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Kidney Week

Abstract: PO2338

Race and Age at Onset of ESRD in Patients with Alport Syndrome

Session Information

Category: Pediatric Nephrology

  • 1700 Pediatric Nephrology

Authors

  • Kizilbash, Sarah J., University of Minnesota Academic Health Center, Minneapolis, Minnesota, United States
  • Chavers, Blanche M., University of Minnesota Academic Health Center, Minneapolis, Minnesota, United States
  • Kashtan, Clifford E., University of Minnesota Academic Health Center, Minneapolis, Minnesota, United States
Background

Angiotensin-converting enzyme (ACE) inhibitors have been shown to delay the onset of end-stage renal disease (ESRD) in both animal models and humans with Alport syndrome. In 2013, expert guidelines recommended the use of ACE inhibitors to delay the onset of ESRD. We examined temporal changes in the age at onset of end-stage renal disease (ESRD) and interaction between race and age at ESRD for patients with Alport syndrome.

Methods

We used the Scientific Registry of Transplant Recipients to identify all patients who received a kidney transplant in the United States for Alport syndrome between 1987 and 2017. We divided the study period into three equal eras (era 1, 1987-1997; era 2, 1998-2007; and era 3, 2008-2017) to assess changes in age at ESRD using a linear regression model adjusting for race and gender. Age at ESRD was defined as the earlier of the age at dialysis or the age at kidney transplant.

Results

Between 1987 and 2017, 4105 Alport patients received a kidney transplant in the United States. Of these patients 3,115 (75.9%) were male and 3176 (77.4%) were white. Pre-emptive transplants were performed in 962 (23.4%) patients. Table 1 demonstrates temporal changes in median age at ESRD, demographic and clinical characteristics for patients with Alport syndrome. After adjusting for race and gender, the age at ESRD increased by a mean of 2.7 years (SE 0.51, p <0.01) for era 2 and 4.4 years (SE 0.51, p <0.01) for era 3 compared with era 1. We also observed that age at ESRD was significantly lower for black (-9.3 years, p < 0.01), Hispanic (-9.4 years, p < 0.01), and other races (-6.9 years, p < 0.01) compared with the white race (Table 2).

Conclusion

The age at ESRD for patients with Alport syndrome has progressively increased over the last 30 years. White patients have delayed onset of ESRD compared with patients of other races.

Temporal changes in ESRD and transplant characteristics
Variables1987-1997
N= 1461
1998-2007
N= 1299
2008-2017
N= 1345
p value
Age at ESRD (years)
Median (range)
28.2 (73.1)30.6 (73.2)30.6 (71.3)<0.001
Age at transplant (years)
Median (range)
31.2 (70.9)33.4 (75.1)33.6 (72.5)<0.001
Age at listing (years)
Median (range)
31.0 (71.0)32.0 (69.0)32.0 (69.0)0.05
Age at dialysis (years)
Median (range)
27.9 (73.1)29.5 (73.2)29.7 (70.4)0.007
Race n (%)
White
Black
Hispanic
Other

1218 (83.4)
111 (7.6)
103 (7.1)
29 (2.0)

1012 (77.9)
93 (7.2)
155 (11.9)
39 (3.0)

946 (70.3)
122 (9.1)
205 (15.2)
72 (5.3)
<0.001
Male n (%)1134 (77.6)964 (74.2)1017 (75.6)0.11