Risk factors for chronic kidney disease progression in VACTERL-association Korean patients: a retrospective study

Renal anomalies account for 50% to 80% of vertebral, anal, cardiac, tracheoesophageal fistula with or without atresia, renal and limb (VACTERL) associations. Some of these patients develop chronic kidney disease (CKD). This study evaluated the clinical characteristics, prevalence, and risk factors of CKD in VACTERL-association patients.

The medical records of pediatric VACTERL-association patients who visited Samsung Medical Center from January 2011 to December 2019 were reviewed retrospectively using a traditional Schwarz formula to estimated glomerular filtration rate by serum creatinine. Cases of CKD were classified according to Kidney Disease Improving Global Outcomes guidelines as stages 2 to 5.

A total of 47 VACTERL-association patients were enrolled. Renal anomalies were presented in 89.4% of patients. The mean follow-up period was 3.26 years. Half of the patients had a urinary tract infection, and 83.3% of these experienced more than one. Vesicoureteral reflux was detected in 23 patients. Twenty patients experienced acute kidney injury (AKI). Among them, 20% progressed to CKD. Two AKI patients required kidney replacement therapy. In this study, the prevalence of CKD was 14.9%. Complex renal anomalies and stage 3 AKI increased the risk of CKD progression in patients with VACTERL association by 11-fold and 9-fold, respectively (P=0.020 and P=0.041).

Several factors such as complex renal anomalies and stage 3 AKI may influence kidney function in VACTERL association. A further longitudinal study of CKD in VACTERL-association patients is required.