Living related kidney donation for alport syndrome spectrum: long-term outcomes

Abstract

Purpose: Data to guide the evaluation of living related donor (LRD) candidates for kidney transplant recipients with Alport syndrome (AS) spectrum are limited. We examined a cohort of LRD to recipients with AS to improve understanding of the clinical course and outcomes of living donation in this context. Methods: The cohort of recipients and their LRD was retrospectively identified from data at 5 transplant centers (1987-2021). LRD were followed for postdonation kidney function measured by estimated glomerular filtration rate (eGFR), proteinuria, major cardiac events (MACE) and death. Results: The cohort comprised 33 LD, where relationship to recipient included mother (14, 42%), father (10, 30%), sibling (5, 32%), grandparent (1, 3%), uncle (1, 3%) and unrelated (2, 6%). Long term outcomes were evaluated in 27 LRD during a follow up of 12 years (IQR, 5-16). None of the donors developed kidney failure (eGFR<15 ml/min/1.73m2 or dialysis) during follow up. Last follow up serum mean (SD) creatinine, eGFR and proteinuria levels were 1.1 (0.2) mg/dL, 68.3 (16.0) ml/min/1.73m2, and 0.19 (0.36) g/g, respectively (Table 1). During follow up, 13 (48%) and 6 (22%) donors developed hypertension and diabetes mellitus, respectively. Five donors (19%) developed MACE [acute coronary ischemia, n=4 (15%), severe congestive heart failure, n=1, (4%)] at a median 5.5 (IQR, 4.5-10.3) years after donation. MACE rate was significantly higher in patients who developed hypertension compared to normotensives after donation (0% vs 35.7%, p=0.017) (Fig. 1). Three donors died during follow-up at median 14 (5-15) years after donation.