Retrospective study of the renal function using estimated glomerular filtration rate and congenital anomalies of the kidney‐urinary tract in pediatric Turner syndrome

  • Yukie Izumita
    Department of Pediatrics Niigata City General Hospital Niigata Japan
  • Satsuki Nishigaki
    Department of Pediatrics Osaka City University Graduate School of Medicine Osaka Japan
  • Mari Satoh
    Department of Pediatrics Toho University Omori Medical Center Tokyo Japan
  • Noriyuki Takubo
    Department of Pediatrics Juntendo University Faculty of Medicine Tokyo Japan
  • Chikahiko Numakura
    Department of Pediatrics Yamagata University School of Medicine Yamagata Japan
  • Ikuko Takahashi
    Department of Pediatrics Akita University Graduate School of Medicine Akita Japan
  • Shun Soneda
    Department of Pediatrics St. Marianna University School of Medicine Kanagawa Japan
  • Yoshifusa Abe
    Children Medical Center Showa University Koto Toyosu Hospital Tokyo Japan
  • Hotaka Kamasaki
    Department of Pediatrics Sapporo Medical University School of Medicine Sapporo Japan
  • Yoshiaki Ohtsu
    Department of Pediatrics Gunma University Graduate School of Medicine Maebashi Japan
  • Junko Igaki
    Department of Pediatric Endocrinology and Metabolism Okinawa Prefectural Nanbu Medical Center & Children's Medical Center Okinawa Japan
  • Yukihiro Hasegawa
    Division of Endocrinology and Metabolism Tokyo Metropolitan Children's Medical Center Tokyo Japan
  • Keisuke Nagasaki
    Division of Pediatrics, Department of Homeostatic Regulation and Development Niigata University Graduate School of Medical and Dental Sciences Niigata Japan

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<jats:title>Abstract</jats:title><jats:p>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 m<jats:sup>2</jats:sup> 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 m<jats:sup>2</jats:sup> which was not necessarily associated with CAKUT.</jats:p>

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