Retrospective study of the renal function using estimated glomerular filtration rate and congenital anomalies of the kidney-urinary tract in pediatric Turner syndrome |
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| Authors: | Yukie Izumita Satsuki Nishigaki Mari Satoh Noriyuki Takubo Chikahiko Numakura Ikuko Takahashi Shun Soneda Yoshifusa Abe Hotaka Kamasaki Yoshiaki Ohtsu Junko Igaki Yukihiro Hasegawa Keisuke Nagasaki |
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| Institution: | 1. Department of Pediatrics, Niigata City General Hospital, Niigata, Japan;2. Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan;3. Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan;4. Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan;5. Department of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan;6. Department of Pediatrics, Akita University Graduate School of Medicine, Akita, Japan;7. Department of Pediatrics, St. Marianna University School of Medicine, Kanagawa, Japan;8. Children Medical Center, Showa University Koto Toyosu Hospital, Tokyo, Japan;9. Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Japan;10. Department of Pediatrics, Gunma University Graduate School of Medicine, Maebashi, Japan;11. Department of Pediatric Endocrinology and Metabolism, Okinawa Prefectural Nanbu Medical Center & Children's Medical Center, Okinawa, Japan;12. Division of Endocrinology and Metabolism, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan;13. Division of Pediatrics, Department of Homeostatic Regulation and Development, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan |
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| Abstract: | Although Turner syndrome (TS) is frequently associated with congenital anomalies of the kidney-urinary tract (CAKUT), which is a major cause of pediatric chronic kidney disease, renal function in TS is usually considered normal. The present study aimed to analyze the frequency of renal dysfunction and CAKUT in pediatric patients with TS. Our study included 122 patients with TS between the ages of 2 and 18 years from 30 hospitals across Japan. Clinical data related to renal function and CAKUT were retrospectively collected. The estimated glomerular filtration rate (eGFR) was calculated using the serum creatinine-based formula recommended by the Japanese Society for Pediatric Nephrology. An eGFR <90 mL/min/1.73 m2 for two consecutive years was defined as renal dysfunction. Fifteen (13.5%) of 122 patients had CAKUT, and four patients had renal dysfunction (3.2%, 95% confidence interval: 0%-6.7%). Three of the four did not have CAKUT. Of the CAKUT manifestations, horseshoe kidney, renal hypodysplasia, and multicystic dysplastic kidney were seen in nine, two, and one patient, respectively. Eight of the nine patients with horseshoe kidney had a normal renal function; however, the remaining patient with renal hypodysplasia had renal dysfunction. A small percentage of patients with pediatric TS may had an eGFR below 90 mL/min/1.73 m2 which was not necessarily associated with CAKUT. |
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| Keywords: | CAKUT horseshoe kidney renal function turner syndrome |
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