Abstract
There are no clinical guidelines for performing nephrectomy in patients with autosomal recessive polycystic kidney disease (ARPKD). Few reports have described the clinical course of ARPKD diagnosed in the neonatal period in detail. Here, we report seven patients diagnosed with ARPKD and treated at our center during the neonatal period. Two died within 48 h of life due to pulmonary hypoplasia. Of the remaining five patients, three had anuria and required for kidney replacement therapy (KRT) within one week after birth, whereas two with a milder phenotype survived without KRT. All three patients who received KRT underwent unilateral nephrectomy and peritoneal dialysis (PD) catheter placement. To prevent fluid leakage, PD was initiated 7–14 days after catheter placement. However, peritoneal leakage occurred in two patients, resulting in peritonitis and discontinuation of PD; one who required long-term hemodialysis contracted a catheter-related bloodstream infection as well as developed subdural and epidural hematomas. Meanwhile, two patients underwent a second nephrectomy within 6 weeks after birth; one developed severe persistent hypotension and neurological complications, while the other died of bacteremia that may have resulted from cholangitis diagnosed on day 67 of life. A severe clinical course, life-threatening adverse events, and severe neurological sequalae may occur in patients with ARPKD who receive KRT in neonatal period.
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Acknowledgements
The authors would like to thank Drs. Kandai Nozu, Naoya Morisada, and Hiroki Kurahashi for genetic testing.
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Koichi Kamei has received research funding from the Public Foundation of Vaccination Research Center, and the Taiju Life Social Welfare Foundation; donations from Chugai Pharmaceutical Co. Ltd., Teijin Pharma Ltd., Kyowa Kirin Co. Ltd., Shionogi & Co. Ltd., Daiichi Sankyo Co. Ltd., Mitsubishi Tanabe Pharma Co. Ltd, and Otsuka Pharmaceutical Co. Ltd.; and lecture fees from Terumo Co. Ltd., Baxter Ltd., and Zenyaku Kogyo Co., Ltd. Other authors have no potential conflicts of interests to disclose.
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The study was conducted according to the Helsinki Declaration’s principle and the Japanese Ministry of Health, Labor, and Welfare’s ethical guidelines. The study was approved by the Ethics Committee of the National Center for Child Health and Development (approval number: 2022-196).
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Inoki, Y., Nishi, K., Osaka, K. et al. Complications and prognosis of patients diagnosed with autosomal recessive polycystic kidney disease in neonatal period. CEN Case Rep (2023). https://doi.org/10.1007/s13730-023-00827-1
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DOI: https://doi.org/10.1007/s13730-023-00827-1