J Korean Soc Pediatr Nephrol.
2010 Apr;14(1):42-50.
Clinical Characteristics and Associated Anomalies in Children with Solitary Kidney
- Affiliations
-
- 1Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea. cheonghi@snu.ac.kr
- 2Research Center for Rare Diseases, Seoul National University Hospital, Seoul, Korea.
- 3Kidney Research Institute, Medical Research Center, Seoul National University College of Medicine, Seoul, Korea.
Abstract
- PURPOSE
The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively.
METHODS
Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded.
RESULTS
Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL.
CONCLUSION
SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.