Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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Nutcracker syndrome combined with immunoglobulin A nephropathy: two case reports

  • So Hyun Ki (Department of Pediatrics, Korea University Guro Hospital) ;
  • Min Hwa Son (Department of Pediatrics, Korea University Ansan Hospital) ;
  • Eujin Park (Department of Pediatrics, Korea University Guro Hospital) ;
  • Hyung Eun Yim (Department of Pediatrics, Korea University Ansan Hospital)
  • Received : 2023.08.08
  • Accepted : 2023.09.30
  • Published : 2023.12.31
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Abstract

Nutcracker syndrome (NCS) is a disease caused by compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. Immunoglobulin A (IgA) nephropathy (IgAN) is characterized by the predominance of IgA deposits in the glomerular mesangial area. Hematuria and proteinuria can be present in both diseases, and some patients can be concurrently diagnosed with NCS and IgAN; however, a causal relationship between the two diseases has not yet been clarified. Here, we report two pediatric cases of NCS combined with IgAN. The first patient presenting with microscopic hematuria and proteinuria was diagnosed with NCS at the initial visit, and the second patient was later diagnosed with NCS when proteinuria worsened. Both patients were diagnosed with IgAN based on kidney biopsy findings and treated with angiotensin-converting enzyme inhibitors and immunosuppressants. A high index of suspicion and timely imaging or biopsy are essential for the proper management of NCS combined with glomerulopathy.

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References

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