ADVANCED LIVING KIDNEY DONATION: A PROPOSED STRATEGY TO INCREASE OPPORTUNITY FOR PREEMPTIVE KIDNEY TRANSPLANTATION IN STAR SYNDROME

Syndactyly-telecanthus-anogenital and renal (STAR) malformation syndrome is a rare genetic disorder that eventually progresses to end-stage kidney disease (ESKD). Kidney transplantation is the treatment of choice for suitable patients with advanced chronic kidney disease (CKD) or ESKD. For patients who likely need future transplantation, early planning to pursue living kidney donation can avoid dialysis, reduce wait times, and improve transplant outcomes and quality of life (QoL). Preemptive living donor kidney transplantation (LDKT) offers better graft survival, lower mortality, and improved QoL compared to deceased donor kidney transplantation (DDKT). Advanced living kidney donation as a part of National Kidney Registry (NKR) Kidney Paired Donation (KPD) program also improves matching and potentially offers better quality of the donated kidneys compared to traditional intended living kidney donation. We report a case of STAR syndrome evaluated for preemptive LDKT, thereby avoiding dialysis and improving outcomes.

The patient is a 26-year-old obese White woman with nephrotic-range proteinuria stage 4 CKD secondary to a congenital atrophic left kidney and multiple congenital anomalies consistent with STAR syndrome (Syndactyly, Telecanthus, Anogenital malformations and Renal anomalies). She presented for a routine general nephrology follow-up after progressive kidney function decline over the past one year, with rising creatinine from 1.4 to 2.7 mg/dL and urinary total protein:urinary creatinine ratio of3,720 mg/g of creatinine and urinary microalbumin:urinary creatinine ration of 2,865 mg/g of creatinine. Her body mass index was 40.6 kg/m2 despite weight loss efforts. Given CKD progression, her underlying behavioral changes that might be contraindicated to hemodialysis, and obesity, limiting peritoneal dialysis, preemptive living donor kidney transplantation was discussed with the patient and her mother, focusing on advanced living kidney donation through KPD through the NKR for better matching and potentially receiving better quality from a younger donor compared to directed donation from her mother. The plan is to list the patient for transplantation once her GFR declines to 20 mL/min/1.73 m², allowing time for weight loss and optimization for candidacy.

While the advanced living kidney donation can provide potentially better quality kidneys for this patient compared to receiving a directly donated kidney from her mother, this donor-recipient pair had at least  one haplotype match. However, one of the advantages of the advanced donation in this case was that the patient will likely receive preemptive LDKT, while avoiding the patient’s mother to loss of candidacy for futher living kidney donation given advanced age (Figure 1).

This case demonstrates the value of early transplant planning in patients with congenital kidney disease such as STAR syndrome, where CKD progresses to ESRD. Preemptive LDKT evaluation should be planned to avoid dialysis-related morbidity and improve outcomes. Advanced donation through KPD program by the NKR was considered, balancing family donor options with better compatibility. Proactive listing and optimization before GFR falls at or below 20 mL/min/1.73 m² or even at advanced stage 3 CKD generally provide superior graft survival and quality of life compared with transplantation after initiation of dialysis.