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Clinical and pathological investigation of oligomeganephronia

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Abstract

Background

Oligomeganephronia (OMN) is a rare congenital anomaly involving the kidney and urinary tract, characterized by decreased number and compensatory hypertrophy of the nephron. It is caused by abnormal kidney development during the embryonic period, especially in patients with low birth weight; however, the actual etiology and clinical features remain unknown. We aim to reveal the clinical and pathological characteristics, treatment, and outcome.

Methods

Ten patients diagnosed with OMN between 2013 and 2020 were retrospectively investigated. The data were presented as the median ± interquartile range, and statistical significance was set at p < 0.05.

Results

The age at diagnosis was 14.1 years, the male-to-female ratio was 6:4, and only four cases were born with low birth weight. The estimated glomerular filtration rate (eGFR) was 62.2 mL/min/1.73 m2. The glomerulus diameter of OMN patients was significantly larger (217 vs. 154 µm, p < 0.001) in OMN patients, and the number of glomeruli of OMN patients was lower (0.89 vs. 2.05/mm2, p < 0.001) than the control group. Eight of the ten cases were identified by urinary screening. Nine patients were treated with renin–angiotensin system (RAS) inhibitors, following which proteinuria successfully decreased or disappeared. Their median eGFR was also stable, 53.3 mL/min/1.73 m2.

Conclusions

As few symptoms can lead to OMN discovery, most patients were found during urine screening at school. Kidney dysfunction was observed in all patients at the time of kidney biopsy. Proteinuria has been significantly reduced and the decline rate of eGFR might be improved by RAS inhibitors.

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Data availability

Data from this study can be obtained from the corresponding authors on reasonable request.

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Authors and Affiliations

  1. Department of Pediatrics, Kobe University Graduate School of Medicine, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan

    Hideaki Kitakado, Tomoko Horinouchi, Chika Masuda, Atsushi Kondo, Sadayuki Nagai, Yuya Aoto, Nana Sakakibara, Takeshi Ninchoji & Kandai Nozu

  2. Department of Pediatrics, Steel Memorial Hirohata Hospital, Himeji, Japan

    Takeshi Ninchoji

  3. Clinical Research Center, Takatsuki General Hospital, Takatsuki, Japan

    Norishige Yoshikawa

Authors
  1. Hideaki Kitakado
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Contributions

HK, TH, and TN designed the study concept, wrote the manuscript, and interpreted the data. HK, TH, CM, AK, SN, YA, NS, and TN conducted kidney biopsies. NY performed pathological evaluation. KN critically reviewed the manuscript. All the authors have read and approved the final manuscript.

Corresponding author

Correspondence to Tomoko Horinouchi.

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This study involving human participants was conducted in accordance with the Declaration of Helsinki. Ethics approval was obtained from the Ethics Review Committee of Kobe University Graduate School of Medicine.

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The authors declare no competing interests.

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Kitakado, H., Horinouchi, T., Masuda, C. et al. Clinical and pathological investigation of oligomeganephronia. Pediatr Nephrol 38, 757–762 (2023). https://doi.org/10.1007/s00467-022-05687-y

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