Abstract: FR-PO0065
CKD, Obesity-Related Complications, and Kidney Care Use in Pediatric Patients with Bardet-Biedl Syndrome: A Multicenter PHIS Study
Session Information
- ADPKD and Cystic Kidney Disease - 2
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Genetic Diseases of the Kidneys
- 1201 Genetic Diseases of the Kidneys: Cystic (Monogenic)
Authors
- Finer, Gal, Ann and Robert H Lurie Children's Hospital of Chicago Foundation, Chicago, Illinois, United States
- Ward, Sarah E., Ann and Robert H Lurie Children's Hospital of Chicago Foundation, Chicago, Illinois, United States
- Pomeroy, Jeremy, Marshfield Clinic Research Institute, Marshfield, Wisconsin, United States
- Davis, Erica, Ann and Robert H Lurie Children's Hospital of Chicago Foundation, Chicago, Illinois, United States
- Ramgopal, Sriram, Ann and Robert H Lurie Children's Hospital of Chicago Foundation, Chicago, Illinois, United States
Background
Bardet-Biedl syndrome (BBS) is a multisystem ciliopathy in which chronic kidney disease (CKD) is a major cause of morbidity and mortality. Contemporary multicenter data on kidney disease burden and kidney care utilization in U.S. children with BBS are limited.
Methods
We conducted a multicenter retrospective cohort study using the Pediatric Health Information System. Children <18 years with an ICD-10-CM code for BBS (Q87.83) between March 1, 2023, and June 30, 2025, were identified. Kidney diagnoses, comorbidities, and kidney-related testing were assessed. Children with BBS-associated CKD were frequency-matched by age and sex 1:4 to children with CKD due to structural kidney anomalies.
Results
Among 294 children with BBS, 25% had CKD and 6% had end-stage renal disease (ESRD). Median age at first documented CKD was 6 years (IQR 3-11), and median age at ESRD was 12 years (IQR 10-14). In the matched analysis, children with BBS-CKD (n=74) had higher rates of obesity-related complications than children with structural anomalies-CKD, including hypertension (51% vs. 39%; OR 2.09, 95% CI 1.17-3.75), obstructive sleep apnea (31% vs. 16%; OR 2.41, 95% CI 1.32-4.41), and diabetes (8% vs. 3%; OR 3.18, 95% CI 1.02-9.43). Kidney ultrasound was performed less frequently in BBS-CKD (81% vs. 92%; OR 0.23, 95% CI 0.09-0.56). In ESRD, BBS was associated with a higher transplantation rate than structural anomalies-related ESRD (HR 3.39, 95% CI 1.42-8.07).
Conclusion
In this multicenter pediatric cohort, CKD in BBS was common, began early, and occurred in the setting of increased obesity-related complications and incomplete kidney surveillance. These findings support earlier kidney monitoring, early nephrology involvement, and multidisciplinary care models for children with BBS.
Acknowledgment
We would like to extend a warm thank you to the Bardet-Biedl Syndrome Foundation for their continued support and collaboration.