Abstract
Aim: This study aimed to characterise the clinical features of Fabry disease (FD) patients who received kidney transplants in Australia and New Zealand.
Background: FD is a X-linked lysosomal storage disease caused by a deficiency in the enzyme, α-galactosidase A. FD progresses to chronic kidney disease, cardiomyopathy, deafness and cerebrovascular disease from 20-30 years of age.
Methods: All patients who received a renal transplant between 1963-2017 were extracted from the Australian and New Zealand Dialysis and Transplant (ANZDATA) registry. Univariate and multivariate Cox-proportional hazards models were used to evaluate the association between patient and transplant factors with mortality. Age, gender, smoking status, body mass index, ethnicity, diabetes, first kidney replacement therapy modality, dialysis commencement relative to enzyme replacement therapy availability, donor source and transplant era were assessed as covariates. Hazard ratios (HR) and 95% confidence intervals (CI) were calculated for each characteristic.
Results: 20 FD and 26,511 non-FD patients were included in the transplant cohort. 1 year patient survival, 3 year patient survival and 5 year patient survival were 90.0%, 85.0% and 80.0% respectively for FD patients and; 94.1%, 89.9% and 86.0% for non-FD patients. The main cause of death in both FD and non-FD patients was cardiac disease. Transplant patients with FD had increased mortality risk compared to transplant patients without FD (HR 3.63, 95% CI 1.63-8.10) in adjusted analyses. Increasing age, any cigarette smoking, underweight or obesity, diabetes and deceased donor were associated with increased mortality risk.
Conclusions: FD patients had higher mortality risk compared to non-FD patients. Further investigations are required to corroborate these findings in FD patients from other jurisdictions.
Background: FD is a X-linked lysosomal storage disease caused by a deficiency in the enzyme, α-galactosidase A. FD progresses to chronic kidney disease, cardiomyopathy, deafness and cerebrovascular disease from 20-30 years of age.
Methods: All patients who received a renal transplant between 1963-2017 were extracted from the Australian and New Zealand Dialysis and Transplant (ANZDATA) registry. Univariate and multivariate Cox-proportional hazards models were used to evaluate the association between patient and transplant factors with mortality. Age, gender, smoking status, body mass index, ethnicity, diabetes, first kidney replacement therapy modality, dialysis commencement relative to enzyme replacement therapy availability, donor source and transplant era were assessed as covariates. Hazard ratios (HR) and 95% confidence intervals (CI) were calculated for each characteristic.
Results: 20 FD and 26,511 non-FD patients were included in the transplant cohort. 1 year patient survival, 3 year patient survival and 5 year patient survival were 90.0%, 85.0% and 80.0% respectively for FD patients and; 94.1%, 89.9% and 86.0% for non-FD patients. The main cause of death in both FD and non-FD patients was cardiac disease. Transplant patients with FD had increased mortality risk compared to transplant patients without FD (HR 3.63, 95% CI 1.63-8.10) in adjusted analyses. Increasing age, any cigarette smoking, underweight or obesity, diabetes and deceased donor were associated with increased mortality risk.
Conclusions: FD patients had higher mortality risk compared to non-FD patients. Further investigations are required to corroborate these findings in FD patients from other jurisdictions.
| Original language | English |
|---|---|
| Pages | 64-64 |
| Number of pages | 1 |
| Publication status | Published - 16 Oct 2020 |
| Externally published | Yes |