A combined low-density lipoprotein apheresis and prednisone therapy for steroid-resistant primary focal segmental glomerulosclerosis in children

Hattori, Motoshi; Chikamoto, Hiroko; Akioka, Yuko; Nakakura, Hyogo; Ogino, Daisuke; Matsunaga, Akira; Fukazawa, Akira; Miyakawa, Sanpei; Khono, Miyuki; Kawaguchi, Hiroshi; Ito, Katsuhiko

doi:10.1053/j.ajkd.2003.08.012

Cited by 68 publications

(80 citation statements)

References 36 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In each case, following a rapid decrease in the LDL levels after initiating treatment with LDL-A, urinary protein excretion decreased dramatically after one month. The remarkable results observed in our cases are similar to those reported in other cases of LDL-A therapy in patients with PSL-resistant NS with focal segmental glomerular sclerosis (7). An in vitro study demonstrated that very lowdensity lipoproteins (VLDLs) induce a dose-dependent reduction in the number of specific binding sites for dexamethasone in cultured smooth muscle cells, which leads to improvements in hyperlipidemia.…”

Section: Discussionsupporting

confidence: 89%

“…Regarding LDL-A, the treatment procedures used in this study were similar to those mentioned in previous reports (6)(7)(8). In each case, following a rapid decrease in the LDL levels after initiating treatment with LDL-A, urinary protein excretion decreased dramatically after one month.…”

Section: Discussionsupporting

confidence: 65%

“…At this time, it would be premature to conclude why our patients achieved good clinical courses following LDL-A treatment. Generally, LDL-A therapy is reported to be effective in steroid resistant patients with focal segmental sclerosis (FSGS) (7,14). Muso et al concluded that among steroid refractory FSGS cases, early rapid reduction induced by LDL-A therapy results in good clinical outcomes (14).…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, LDL-A may upregulate the steroid binding of systemic cells for both high LDL and VLDL. LDL-A may also improve sensitivity to PSL, thus resulting in a good clinical course (7,9).…”

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

Low-Density Lipoprotein Apheresis Therapy for Steroid- and Cyclosporine-Resistant Idiopathic Membranous Nephropathy

et al. 2012

View full text Add to dashboard Cite

We herein report three cases of Nephrotic Syndrome (NS) with idiopathic membranous nephropathy (IMN) in which the patients were resistant to steroid (PSL) and cyclosporine (CyA) therapy. All three patients had a high risk of renal failure because of persistently high levels of proteinuria. We performed low-density lipoprotein apheresis (LDL-A) and achieved complete remission in two cases. The third patients developed NS relapse and a deteriorating renal function; however, withdrawal from dialysis therapy was achieved. There are no proven therapies for treating patients with PSL-and CyA-resistant IMN with acute deteriorating renal function or difficulties in maintaining fluid balance. We evaluated the treatment course and physiological mechanisms and reviewed similar cases in the pertinent literature. A 75-year-old woman was admitted to our hospital with tibial edema and severe proteinuria that had persisted for one year. She presented with hypoalbuminemia and hypoproteinemia. Her laboratory data were as follows: total protein (TP) 5.1 g/day, albumin (Alb) 2.5 g/dL, low-density lipoprotein cholesterol (LDL-Chol) 298 mg/dL and urinary protein (U-pro) 4.1 g/dL. A renal biopsy performed on day 2 revealed membranous nephropathy (Fig. 1a, b). Two courses of intravenous methylprednisolone (mPSL; 0.5 g/ day) were administered over three consecutive days starting on days 19 and 53. Treatment with 50 mg (1 mg/kg) of prednisolone (PSL) was initiated after mPSL therapy on day 22. Seventy-five mg of cyclosporine (CyA) twice daily was added to the PSL therapy on day 37. The patient's CyA trough level was 96 mg/dL. Despite treatment with two courses of mPSL therapy, PSL therapy and CyA therapy, the heavy proteinuria and hypoproteinemia persisted. LDL-A therapy was commenced on day 67 and repeated twice a week for three weeks. Gradually, the proteinuria decreased and the serum protein levels increased (Fig. 2). After day 201, the estimated urinary protein level was <0.3 g/day. Complete remission (CR) was maintained for almost two years after discharge without the need for immunosuppressive therapy. Case 2A 35-year-old man was admitted to our hospital with tibial edema and severe proteinuria. He had been previously hospitalized for thrombotic thrombocytopenic purpura (TTP) almost four years earlier. He had recovered completely even without steroid therapy and suffered no relapses until the present day. The tibial edema was noticed almost five months prior to admission. On admission, laboratory data

show abstract

Section: Discussionsupporting

confidence: 89%

Section: Discussionsupporting

confidence: 65%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Low-Density Lipoprotein Apheresis Therapy for Steroid- and Cyclosporine-Resistant Idiopathic Membranous Nephropathy

et al. 2012

View full text Add to dashboard Cite

show abstract

“…A specific type of pheresis therapy, LDL apheresis with an absorptive column, which has been used to treat familial hypercholesterolemia, has been shown to be effective in a small series of patients with FSGS [61]. This procedure is FDA approved for the management of primary and recurrent FSGS.…”

Section: Treatmentmentioning

confidence: 99%

Recurrent focal segmental glomerulosclerosis after kidney transplantation

2015

View full text Add to dashboard Cite

Focal segmental glomerulosclerosis (FSGS) is an important cause of glomerular disease in children and adolescents and nearly 50 % of affected patients will progress to endstage kidney disease over a 5 to 10-year period. Unfortunately, there is no established treatment for disease in the native kidney. Moreover, up to 55 % of patients develop recurrent disease after receiving a kidney transplant, with a substantially higher risk in patients who have already experienced recurrent disease in a prior transplant. A number of clinical and laboratory factors have been identified as risk factors for this complication. In addition, new investigations into podocyte biology and circulating permeability factors have shed light on the cause of recurrent the disease. While a number of novel therapeutic agents have been applied in the management of this problem, there still is no proven treatment. In this review, we summarize recent advances in the epidemiology, pathophysiology, and treatment of recurrent FSGS in pediatric patients who have received a kidney transplant.

show abstract

Apheresis therapy in children: An overview of key technical aspects and a review of experience in pediatric renal disease

Hunt

Jain

Somers

2013

J of Clinical Apheresis

View full text Add to dashboard Cite

Although there is less experience with its use in children than adults, apheresis can be a life-saving treatment modality in certain pediatric diseases. With attention to specific technical aspects of the treatment, especially circuit volume, apheresis can be safely performed in children of any age or size. Even in pediatric diseases where it is recognized as an important part of therapy, apheresis is unfortunately still underutilized in North America and there needs to be increased awareness of its role and its availability within the pediatric community. Apheresis has been used particularly in children with certain renal diseases, notably ANCA-associated nephritis, anti-GBM disease, and atypical HUS. In addition, it can improve outcomes in transplantation of children with FSGS and can be part of a pre-transplant strategy for children who are highly sensitized and at high risk for graft failure.

show abstract

A combined low-density lipoprotein apheresis and prednisone therapy for steroid-resistant primary focal segmental glomerulosclerosis in children

Cited by 68 publications

References 36 publications

Low-Density Lipoprotein Apheresis Therapy for Steroid- and Cyclosporine-Resistant Idiopathic Membranous Nephropathy

Low-Density Lipoprotein Apheresis Therapy for Steroid- and Cyclosporine-Resistant Idiopathic Membranous Nephropathy

Recurrent focal segmental glomerulosclerosis after kidney transplantation

Apheresis therapy in children: An overview of key technical aspects and a review of experience in pediatric renal disease

Contact Info

Product

Resources

About