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Multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney: CT and MRI findings and clinical characteristic

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Abstract

Purpose

The aim of this study was to clarify the radiologic and clinical characteristics of multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney.

Method

Fourteen patients with unique and characteristic multiple hemorrhagic subcapsular cortical cysts of the kidney, not categorized in any existing renal cystic diseases, were retrospectively reviewed. The clinical information including age, sex, symptom, family history of renal or renal cystic disease, and laboratory data were collected. CT and MRI findings including distribution, number and size of cysts, and CT attenuation and signal intensity on T1- and T2-weighted MRI of cysts were analyzed.

Results

All patients except one were young and none had a family history of renal or renal cystic disease. Common clinical symptoms were flank or abdominal pain and hematuria. In all cases, only the left kidney was involved at initial presentation. Cysts were small (median cyst size, 4–15 mm), numerous, and distributed mainly along the subcapsular cortex of the kidney. Cysts were hyper-attenuated on unenhanced CT, extremely hypointense on T2-weighted MRI, and mildly hyperintense on T1-weighted MRI. All patients except one had normal renal function. Imaging follow-up revealed stable or mildly progressive disease in seven patients. Two patients developed several hemorrhagic subcapsular cortical cysts in the right kidney at follow-up. Three of five patients with a renal pathology specimen showed concurrent IgA nephropathy.

Conclusion

We have identified a unique renal cystic disease with multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney that has a characteristic manifestation both radiologically and clinically.

Key Points

• Multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney is a unique non-familial renal cystic disease with a characteristic manifestation both radiologically and clinically.

• Most cases of multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney are stable or slowly progressive, and do not require invasive intervention.

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Abbreviations

ADPKD:

Autosomal dominant polycystic kidney disease

CT:

Computed tomography

MRI:

Magnetic resonance imaging

MUCH cystic disease:

Multiple unilateral subcapsular cortical hemorrhagic cystic disease

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Funding

This work was supported by the National Institute of Diabetes and Digestive and Kidney Diseases under NIH Grant/Award Number P30 DK090728 to the Mayo Clinic Robert M. and Billie Kelley Pirnie Translational Polycystic Kidney Disease Center.

Author information

Authors and Affiliations

  1. Department of Radiology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Kotaro Yoshida, Naoki Takahashi, Bernard F. King & Dai Inoue

  2. Department of Radiology, Mayo Clinic, Scottsdale, AZ, USA

    Akira Kawashima

  3. Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA

    Peter C. Harris, Emilie Cornec-Le Gall & Vicente E. Torres

  4. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA

    Lynn D. Cornell

  5. Department of Nephrology, University Hospital, Brest, France

    Emilie Cornec-Le Gall

  6. Department of Radiology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan

    Dai Inoue

  7. Division of Rheumatology, Department of Cardiovascular and Internal Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Japan

    Ichiro Mizushima & Mitsuhiro Kawano

  8. Department of Nephrology, European Hospital Georges Pompidou, APHP, Paris Descartes University, Paris, France

    Eric Thervet

  9. Department of Medicine, Division of Nephrology, Laval University, Quebec, Canada

    Paul René de Cotret

Authors
  1. Kotaro Yoshida
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  2. Naoki Takahashi
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  3. Bernard F. King
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  8. Dai Inoue
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  10. Mitsuhiro Kawano
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  11. Eric Thervet
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  12. Paul René de Cotret
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  13. Vicente E. Torres
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Corresponding author

Correspondence to Naoki Takahashi.

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Guarantor

The scientific guarantor of this publication is Naoki Takahashi.

Conflict of interest

The authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article.

Statistics and biometry

No complex statistical methods were necessary for this paper.

Informed consent

Written informed consent was waived by the Institutional Review Board.

Ethical approval

Institutional Review Board approval was obtained.

Study subjects or cohorts overlap

Any study subjects have not been previously reported.

Methodology

• retrospective

• observational

• multicenter study

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Yoshida, K., Takahashi, N., King, B.F. et al. Multiple unilateral subcapsular cortical hemorrhagic cystic disease of the kidney: CT and MRI findings and clinical characteristic. Eur Radiol 29, 4843–4850 (2019). https://doi.org/10.1007/s00330-019-06057-3

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  • DOI: https://doi.org/10.1007/s00330-019-06057-3

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