Abstract: TH-PO1040
Durable Third Kidney Allograft Function in Jeune Syndrome with Calculated Panel Reactive Antibody (cPRA) 99%-100% and Donor-Specific Antibody (DSA) Positivity
Session Information
- Transplantation: Clinical - Outcomes, Malignancy, and Pathology
October 22, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 2002 Transplantation: Clinical
Authors
- Tijerina, Diego, South Texas Health System, Edinburg, Texas, United States
- EL-Najjar, Yassin, South Texas Health System, Edinburg, Texas, United States
- Touma, Mary-Joe, South Texas Health System, Edinburg, Texas, United States
- Alsabbagh, Mourad, South Texas Health System, Edinburg, Texas, United States
Introduction
Jeune syndrome, or asphyxiating thoracic dystrophy, is a rare skeletal ciliopathy (~1:100,000–130,000 live births) in which survivors may develop tubulointerstitial renal disease progressing to ESKD. Kidney transplantation in Jeune syndrome is sparsely reported, and outcomes after highly sensitized third transplantation are not well characterized.
Case Description
A 63-year-old woman with Jeune syndrome and childhood-onset renal failure attributed to Jeune-associated interstitial fibrosis underwent bilateral native nephrectomies and dialysis in childhood. A 1978 living-related transplant from her father functioned ~22 years before failing from chronic rejection/chronic allograft nephropathy (CAN). A 2003 living-unrelated transplant was complicated by transplant-ureter reflux with recurrent pyelonephritis, ureteroureterostomy, biopsy-documented CAN, suspected CNI toxicity, hypertensive emergency, zoster treated with IV valacyclovir complicated by AKI/metabolic acidosis, severe pancreatitis, and return to PD in 2013.
Pre-third-transplant: She was blood type O with cPRA 99–100%, >20 prior transfusions, anti-HLA-DQA1*05:01 with later broadened reactivity, and DSA positivity. In 2015, she received a deceased-donor kidney transplant with TPE ×4, thymoglobulin, steroids, tacrolimus, and rituximab. Creatinine fell from 17.92 to 0.80 mg/dL by discharge. Early cytopenias and transient hypogammaglobulinemia delayed mycophenolate. At ~10 years, creatinine remained 0.56–0.61 mg/dL, eGFR ~100 mL/min/1.73 m2, UPCR 82 mg/g, on tacrolimus, mycophenolate, and low-dose prednisone.
Discussion
This is among the first reports of durable third kidney allograft function in Jeune syndrome. Despite cPRA 99–100%, DSA positivity, and two prior allograft losses, desensitization/induction with TPE, thymoglobulin, and rituximab yielded rapid recovery and sustained function. Second-graft loss was multifactorial, underscoring the value of integrated longitudinal review in complex retransplant candidates. Key teaching points: Jeune-associated renal disease can require repeat transplantation; durable function is achievable with individualized high-risk protocols; and adult syndromic recipients need multidisciplinary urologic, hepatobiliary, pancreatic, and skeletal care.