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Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
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IntroductionThe mitral valve, one of the heart's four valves, regulates blood flow. These valves have flaps that open and close to direct blood properly through the heart and body. During each heartbeat, the flaps open for blood to pass through and seal shut to prevent backflow. Specifically, the mitral valve opens to allow blood flow from the heart's upper left chamber to the lower left chamber. It then closes securely as the lower left chamber contracts to pump blood to the body, preventing...
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Related Experiment Video

Updated: Mar 1, 2026

Mechanism of Kemeng Fang's Inhibition of Podocyte Apoptosis in Rats with Membranous Nephropathy through the PI3K/AKT Signaling Pathway
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Primary Membranous Nephropathy.

William G Couser1

  • 1Division of Nephrology, Department of Medicine, University of Washington, Seattle, Washington.

Clinical Journal of the American Society of Nephrology : CJASN
|May 28, 2017
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Summary

Primary membranous nephropathy (PMN) is often linked to anti-PLA2R antibodies. Early supportive care is crucial, with immunosuppression considered for persistent proteinuria or complications, improving outcomes and reducing kidney failure risk.

Keywords:
AdultB-LymphocytesBiopsyCalcineurin InhibitorsChronicCyclophosphamideGlomerulonephritisHumansImmunoglobulin GKidney FailureKidney GlomerulusLipoidMembranousNephrosisPLA2RPLA2R1 proteinPhospholipase A2PodocytesReceptorsRemissionRenal InsufficiencySpontaneousStaining and LabelingTHSD7Acongenitalhumanmembranous nephropathynephrotic syndromeproteinuria

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Area of Science:

  • Nephrology
  • Immunology
  • Glomerular Diseases

Background:

  • Membranous nephropathy (MN) is a leading cause of nephrotic syndrome in adults.
  • Primary MN (PMN) constitutes 80% of cases, often involving autoantibodies.
  • Key autoantibodies include IgG4 anti-PLA2R and anti-THSD7A.

Purpose of the Study:

  • To review the understanding of primary membranous nephropathy (PMN).
  • To discuss the pathogenic role of autoantibodies in PMN.
  • To outline diagnostic and therapeutic strategies for PMN.

Main Methods:

  • Review of clinical and experimental data on PMN.
  • Analysis of autoantibody prevalence (PLA2R, THSD7A) and diagnostic implications.
  • Evaluation of treatment outcomes with supportive care and immunosuppression.

Main Results:

  • Anti-PLA2R antibodies are found in ~70% of PMN cases; anti-THSD7A in 3-5%.
  • Untreated PMN shows spontaneous remission in one-third, progression to ESRD in one-third, and CKD in the remainder.
  • Supportive care is standard; immunosuppression benefits patients with significant proteinuria or complications.

Conclusions:

  • Autoantibodies like anti-PLA2R are pathogenic drivers of PMN.
  • Timely diagnosis and management, including judicious use of immunosuppression, can prevent ESRD.
  • Effective treatment strategies have reduced the long-term risk of end-stage renal disease (ESRD) in PMN patients.