ESPE Abstracts

Introduction: The prevalence of renal anomalies in Turner syndrome (TS) has been reported to vary from 30 to 70%. However, the influence of renal anomalies on renal function and morbidity have been less well investigated. We evaluate the status of renal function and the presence of urinary abnormalities in early adult TS patients.

Patients and method: Sixty-three girls with TS, who are attending Pediatric Endocrine Clinics in Busan Paik Hospital, were studied. The mean age of the patients at last follow-up was 23.64±4.51 years and at diagnosis was 10.49±4.04 years. The mean duration of follow-up was 6.09±4.12 years. KUB sonography was performed in all TS patients and some of them also had IVP, renal DMSA scan, and renal CT. Renal function test with R-UA were examined in every visiting times.

Results: Of the 63 patients, the karyotype showed 45,X in 32 (50.8%) patients, mosaicism in 22 (34.9%) and structural aberration in 9 (14.3%). Renal anomalies were observed in 20 of the 63 TS (31.7%). Of the 32 TS patients with 45,X karyotype, 13 (40.6%) had renal anomalies, while these were found in 7 (22.6%) of 31 TS patients with mosaicism/structural aberration. But there is no significant statistical differences between two karyotype groups. The renal anomalies included ten cases of horseshoe kidney, one case of renal agenesis, eight cases of abnormal renal collecting system, and one case of malrotation. At last follow-up time, the mean serum level of BUN and Cr level were 9.72±2.60 and 0.64±0.11 respectively. Hematuria was observed in 7 (11.1%) TS patients. Among them three TS patients have renal anomalies. One TS patient suffer from nephrotic syndrome for 2.5 years.

Conclusion: The prevalence of renal anomalies in Korean TS patients was 31.7% and there is no significant differences between two different karyotypes. At last follow-up, all TS patients have normal renal function. Hematuria was observed 11% of TS patients. Although associated renal anomalies may not influence renal function in early adult, careful attention should be necessary in TS patients with hematuria to prevent progressing renal problems.