title: Optimizing outcomes for neonatal ARPKD.
abstract: A retrospective analysis was conducted on 10 consecutive cases of neonatal ARPKD, 9 of whom received kidney transplants (KT). All were diagnosed antenatally (n = 6) or at birth. In the first month of life 70% required ventilatory support. Pre-emptive bilateral nephrectomy and peritoneal dialysis (PD) catheter placement were performed in 9 at a mean age of 7.8 +/- 11.9 months. The indications for nephrectomy were massive kidneys, resulting in suboptimal nutrition and respiratory compromise. All patients received assisted enteral nutrition, with significant increase in mean tolerated feeds following nephrectomy (p < 0.05), with increase in mean normalized weight and height (0.92 and 1.2 delta SDS respectively), by one year post-transplantation. KT was performed at a mean age and weight of 2.5 +/- 1.4 years and 13.3 +/- 6.1 kg. The mean creatinine clearance at one year post-KT was 91.3 +/- 38.1 mls/min/1.73 m(2), with a projected graft life expectancy of 18.4 years. Patient survival was 89% and death censored graft survival was 100%, at a mean follow-up of 6.1 +/- 4.5 years post-transplant. Six patients demonstrated evidence of hepatic fibrosis, one of which required liver transplantation. In patients with massive kidneys from ARPKD, pre-emptive bilateral nephrectomy, supportive PD and early aggressive nutrition, can minimize early infant mortality, so that subsequent KT can be performed with excellent patient and graft survival.
authors: Department of Transplant Surgery, Stanford University, Stanford, CA 94304, USA.
authors: Mona
authors: M
authors: Beaunoyer
authors: Mohile
authors: M
authors: Snehal
authors: Li
authors: L
authors: Li
authors: Waldo
authors: W
authors: Concepcion
authors: Oscar
authors: O
authors: Salvatierra
authors: Minnie
authors: M
authors: Sarwal
mesh_terms: D002675:Child, Preschool; D004750:Enteral Nutrition; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D016030:Kidney Transplantation; D008297:Male; D009392:Nephrectomy; D010530:Peritoneal Dialysis; D017044:Polycystic Kidney, Autosomal Recessive / Q000151:congenital* / Q000628:therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome