Patient and Graft Survival Following Kidney Transplantation in Recipients With Cystinosis: A Cohort Study

Spicer, Rebecca A.; Clayton, Philip; McTaggart, Steven; Zhang, Geoff Y.; Alexander, Stephen I.

doi:10.1053/j.ajkd.2014.07.020

Cited by 13 publications

(9 citation statements)

References 10 publications

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“…We found a progressive improvement in patient survival for patients with cystinosis. Australian and European cystinosis studies also found progressive improvements; however, their findings were not statistically significant . We found no difference in the causes of death or graft loss between eras.…”

Section: Discussioncontrasting

confidence: 63%

“…Australian and European cystinosis studies also found progressive improvements; however, their findings were not statistically significant. 3,14,18 We found no difference in the causes of death or graft loss between eras. Notably, no patient death was attributed to kidney failure.…”

Section: Discussionmentioning

confidence: 51%

“…Cohen et al retrospectively studied 36 cystinosis patients from five French university centers and found 5‐year graft survival to be 92% for patients transplanted between 1980 and 2013. A retrospective study of 36 cystinosis patients from ANZDATA registry found 10‐year graft survival to be 80.0% for cystinosis patients transplanted between 1995 and 2008 . Van Stralen et al examined kidney transplant outcomes in 185 cystinosis patients using European registry data and found 5‐year graft survival of 86.1% for transplants performed before 1990, 96.9% for transplants performed between 1990 and 1999, and 100% for transplants performed thereafter (survival difference was not significant).…”

Section: Discussionmentioning

confidence: 99%

“…Cystinosis has three distinct manifestations based on the severity of the mutations in the CTNS gene that encodes the transporter: infantile, juvenile, and non‐nephropathic ocular cystinosis. Infantile cystinosis, which is the most prevalent form of cystinosis, manifests as the renal Fanconi syndrome and, if left untreated, causes progressive tubulointerstitial and glomerular disease, which results in ESRD within the first decade of life …”

Section: Introductionmentioning

confidence: 99%

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Trends in kidney transplant outcomes in children and young adults with cystinosis

Kizilbash

Snyder

Vock

et al. 2019

Pediatric Transplantation

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Temporal changes in kidney transplant outcomes for cystinosis are unknown. We used the SRTR to identify all kidney transplants performed for cystinosis in patients younger than 31 years between 1987 and 2017. We divided time into three equal eras (1987‐1997, 1998‐2007, and 2008‐2017) to assess changes in outcomes using Cox proportional and linear regression models. We examined 441 transplants in 362 patients. Age at ESRD progressively increased (12.1 vs 13.3 vs 13.4; P = .046). Eras 2 and 3 had lower risk of acute rejection (aHR 2 vs 1:0.45; P < .001) (aHR 3 vs 1:0.26; P < .001) and higher 5‐year mean GFR (difference 2 vs 1:9.2 mL/min/1.73 m2; P = .005) (difference 3 vs 1:12.9 mL/min/1.73 m2; P = .002) compared with era 1. Five‐year graft survival was similar across eras, but 5‐year patient survival was higher for era 2 (aHR: 0.25; P = .01). Seventy‐nine patients underwent retransplantation. Five‐year patient (94.2% vs 92.5%; P = .57) and graft survival (79.1% vs 74.1%; P = .52) were similar between primary and subsequent transplants. Age at ESRD, acute rejection, GFR at 5 years, and patient survival improved over time. Kidney retransplantation is associated with excellent outcomes in children and young adults with cystinosis.

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Section: Discussioncontrasting

confidence: 63%

Section: Discussionmentioning

confidence: 51%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Trends in kidney transplant outcomes in children and young adults with cystinosis

Kizilbash

Snyder

Vock

et al. 2019

Pediatric Transplantation

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“…La transplantation rénale est indiquée lors du passage en insuffisance rénale terminale. L'indication de la néphrectomie des reins natifs dépend du degré de la polyurie [ 13 , 16 ]. Des cristaux de cystine peuvent être observés sur le rein greffé [ 9 , 10 ].…”

Section: Discussionunclassified

Particularité de la cystinose infantile chez l’enfant tunisien

Jellouli¹,

Turkia²,

Abidi³

et al. 2015

Pan Afr Med J

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La cystinose est une maladie rare qui résulte d'un défaut d'expression de la cystinosine transporteur de la cystine du lysosome. La forme infantile est la plus fréquente et la plus sévère. Elle conduit en dehors du traitement à l'insuffisance rénale chronique terminale au cours de la première décade de la vie. Nous rapportons l'expérience tunisienne de la cystinose infantile. Une étude rétrospective sur une période de 25 ans (1990-2014) était menée. Nous avons colligé 8 dossiers de cystinose infantile dans les services de pédiatrie des hôpitaux Charles Nicolle de Tunis et la Rabta de Tunis. Il s'agissait de 5garçons et de 3 filles. L’âge moyen au début des symptômes était de 6,37 mois (2-14 mois). L’âge moyen au moment du diagnostic était de 4 ans (7 mois-6 ans). Les dépôts cornéens de cystine étaient observés chez 7 patients. Sept patients présentaient une hypothyroïdie. La cystéamine était prescrite chez 6 patients. L’âge moyen au moment de la prescription de cystéamine était de 5,12 ans (8 mois- 13 ans). L’âge moyen lors de passage en insuffisance rénale chronique était de 3,4 ans. L’âge moyen lors du passage en insuffisance rénale chronique terminale était de 6,37 ans Actuellement, un patient garde une fonction rénale normale, trois patients sont en insuffisance rénale, deux patients sont décédés et un patient était transplanté. Il faut instaurer dans notre pays les moyens de diagnostic pour traiter tôt la maladie.

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Cystinosis

Levtchenko

Monnens²

2023

Pediatric Kidney Disease

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Patient and Graft Survival Following Kidney Transplantation in Recipients With Cystinosis: A Cohort Study

Cited by 13 publications

References 10 publications

Trends in kidney transplant outcomes in children and young adults with cystinosis

Trends in kidney transplant outcomes in children and young adults with cystinosis

Particularité de la cystinose infantile chez l’enfant tunisien

Cystinosis

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