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Unilateral nephrectomy for young infants with congenital nephrotic syndrome of the Finnish type

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Abstract

Background

The management of congenital nephrotic syndrome of the Finnish type (CNF) is challenging. It is difficult to withdraw intravenous albumin infusions, resulting in long-term hospitalization. In addition, fatal hypotension after bilateral nephrectomy has been reported. In our center, we have performed unilateral nephrectomy during early infancy.

Methods

Infants diagnosed with CNF between 2011 and 2020 in our institution were enrolled. We examined the clinical course before and after unilateral nephrectomy and evaluated the effectiveness of this strategy.

Results

Seven patients (all showing NPHS1 mutations) were enrolled. All required daily intravenous albumin infusion via central venous catheter (CVC). Unilateral nephrectomy was performed at a median of 76 days of age (59–208 days). Surgical complications did not occur in any of patients. The mean albumin dose was decreased after unilateral nephrectomy (2.0 vs 0.4 g/kg/day; p = 0.02). Intravenous albumin infusion could be withdrawn at a median of 17 days, the CVC removed at a median of 21 days, and they discharged at a median of 82 days after unilateral nephrectomy. Although bacterial infections were noted seven times before unilateral nephrectomy, only one episode occurred after surgery. Four patients initiated peritoneal dialysis at two to three years of age and all of them underwent kidney transplantation thereafter.

Conclusions

Unilateral nephrectomy during early infancy may be an effective treatment allowing for withdrawal from albumin infusion, prevention of complications, withdrawal from CVCs and shortening hospital stay for patients with CNF.

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Acknowledgements

We would like to thank Drs. J. Hashimoto, Y. Hamasaki, M. Shimizu, K. Ishikura, and S. Ito for their contributions to this study. We would also like to thank the families of the patients for their willingness to provide clinical data for this study.

Author information

Authors and Affiliations

  1. Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, Japan

    Miki Murakoshi, Koichi Kamei, Masao Ogura, Mai Sato, Taishi Nada, Ryutaro Suzuki, Chikako Kamae, Kentaro Nishi & Toru Kanamori

  2. Department of Pediatrics, Tokyo Medical and Dental University, Tokyo, Japan

    Miki Murakoshi & Toru Kanamori

  3. Department of Pediatrics, Self Defense Forces Central Hospital, Tokyo, Japan

    Chikako Kamae

  4. Department of Pediatrics, Kobe University Graduate School of Medicine, Hyogo, Japan

    China Nagano, Kandai Nozu & Kazumoto Iijima

  5. Department of Child Health and Welfare (Pediatrics), Graduate School of Medicine, University of the Ryukyus, Okinawa, Japan

    Koichi Nakanishi

  6. Hyogo Prefectural Kobe Children’s Hospital, Hyogo, Japan

    Kazumoto Iijima

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  1. Miki Murakoshi
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  2. Koichi Kamei
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  3. Masao Ogura
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  4. Mai Sato
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Corresponding author

Correspondence to Koichi Kamei.

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Conflicts of interest

K. Kamei received grants from Terumo Foundation for Life Sciences and Arts and Ono Pharmaceutical Co. K. Iijima has received grants from Daiichi Sankyo, Co., Ltd., Astellas Pharma Inc., and Zenyaku Kogyo Co. Ltd.

Research involving human and animal participants

The study was conducted according to the principles of the Declaration of Helsinki and the ethical guidelines issued by the Ministry of Health, Labour and Welfare, Japan. This study was approved by the ethics committee of the National Center for Child Health and Development (Approval Number: 2019-172).

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Informed consent for participating in this study was not required in agreement with the above-mentioned guidelines.

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Murakoshi, M., Kamei, K., Ogura, M. et al. Unilateral nephrectomy for young infants with congenital nephrotic syndrome of the Finnish type. Clin Exp Nephrol 26, 162–169 (2022). https://doi.org/10.1007/s10157-021-02141-5

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  • DOI: https://doi.org/10.1007/s10157-021-02141-5

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