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Kidney Week

Abstract: TH-PO961

Is Liposorber an Option for Lipoprotein Glomerulopathy in Pediatric African American Males?

Session Information

Category: Trainee Case Report

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Gor, Juhi A., Brody School of Medicine at East Carolina University, Greenville, North Carolina, United States
  • Hidalgo, Guillermo, Eastern Carolina University, Greenville, North Carolina, United States
Introduction

Lipoprotein glomerulopathy is a rare inherited renal disease that is characterized by the accumulation of lipoproteins leading to the formation of lipoprotein thrombi with markedly dilated glomerular capillaries. The disorder is associated with an ApoE mutation leading to massive proteinuria and dyslipidemia with high chance of progression to chronic kidney disease. The first publication of a case was reported in 1989 by Saito, and has since been commonly reported in adult individuals of Japanese and Chinese descent.

Case Description

Interestingly, our report looks into the presentation of lipoprotein glomerulopathy in a 7 year old African American male who has been followed since birth for hypertension, hypercholesterolemia, nephrotic range proteinuria, and multiple past episodes of prerenal AKI. A renal biopsy was performed and diagnosis of lipoprotein glomerulopathy was confirmed by histological diagnosis from Vanderbilt. Genetic testing revealed a heterogeneous mutation of ApoE2. The patient also presents with hemihyperplasia of the right lower extremity, increasing our suspicion for a possible accompanying WT1 gene mutation. Treatment has been focused on controlling hypertension and symptoms of chronic kidney disease. Patient has been taking a beta blocker, angiotensin converting enzyme inhibitor, and calcium channel blockers for hypertension. These medications have been effective in reducing the blood pressure from 140-150/100 to 120-130/80. To treat for hypercholesterolemia that imposes continued damage on the kidneys of patients with this disorder, he has been taking fibrates and statins, but his cholesterol levels continue to be elevated, while triglyceride levels are well controlled. Further treatment evaluation is being focused on the possibility of beginning patient on Liposorber once per month to remove the high levels of LDL. Kidney transplant consideration is low due to recurrence being reported in all past transplanted kidneys.

Discussion

This case illustrates the possible expansion of a rare renal disease outside of commonly targeted population. Recognition of lipoprotein glomerulopathy and its clinical features will become critical in future evaluation of nephrotic syndromes in the pediatric population.