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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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IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document...
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Antigenic Liposomes for Generation of Disease-specific Antibodies
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Lipoprotein Glomerulopathy, First Case Report from Canada.

Julie Anne Ting1, Susanna A McRae1,2, Daniel Schwartz1

  • 1Division of Nephrology, University of British Columbia, Vancouver, BC, V5Z 1M9, Canada.

International Journal of Nephrology and Renovascular Disease
|June 28, 2022
PubMed
Summary
This summary is machine-generated.

Lipoprotein glomerulopathy (LPG), a kidney disease caused by apolipoprotein E gene mutations, was diagnosed in Canada. Treatment with fenofibrate and perindopril resolved lipoprotein thrombi in this first North American case of the APOE Tokyo/Maebashi mutation.

Keywords:
apoE Tokyo/Maebashiapolipoprotein Edyslipidemiafibratelipoprotein glomerulopathyproteinuria

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Area of Science:

  • Nephrology
  • Genetics
  • Internal Medicine

Background:

  • Lipoprotein glomerulopathy (LPG) is a rare kidney disease linked to apolipoprotein E (APOE) gene mutations.
  • It presents with lipoprotein thrombi obstructing glomerular capillaries.

Observation:

  • A 49-year-old man with dyslipidemia and hypertension presented with proteinuria.
  • Renal biopsy revealed lipoprotein thrombi within dilated glomerular capillaries.
  • APOE gene sequencing identified the Tokyo/Maebashi mutation (p.Leu162_Lys164del).

Findings:

  • This is the first reported case of LPG in Canada and North America with the APOE Tokyo/Maebashi mutation.
  • Treatment with fenofibrate and perindopril normalized lipid profiles and reduced proteinuria.
  • Follow-up biopsy showed resolution of thrombi, with residual subendothelial material suggesting precursor lipoproteins.

Implications:

  • This case expands the geographic and genetic spectrum of LPG.
  • It highlights the efficacy of lipid-lowering and antihypertensive therapy in managing LPG.
  • The findings support the role of subendothelial lipoproteins as precursors to thrombus formation in LPG.