Kentaro Nishi1, Osamu Uemura2, Ryoko Harada3, Masaki Yamamoto4, Yusuke Okuda5, Kenichiro Miura6, Yoshimitsu Gotoh7, Tomoo Kise8, Daishi Hirano9, Yuko Hamasaki10, Naoya Fujita11, Toru Uchimura12, Takeshi Ninchoji13, Tetsuya Isayama14, Riku Hamada3, Koichi Kamei1, Tetsuji Kaneko15,16, Kenji Ishikura17. 1. Division of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan. 2. Ichinomiya Medical Treatment and Habilitation Center, Aichi, Japan. 3. Department of Nephrology and Rheumatology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan. 4. Department of Pediatrics, Seirei Hamamatsu General Hospital, Shizuoka, Japan. 5. Department of Pediatrics, Kitasato University School of Medicine, 1-15-1 Kitazato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan. 6. Department of Pediatric Nephrology, Tokyo Women's Medical University, Tokyo, Japan. 7. Department of Pediatric Nephrology, Japanese Red Cross Aichi Medical Center Nagoya Daini Hospital, Aichi, Japan. 8. Department of Pediatric Nephrology, Okinawa Prefectural Nanbu Medical Center, Children's Medical Center, Okinawa, Japan. 9. Department of Pediatrics, The Jikei University School of Medicine, Tokyo, Japan. 10. Department of Nephrology, Toho University Faculty of Medicine, Tokyo, Japan. 11. Department of Nephrology, Aichi Children's Health and Medical Center, Aichi, Japan. 12. Department of Pediatrics, Yokohama City University Medical Center, Kanagawa, Japan. 13. Department of Pediatrics, Kobe University Graduate School of Medicine, Hyogo, Japan. 14. Division of Neonatology, Center of Maternal-Fetal Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan. 15. Clinical Research Support Center, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan. 16. Teikyo Academic Research Center, Teikyo University, Tokyo, Japan. 17. Department of Pediatrics, Kitasato University School of Medicine, 1-15-1 Kitazato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan. k-ishikura@umin.ac.jp.
Abstract
BACKGROUND: Severe congenital anomalies of the kidney and urinary tract (CAKUT) progress to infantile kidney failure with replacement therapy (KFRT). Although prompt and precise prediction of kidney outcomes is important, early predictive factors for its progression remain incompletely defined. METHODS: This retrospective cohort study included patients with CAKUT treated at 12 centers between 2009 and 2020. Patients with a maximum serum creatinine level ≤ 1.0 mg/dL during the first 3 days, patients who died of respiratory failure during the neonatal period, patients who progressed to KFRT within the first 3 days, and patients lacking sufficient data were excluded. RESULTS: Of 2187 patients with CAKUT, 92 were finally analyzed. Twenty-five patients (27%) progressed to KFRT and 24 (26%) had stage 3-5 chronic kidney disease without replacement therapy during the median observation period of 52.0 (interquartile range, 22.0-87.8) months. Among these, 22 (24%) progressed to infantile KFRT. The kidney survival rate during the infantile period was significantly lower in patients with a maximum serum creatinine level during the first 3 days (Cr-day3-max) ≥ 2.5 mg/dL (21.8%) compared with those with a Cr-day3-max < 2.5 mg/dL (95.2%) (log-rank, P < 0.001). Multivariate analysis demonstrated Cr-day3-max (P < 0.001) and oligohydramnios (P = 0.025) were associated with higher risk of infantile KFRT. Eighty-two patients (89%) were alive at the last follow-up. CONCLUSIONS: Neonatal kidney function, including Cr-day3-max, was associated with kidney outcomes in patients with severe CAKUT. Aggressive therapy for severe CAKUT may have good long-term life outcomes through infantile dialysis and kidney transplantation. A higher resolution version of the Graphical abstract is available as Supplementary information.
BACKGROUND: Severe congenital anomalies of the kidney and urinary tract (CAKUT) progress to infantile kidney failure with replacement therapy (KFRT). Although prompt and precise prediction of kidney outcomes is important, early predictive factors for its progression remain incompletely defined. METHODS: This retrospective cohort study included patients with CAKUT treated at 12 centers between 2009 and 2020. Patients with a maximum serum creatinine level ≤ 1.0 mg/dL during the first 3 days, patients who died of respiratory failure during the neonatal period, patients who progressed to KFRT within the first 3 days, and patients lacking sufficient data were excluded. RESULTS: Of 2187 patients with CAKUT, 92 were finally analyzed. Twenty-five patients (27%) progressed to KFRT and 24 (26%) had stage 3-5 chronic kidney disease without replacement therapy during the median observation period of 52.0 (interquartile range, 22.0-87.8) months. Among these, 22 (24%) progressed to infantile KFRT. The kidney survival rate during the infantile period was significantly lower in patients with a maximum serum creatinine level during the first 3 days (Cr-day3-max) ≥ 2.5 mg/dL (21.8%) compared with those with a Cr-day3-max < 2.5 mg/dL (95.2%) (log-rank, P < 0.001). Multivariate analysis demonstrated Cr-day3-max (P < 0.001) and oligohydramnios (P = 0.025) were associated with higher risk of infantile KFRT. Eighty-two patients (89%) were alive at the last follow-up. CONCLUSIONS: Neonatal kidney function, including Cr-day3-max, was associated with kidney outcomes in patients with severe CAKUT. Aggressive therapy for severe CAKUT may have good long-term life outcomes through infantile dialysis and kidney transplantation. A higher resolution version of the Graphical abstract is available as Supplementary information.
Authors: Kariljn J van Stralen; Dagmara Borzych-Dużalka; Hiroshi Hataya; Sean E Kennedy; Kitty J Jager; Enrico Verrina; Carol Inward; Kai Rönnholm; Karel Vondrak; Bradley A Warady; Aleksandra M Zurowska; Franz Schaefer; Pierre Cochat Journal: Kidney Int Date: 2014-02-05 Impact factor: 10.612
Authors: Aleksandra M Zurowska; Michel Fischbach; Alan R Watson; Alberto Edefonti; Constantinos J Stefanidis Journal: Pediatr Nephrol Date: 2012-10-09 Impact factor: 3.714