Low-density lipoprotein apheresis in a pediatric patient with refractory nephrotic syndrome due to focal segmental glomerulosclerosis

Oto, Jun; Suga, Kenichi; Matsuura, Sato; Kondo, Shuji; Ohnishi, Yoshiaki; Inui, Daisuke; Imanaka, Hideaki; Kubo, Shoji; Nishimura, Masaji

doi:10.1007/s00540-008-0726-z

Cited by 11 publications

(6 citation statements)

References 10 publications

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“…FSGS with steroid resistance is often treated by immunosuppressive drugs such as CsA, mizoribine, and cyclophosphamide. LDL-A is also effective therapeutic tool for FSGS [19]. Regarding treatment with our patient, the patient was diagnosed as FSGS by renal biopsy.…”

Section: Discussionmentioning

confidence: 93%

Successful treatment by mycophenolate mofetil in a patient with focal segmental glomerulosclerosis associated with posterior reversible encephalopathy syndrome

et al. 2014

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It has been reported that cyclosporine A (CsA) treatment may be associated with posterior reversible encephalopathy syndrome. We report a 16-year-old man who exhibited nephrotic syndrome and posterior reversible encephalopathy syndrome. Intensive antihypertensive therapy restored him to consciousness. Renal biopsy revealed that he suffered from focal segmental glomerulosclerosis. Although he was treated with prednisolone and low-density lipoprotein apheresis therapy, his proteinuria remained at high level. Then, mycophenolate mofetil (MMF) with less influence on vessel endothelium compared with CsA and tacrolimus was administered. Soon after, he reached remission of nephrotic syndrome without recurrence of posterior reversible encephalopathy syndrome. This is the first case that a young patient of focal segmental glomerulosclerosis with posterior reversible encephalopathy syndrome achieved a complete remission by MMF treatment without recurrence of posterior reversible encephalopathy syndrome. MMF may be effective for young patients of focal segmental glomerulosclerosis especially with clinical condition of vascular endothelial damage such as posterior reversible encephalopathy syndrome.

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Section: Discussionmentioning

confidence: 93%

Successful treatment by mycophenolate mofetil in a patient with focal segmental glomerulosclerosis associated with posterior reversible encephalopathy syndrome

et al. 2014

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“…Clinical parameters immediately after lipoprotein apheresis therapy that contributed significantly for favorable outcome were serum total protein (4.9 ± 0.7 g/dl), serum albumin (2.9 ± 0.8 g/dl), serum creatinine (1.2 ± 0.7 mg/dl), eGFR (61 ± 27.2 ml/min/m 2 ), urine protein (1.7 ± 1.8 g/day), triglycerides (240.2 ± 156.3 mg/dl), total cholesterol (194.3 ± 65.6 mg/dl), LDL (83.1 ± 60.4 mg/dl), HDL (66.5 ± 18.3 mg/dl), fibrinogen (271.1 ± 77.2 mg/dl), and thrombin-antithrombin III complex (14.7 ± 38.6 ng/ml) (26). In addition, published literature has revealed few case studies showing remission of NS following dextran-sulfate plasma adsorption lipoprotein apheresis along with steroid and immunosuppressive therapies in children with drug resistant FSGS (14, 18, 19).…”

Section: Discussionmentioning

confidence: 99%

Dextran-Sulfate Plasma Adsorption Lipoprotein Apheresis in Drug Resistant Primary Focal Segmental Glomerulosclerosis Patients: Results From a Prospective, Multicenter, Single-Arm Intervention Study

et al. 2019

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Background: Focal segmental glomerulosclerosis (FSGS) causes end stage renal disease (ESRD) in significant proportion of patients worldwide. Primary FSGS carries poor prognosis and management of FSGS patients, refractory to standard treatments or resistant to steroids, remains a major challenge. Lipoprotein apheresis is a therapeutic approach for drug resistant primary FSGS and post-renal transplant primary FSGS recurrence.Objectives: To examine the safety and probable benefit at 1, 3, 6, 12, and 24-months following completion of apheresis treatment using Liposorber® LA-15 system in patients with nephrotic syndrome (NS), due to refractory primary FSGS or primary FSGS associated NS, in post renal transplant children.Material and Methods: Prospective, multicenter, single-arm intervention study using Liposorber® LA-15 system. Patients ≤21 years old with drug resistant or drug intolerant NS secondary to primary FSGS with glomerular filtration rate (GFR) ≥60 ml/min/1.73 m2 or post renal transplant patients ≤21 years old with primary FSGS associated NS were included in the study. Each patient had 12 dextran-sulfate plasma adsorption lipoprotein apheresis sessions over a period of 9 weeks. All patients were followed up at 1, 3, 6, 12, and 24-months following completion of treatment.Results: Of 17 patients enrolled, six were excluded from the outcome analysis (protocol deviations). Of the remaining 11 patients, all but one have completed apheresis treatments. Three patients were lost to follow-up immediately after completion of apheresis and excluded from outcome analysis. At one-month follow-up, 1 of 7 patients (14.3%) attained partial remission of NS while 2 of 4 subjects (50%) and 2 of 3 subjects (66.7%) had partial/complete remission at 3- and 6-months follow-up, respectively. One of two patients followed up for 12 months had complete remission and one patient had partial remission of NS after 24 months. Improved or stable eGFR was noted in all patients over the follow-up period.Conclusion: The results of our multicenter study showed improvement in the response rates to steroid or immunosuppressive therapy and induced complete or partial remission of proteinuria in some of the patients with drug resistant primary FSGS. The main limitation of our study is the small number of subjects and high dropout rate.

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“…Out of the other 4 patients, 1 patient had partial remission, while the other 3 became resistant to treatment [43]. Several additional studies have illustrated similar beneficial effects (Table 4) [1,35,[40][41][42][43][44][45].…”

Section: Evidence Of Efficacy Ldl Apheresis For Fsgs In the Pediatricmentioning

confidence: 97%

Extracorporeal Therapies in the Treatment of Focal Segmental Glomerulosclerosis

et al. 2020

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Focal segmental glomerulosclerosis (FSGS) is one of the most frequent and severe glomerular kidney disease with frequent progression to end-stage renal disease and a high rate of recurrence in renal transplantations. Due to intolerance or resistance to the current immunomodulatory treatments, the management of FSGS is a therapeutic challenge. Over the last few years, development in extracorporeal therapies has shown potential beneficial outcomes in drug-resistant and recurrent FSGS patients. Thus, this study reviews the current literature on the use of extracorporeal therapies, such as plasma exchange therapy, immunoadsorption, and low-density lipoprotein apheresis, for the treatment of FSGS in the pediatric population.

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Low-density lipoprotein apheresis in a pediatric patient with refractory nephrotic syndrome due to focal segmental glomerulosclerosis

Cited by 11 publications

References 10 publications

Successful treatment by mycophenolate mofetil in a patient with focal segmental glomerulosclerosis associated with posterior reversible encephalopathy syndrome

Successful treatment by mycophenolate mofetil in a patient with focal segmental glomerulosclerosis associated with posterior reversible encephalopathy syndrome

Dextran-Sulfate Plasma Adsorption Lipoprotein Apheresis in Drug Resistant Primary Focal Segmental Glomerulosclerosis Patients: Results From a Prospective, Multicenter, Single-Arm Intervention Study

Extracorporeal Therapies in the Treatment of Focal Segmental Glomerulosclerosis

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