Nutcracker syndrome combined with immunoglobulin A nephropathy: two case reports

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 proteinur...

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Main Authors: So Hyun Ki, Min Hwa Son, Eujin Park, Hyung Eun Yim
Format: Article
Language:English
Published: Korean Society of Pediatric Nephrology 2023-12-01
Series:Childhood Kidney Diseases
Subjects:
Online Access:http://www.chikd.org/upload/ckd-23-013.pdf
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author So Hyun Ki
Min Hwa Son
Eujin Park
Hyung Eun Yim
author_facet So Hyun Ki
Min Hwa Son
Eujin Park
Hyung Eun Yim
author_sort So Hyun Ki
collection DOAJ
description 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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spelling doaj.art-39d80e8ce0b448108f9446fd8e4862622024-05-22T05:33:17ZengKorean Society of Pediatric NephrologyChildhood Kidney Diseases2384-02422384-02502023-12-0127213313810.3339/ckd.23.013789Nutcracker syndrome combined with immunoglobulin A nephropathy: two case reportsSo Hyun Ki0Min Hwa Son1Eujin Park2Hyung Eun Yim3 Department of Pediatrics, Korea University Guro Hospital, Seoul, Republic of Korea Department of Pediatrics, Korea University Ansan Hospital, Ansan, Republic of Korea Department of Pediatrics, Korea University Guro Hospital, Seoul, Republic of Korea Department of Pediatrics, Korea University Ansan Hospital, Ansan, Republic of KoreaNutcracker 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.http://www.chikd.org/upload/ckd-23-013.pdfcase reportsglomerulonephritisimmunoglobulin arenal nutcracker syndrome
spellingShingle So Hyun Ki
Min Hwa Son
Eujin Park
Hyung Eun Yim
Nutcracker syndrome combined with immunoglobulin A nephropathy: two case reports
Childhood Kidney Diseases
case reports
glomerulonephritis
immunoglobulin a
renal nutcracker syndrome
title Nutcracker syndrome combined with immunoglobulin A nephropathy: two case reports
title_full Nutcracker syndrome combined with immunoglobulin A nephropathy: two case reports
title_fullStr Nutcracker syndrome combined with immunoglobulin A nephropathy: two case reports
title_full_unstemmed Nutcracker syndrome combined with immunoglobulin A nephropathy: two case reports
title_short Nutcracker syndrome combined with immunoglobulin A nephropathy: two case reports
title_sort nutcracker syndrome combined with immunoglobulin a nephropathy two case reports
topic case reports
glomerulonephritis
immunoglobulin a
renal nutcracker syndrome
url http://www.chikd.org/upload/ckd-23-013.pdf
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