A critical re-analysis of cases of post-transplantation recurrence in genetic nephrotic syndrome.

ABSTRACT:

Background

Genetic defects in podocyte proteins account for up to 30% of steroid-resistant nephrotic syndrome (SRNS) in the paediatric population. Most children with genetic SRNS are resistant to immunosuppression and at high risk of progression to stage 5 chronic kidney disease. Kidney transplantation is often the treatment of choice. The possibility of post-transplantation disease recurrence in genetic SRNS remains controversial, and poses fundamental questions about disease biology.

Methods

We critically evaluated the published cases of post-transplantation recurrence in genetic patients, particularly testing 'mutations' against the most recent population variant databases, in order to clarify the diagnoses, and compare the clinical courses and responses to therapy.

Re

SUBMITTER: Mason AE 

PROVIDER: S-EPMC8497325 | biostudies-literature | 2021 Nov

REPOSITORIES: biostudies-literature