Re‐transplantation in pediatric patients with failure of primary transplant due to recurrent focal segmental glomerulosclerosis: A pediatric nephrology research consortium study

Every year, the transplant waiting list gets longer, while donor numbers essentially remain the same. This makes the responsibility of being good stewards of this precious and limited resource greater than ever. Transplant teams, who are both committed to their patients and aware of this important responsibility, are left to make the difficult and ethical decisions regarding retransplantation. Retransplantation of organs in pediatric patients presents a different set of issues to consider, and the results are promising. This case study presents a boy who received a kidney transplant for focal segmental glomerulosclerosis at age 5. At age 11, because of the recurrence of focal segmental glomerulosclerosis and severe cardiomyopathy, he required a rare combined kidney-heart transplant. At age 17, he developed chronic renal failure and posttransplant coronary artery disease, most likely related to a period of noncompliance, and required yet another combined kidney-heart transplant. He is currently alive and well 2 years after transplantation.

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The role of cationic channels of the potential TRPC receptor in the pathogenesis of idiopathic nephrotic syndrome in children

Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics) ◽

10.21508/1027-4065-2021-66-5-67-74 ◽

2021 ◽

Vol 66 (5) ◽

pp. 67-74

Author(s):

S. L. Morozov ◽

V. V. Dlin

Keyword(s):

Nephrotic Syndrome ◽

Focal Segmental Glomerulosclerosis ◽

Idiopathic Nephrotic Syndrome ◽

Pediatric Nephrology ◽

Urgent Problem ◽

Steady Decrease ◽

Segmental Glomerulosclerosis ◽

Cationic Channels ◽

Morphological Picture

Idiopathic nephrotic syndrome is the most common glomerulopathy in children, with a prevalence of approximately 16 per 100,000 of child population worldwide. Any chronic glomerular disease has the same type of development mechanism. Regardless of the damaging factor, after the death of a significant part of the nephrons, there occurs a steady decrease in the glomerular filtration rate, while morphologically we most often determine focal segmental glomerulosclerosis. Studying the causes of focal segmental glomerulosclerosis is an urgent problem in pediatric nephrology. Recently, there has been discussed the role of the cation channels of the potential receptor TRPC of podocytes in the development of proteinuria and focal segmental glomerulosclerosis. The article provides data on the role of TRPC receptors in the pathogenesis of focal segmental glomerulosclerosis. The authors present their our own data demonstrating gene expression of the cationic channels family of the potential receptor TRPC1, TRPC3, TRPC4, TRPC5 and TRPC6 in children with idiopathic nephrotic syndrome, depending on the morphological picture of the disease and sensitivity to steroid therapy.

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Genetic Study in Korean Pediatric Patients with Steroid-Resistant Nephrotic Syndrome or Focal Segmental Glomerulosclerosis

Journal of Clinical Medicine ◽

10.3390/jcm9062013 ◽

2020 ◽

Vol 9 (6) ◽

pp. 2013

Author(s):

Eujin Park ◽

Chung Lee ◽

Nayoung K. D. Kim ◽

Yo Han Ahn ◽

Young Seo Park ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Focal Segmental Glomerulosclerosis ◽

Detection Rate ◽

Pediatric Patients ◽

Mutation Detection ◽

Genetic Diagnosis ◽

Mutation Detection Rate ◽

Steroid Resistant ◽

Steroid Resistant Nephrotic Syndrome ◽

Segmental Glomerulosclerosis

Steroid-resistant nephrotic syndrome (SRNS) is one of the major causes of end-stage renal disease (ESRD) in childhood and is mostly associated with focal segmental glomerulosclerosis (FSGS). More than 50 monogenic causes of SRNS or FSGS have been identified. Recently, the mutation detection rate in pediatric patients with SRNS has been reported to be approximately 30%. In this study, genotype-phenotype correlations in a cohort of 291 Korean pediatric patients with SRNS/FSGS were analyzed. The overall mutation detection rate was 43.6% (127 of 291 patients). WT1 was the most common causative gene (23.6%), followed by COQ6 (9.4%), NPHS1 (8.7%), NUP107 (7.1%), and COQ8B (6.3%). Mutations in COQ6, NUP107, and COQ8B were more frequently detected, and mutations in NPHS2 were less commonly detected in this cohort than in study cohorts from Western countries. The mutation detection rate was higher in patients with congenital onset, those who presented with proteinuria or chronic kidney disease/ESRD, and those who did not receive steroid treatment. Genetic diagnosis in patients with SRNS provides not only definitive diagnosis but also valuable information for decisions on treatment policy and prediction of prognosis. Therefore, further genotype-phenotype correlation studies are required.

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Renal Artery Embolization for the Treatment of Refractory Proteinuria in Three Pediatric Patients with Focal Segmental Glomerulosclerosis

Journal of Vascular and Interventional Radiology ◽

10.1016/j.jvir.2017.08.011 ◽

2018 ◽

Vol 29 (1) ◽

pp. 136-138

Author(s):

Anjaneya S. Kathait ◽

Kyung Rae Kim

Keyword(s):

Renal Artery ◽

Focal Segmental Glomerulosclerosis ◽

Pediatric Patients ◽

Artery Embolization ◽

Segmental Glomerulosclerosis ◽

Renal Artery Embolization

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Treatment of Recurrent Focal Segmental Glomerulosclerosis in Pediatric Kidney Transplant Recipients: Effect of Rituximab

Journal of Transplantation ◽

10.1155/2011/389542 ◽

2011 ◽

Vol 2011 ◽

pp. 1-5 ◽

Cited By ~ 15

Author(s):

Christine Sethna ◽

Corinne Benchimol ◽

Hilary Hotchkiss ◽

Rachel Frank ◽

Lulette Infante ◽

...

Keyword(s):

Monoclonal Antibody ◽

Renal Transplantation ◽

Side Effects ◽

Focal Segmental Glomerulosclerosis ◽

Pediatric Patients ◽

Graft Loss ◽

Transplant Recipients ◽

Kidney Transplant Recipients ◽

Adverse Side Effects ◽

Segmental Glomerulosclerosis

Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation is a complication that often leads to graft loss. There is no consensus on the optimal treatment of recurrent FSGS. Rituximab, a monoclonal antibody to CD20, may be a useful treatment of this complication.Methods. We report four pediatric cases of recurrent FSGS treated with rituximab and plasmapheresis.Results. Four children (2M/2F), age 15.3 ± 2.6, with recurrent FSGS posttransplant were identified. Four doses of rituximab were administered 171 ± 180 days posttransplant and 114 ± 169 days after the start of plasmapheresis. Three children responded with complete remission, one of whom relapsed after four months. One child had a partial response with a decrease in proteinuria that was not sustained. No adverse side effects were reported during treatment or followup (mean 22.5 months).Conclusions. Rituximab is a safe and well-tolerated ancillary treatment for recurrent FSGS in pediatric patients in conjunction with plasmapheresis.

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