Journal article
Kidney transplantation in patients with tuberous sclerosis complex
Pediatric transplantation, Vol.28(4), e14765
06/2024
DOI: 10.1111/petr.14765
PMCID: PMC11125526
PMID: 38778713
Appears in UI Libraries Support Open Access
Abstract
Tuberous sclerosis complex (TSC) is a disorder of the mammalian target of the rapamycin (mTOR) pathway associated with the development of multisystem tumors, including renal angiomyolipoma (AML). These renal tumors are benign by nature but locally invasive and carry a risk for the progression of chronic kidney disease (CKD) to end stage kidney disease (ESKD). The frequency of subsequent renal transplantation in this population is largely uncharacterized, although single-center data suggests that 5%-15% of adult TSC patients are kidney transplant recipients.
This retrospective cohort study utilized United Network for Organ Sharing (UNOS) data. We included candidates waitlisted between 1987 and 2020 for a first kidney transplant with TSC-associated kidney failure. We utilized descriptive statistics to characterize the frequency of first-time kidney transplant waitlisting and transplantation among persons with TSC and the Fine-Gray subdistribution hazard model to evaluate characteristics associated with progression from waitlist.
We identified 200 TSC-associated kidney failure patients within the waitlist cohort. Of these, 12 were pediatric patients. Two-thirds (N = 134) of waitlisted persons were female. One hundred forty patients received a transplant with a median waitlist time of 2 years. Younger age at waitlisting was associated with a greater probability of progressing to transplant (HR 0.98 [95% CI: 0.96-0.99]). 91.8% of kidney transplant recipients survived 1-year post-transplant with a functioning allograft.
The majority of patients with TSC who are waitlisted for a kidney transplant progress onto transplantation with excellent 1-year post transplant patient and allograft survival.
Details
- Title: Subtitle
- Kidney transplantation in patients with tuberous sclerosis complex
- Creators
- Amber M Goedken - University of IowaWesam W Ismail - Department of Pharmacy Practice and Science, University of Iowa College of Pharmacy, Iowa City, Iowa, USALucas D G Barrett - University of IowaLyndsay A Harshman - University of Iowa
- Resource Type
- Journal article
- Publication Details
- Pediatric transplantation, Vol.28(4), e14765
- Publisher
- Wiley
- DOI
- 10.1111/petr.14765
- PMID
- 38778713
- PMCID
- PMC11125526
- eISSN
- 1399-3046
- Grant note
- R01DK128835 / NIDDK NIH HHS R18HS029292 / Agency for Healthcare Research and Quality T32 GM139776 / NIH HHS HHSH250-2019-00001C / HRSA HHS
- Language
- English
- Date published
- 06/2024
- Academic Unit
- Pharmacy; Nephrology, Dialysis and Transplantation; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Radiation Oncology; Pharmacy Practice and Science
- Record Identifier
- 9984628054702771
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