Treatment of FSGS in Children

Sethna, Christine B.; Gipson, Debbie S.

doi:10.1053/j.ackd.2014.01.010

Cited by 28 publications

(26 citation statements)

References 45 publications

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“…Critical is the elimination of other systemic diseases or primary renal diseases that may result in a similar presentation. Many reviews cover various aspects of FSGS and include comprehensive reviews (1)(2)(3)(4), disease mechanisms (5)(6)(7)(8), pediatric disease (9), immunologic aspects (10), treatment in children (11), and treatment in adults (12)(13)(14). We focus here on a practical approach to FSGS assessment on clinical and histopathologic grounds in the context of our current understanding of disease mechanisms and genetics.…”

Section: Introductionmentioning

confidence: 99%

Focal Segmental Glomerulosclerosis

Rosenberg

Kopp

2017

CJASN

378

308

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Focal segmental glomerulosclerosis (FSGS) is a leading cause of kidney disease worldwide. The presumed etiology of primary FSGS is a plasma factor with responsiveness to immunosuppressive therapy and a risk of recurrence after kidney transplant-important disease characteristics. In contrast, adaptive FSGS is associated with excessive nephron workload due to increased body size, reduced nephron capacity, or single glomerular hyperfiltration associated with certain diseases. Additional etiologies arenow recognized asdrivers of FSGS: high-penetrance geneticFSGSdue to mutations in one of nearly 40 genes, virus-associated FSGS, and medication-associated FSGS. Emerging data support the identification of a sixth category: APOL1 risk allele-associated FSGS in individuals with sub-Saharan ancestry. The classification of a particular patient with FSGS relies on integration of findings from clinical history, laboratory testing, kidney biopsy, and in some patients, genetic testing. The kidney biopsy can be helpful, with clues provided by features on light microscopy (e.g., glomerular size, histologic variant of FSGS, microcystic tubular changes, and tubular hypertrophy), immunofluorescence (e.g., to rule out other primary glomerulopathies), and electron microscopy (e.g., extent of podocyte foot process effacement, podocyte microvillous transformation, and tubuloreticular inclusions). A complete assessment of renal histology is important for establishing the parenchymal setting of segmental glomerulosclerosis, distinguishing FSGS associated with one of many other glomerular diseases from the clinical-pathologic syndrome of FSGS. Genetic testing is beneficial in particular clinical settings. Identifying the etiology of FSGS guides selection of therapy and provides prognostic insight. Much progress has been made in our understanding of FSGS, but important outstanding issues remain, including the identity of the plasma factor believed to be responsible for primary FSGS, the value of routine implementation of genetic testing, and the identification of more effective and less toxic therapeutic interventions for FSGS.

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Section: Introductionmentioning

confidence: 99%

Focal Segmental Glomerulosclerosis

Rosenberg

Kopp

2017

CJASN

378

308

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“…Secondary FSGS may be due to genetic mutations, obesity, infections (ie, human immunodeficiency virus), medications (ie, interferon), or systemic illness (ie, sickle cell disease) . FSGS can occur as a result of monogenetic mutations and may be associated with familial forms . According to the NAPRTCS database, 60% of children with FSGS will progress to dialysis or require kidney transplantation .…”

Section: Introductionmentioning

confidence: 99%

“…According to the NAPRTCS database, 60% of children with FSGS will progress to dialysis or require kidney transplantation . Kidney survival is associated with the degree of proteinuria . Patients who achieve complete remission have a five‐year renal survival rate of 90% .…”

Section: Introductionmentioning

confidence: 99%

“…Treatment of FSGS is complex and includes both non‐immunosuppressive and immunosuppressive medications . In patients with genetic mutations, there is controversial data about the effectiveness of immunosuppressive medications . Up to 50% of patients with FSGS will have recurrence in the transplanted kidney .…”

Section: Introductionmentioning

confidence: 99%

“…2,4 FSGS can occur as a result of monogenetic mutations and may be associated with familial forms. 5 According to the NAPRTCS database, 60% of children with FSGS will progress to dialysis or require kidney transplantation. 3 Kidney survival is associated with the degree of proteinuria.…”

Section: Introductionmentioning

confidence: 99%

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Ofatumumab in post‐transplantation recurrence of focal segmental glomerulosclerosis in a child

Solomon

Zolotnitskaya

Río

2019

Pediatric Transplantation

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FSGS is a potentially devastating form of nephrotic syndrome. Treatment of SRNS can be difficult, especially post‐transplantation. The current therapy of post‐transplant SRNS includes plasmapheresis, ACE‐I, CNI, and monoclonal antibodies (rituximab). Patients who are refractory to these interventions have limited therapeutic alternatives. We present a case of a patient with SRNS secondary to FSGS. He did not respond to immunosuppressive medications prior to transplant, progressed to ESRD, and was started on chronic hemodialysis. He received a DDKT which was complicated by post‐transplant FSGS recurrence. A course of plasmapheresis, rituximab, and CNI were administered with some response. Ofatumumab was then given to the patient. As a result, the patient achieved partial remission. Ofatumumab may be a safe and effective option for post‐transplant recurrence of FSGS.

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Therapeutic Apheresis in Children

Taylan

Sutherland

2021

Pediatric Dialysis

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Treatment of FSGS in Children

Cited by 28 publications

References 45 publications

Focal Segmental Glomerulosclerosis

Focal Segmental Glomerulosclerosis

Ofatumumab in post‐transplantation recurrence of focal segmental glomerulosclerosis in a child

Therapeutic Apheresis in Children

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