am Soc Nephrol (May 2008)
Rituximab treatment of idiopathic membranous nephropathy Kidney Int (Jan 2008)
Rapid remission of steroid and mycophenolate mofetil-resistant minimal change nephrotic syndrome after rituximab therapy Nephrol Dial Transplant (Jan 2008)
Inherited diseases of the glomerular basement membrane Nature Clin Pract Nephrol (Jan 2008)
Renal transplantation in the ANCA-associated vasculitides (Geetha et al) Am J Transplant (Dec 2007)
Quantitative appraisal of treatment options for IgA nephropathy (Ballardie et al) J Amer Soc Nephrol (Nov 2007)
Prophylactic anticoagulation in nephrotic syndrome: a clinical conundrum (Glassock) J Am Soc Nephrol (Aug 2007)
ANCA are detectable in nearly all patients with active severe Wegener's Granulomatosis (Finkielman et al) Am J Med (Jul 2007)
Renal manifestations of plasma cell disorders (Leung et al) Am J Kidney Dis (Jul 2007)
Why are rheumatologists treating lupus nephritis? (Bargman JM) NCPN Jun 07
Pathogenesis and prevention of bone loss in patients who have kidney disease and receive long-term immunosuppression. JASN (01-07)
Lupus nephritis: Toll the trigger! (KD Smith, commenting on a study by Pawar et al). JASN 12-06
Recurrent glomerulonephritis after kidney transplantation (Choy, Chan, Lai) Am J Transplant 11-06
Glomerulonephritis Links
GENERAL
Am Coll Rheumatology
Arthritis Foundation
Clinical Trials.gov
CenterWatch trial list
LUPUS
Lupus Foundation
NIAMS Lupus Patient info
Wegener's
Wegener Foundation
FSGS
NephCure Foundation
Vasculitis
INSSYS
Cleveland Clinic Vasculitis Ctr
Johns Hopkins
Pathology
Atlas Renal Pathology
HDCN Topic Pages (KEY)
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