Complete remission of nephrotic syndrome in a young woman with anti-LRP2 nephropathy after immunosuppressive therapy.

BACKGROUND: Anti-low density lipoprotein receptor-related protein 2 (LRP2) nephropathy/anti-brush border antibody (ABBA) disease is a disorder characterized by acute tubulointerstitial injury associated with circulating antibodies to kidney proximal tubular brush border protein LRP2/megalin. Patients are typically elderly and present with acute kidney injury and subnephrotic proteinuria. They progress to end-stage renal disease with poor response to immunosuppressive therapies. CASE PRESENTATION: We report a case of a 29-year-old Chinese woman, who presented with nephrotic syndrome with normal kidney function. Kidney biopsy showed no obvious tubular injury or interstitial inflammation. Positive immunoglobulin G (IgG) staining was revealed along the brush border of proximal tubular cells. Anti-LRP2 antibody was identified in serum, consistent with a diagnosis of anti-LRP2 nephropathy. The patient achieved complete remission after receiving prednisone and cyclophosphamide. CONCLUSIONS: Anti-LRP2 nephropathy can also present as nephrotic syndrome in young patients and complete remission from nephrotic syndrome may be achieved after immunosuppressive therapy.

Effect of long-term use of unfractionated or low-molecular-weight heparin on bone mineral density in maintenance hemodialysis patients.

INTRODUCTION: Long-term use of unfractionated heparin (UFH) or low-molecular-weight heparin (LMWH) causes bone loss and osteoporosis in patients not receiving hemodialysis. This study aimed to investigate the effect of UFH and LMWH on bone mineral density (BMD) in patients undergoing maintenance hemodialysis (MHD). METHODS: Patients undergoing MHD using UFH or LMWH as anticoagulants were enrolled. BMD (in g/cm2 ), T-score and Z-score (BMDs) were measured at the lumbar spine and femur neck using dual-energy X-ray absorptiometry (DXA) at baseline and 2 years later. Patient demographics and clinical indices were collected. Correlation analysis was used to identify significant predictors of bone loss. Multiple linear regression was used to explore the relationship between heparin type and bone loss progression. FINDINGS: A total of 104 patients were enrolled and completed the baseline BMD test; 72 completed the test again 2 years later. Six patients were excluded because they used both UFH and LMWH. Although BMD decreased in some patients in the UFH group, a slight increase in the BMD was observed on an average in the LMWH group after 2 years. The mean change in BMD (in g/cm2 ) [0(-0.03,0.04) vs. 0.04(0,0.06), P = 0.023], T-score [0(-0.40,0.30) vs. 0.35(-0.03,0.53), P = 0.038], and Z-score [0.10(-0.30,0.40) vs. 0.45(0.08,0.63), P = 0.031] in the lumbar spine in the UFH group was lower than those in the LMWH group. Femur neck BMD did not change significantly. In a linear regression model, after adjusting for diabetes mellitus, parathyroid hormone, and serum phosphate, we did not find an association between heparin substances and BMD. DISCUSSION: UFH might be associated with loss of lumbar spine BMD in patients undergoing MHD.