Novel variant in MYH9 in a child with proteinuria and thrombocytopenia: a case report and literature review

There is a lack of awareness of the diagnosis and treatment of MYH9-related disorder (MYH9-RD), which is an autosomal dominant disease with heterogeneous clinical manifestations. We summarized the clinical phenotype and reported a novel variant in MYH9 in a child with focal segmental glomerulosclero...

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Main Authors: Dan-Feng Xie, Lin Zhu, Xiao-Meng Wang, Yun Li, Ping Zhou
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-05-01
Series:Frontiers in Pediatrics
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Online Access:https://www.frontiersin.org/articles/10.3389/fped.2025.1502727/full
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author Dan-Feng Xie
Dan-Feng Xie
Lin Zhu
Lin Zhu
Xiao-Meng Wang
Xiao-Meng Wang
Yun Li
Yun Li
Ping Zhou
Ping Zhou
author_facet Dan-Feng Xie
Dan-Feng Xie
Lin Zhu
Lin Zhu
Xiao-Meng Wang
Xiao-Meng Wang
Yun Li
Yun Li
Ping Zhou
Ping Zhou
author_sort Dan-Feng Xie
collection DOAJ
description There is a lack of awareness of the diagnosis and treatment of MYH9-related disorder (MYH9-RD), which is an autosomal dominant disease with heterogeneous clinical manifestations. We summarized the clinical phenotype and reported a novel variant in MYH9 in a child with focal segmental glomerulosclerosis (FSGS) and reviewed the relevant literature to better understand MYH9-RD. Unlike previous cases, this patient exhibited IgA deposition in the mesangial region, suggesting need for further investigation into prognostic and therapeutic significance of this finding. To reduce the risk of MYH9-RD misdiagnosis, we recommend assessing mean platelet diameter and granulocyte inclusions in patients with unexplained proteinuria and refractory thrombocytopenia.
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publishDate 2025-05-01
publisher Frontiers Media S.A.
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series Frontiers in Pediatrics
spelling doaj-art-880fa9c5895e41f9bd5ddc68e838d4e92025-08-20T02:15:16ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602025-05-011310.3389/fped.2025.15027271502727Novel variant in MYH9 in a child with proteinuria and thrombocytopenia: a case report and literature reviewDan-Feng Xie0Dan-Feng Xie1Lin Zhu2Lin Zhu3Xiao-Meng Wang4Xiao-Meng Wang5Yun Li6Yun Li7Ping Zhou8Ping Zhou9Department of Pediatric Nephrology, Allergy, and Rheumatology, Sichuan Provincial Women’s and Children’s Hospital, The Affiliated Women’s and Children’s Hospital of Chengdu Medical College, Chengdu, Sichuan, ChinaSichuan Clinical Research Center for Pediatric Nephrology, Chengdu, Sichuan, ChinaDepartment of Pediatric Nephrology, Allergy, and Rheumatology, Sichuan Provincial Women’s and Children’s Hospital, The Affiliated Women’s and Children’s Hospital of Chengdu Medical College, Chengdu, Sichuan, ChinaSichuan Clinical Research Center for Pediatric Nephrology, Chengdu, Sichuan, ChinaDepartment of Pediatric Nephrology, Allergy, and Rheumatology, Sichuan Provincial Women’s and Children’s Hospital, The Affiliated Women’s and Children’s Hospital of Chengdu Medical College, Chengdu, Sichuan, ChinaSichuan Clinical Research Center for Pediatric Nephrology, Chengdu, Sichuan, ChinaDepartment of Pediatric Nephrology, Allergy, and Rheumatology, Sichuan Provincial Women’s and Children’s Hospital, The Affiliated Women’s and Children’s Hospital of Chengdu Medical College, Chengdu, Sichuan, ChinaSichuan Clinical Research Center for Pediatric Nephrology, Chengdu, Sichuan, ChinaDepartment of Pediatric Nephrology, Allergy, and Rheumatology, Sichuan Provincial Women’s and Children’s Hospital, The Affiliated Women’s and Children’s Hospital of Chengdu Medical College, Chengdu, Sichuan, ChinaSichuan Clinical Research Center for Pediatric Nephrology, Chengdu, Sichuan, ChinaThere is a lack of awareness of the diagnosis and treatment of MYH9-related disorder (MYH9-RD), which is an autosomal dominant disease with heterogeneous clinical manifestations. We summarized the clinical phenotype and reported a novel variant in MYH9 in a child with focal segmental glomerulosclerosis (FSGS) and reviewed the relevant literature to better understand MYH9-RD. Unlike previous cases, this patient exhibited IgA deposition in the mesangial region, suggesting need for further investigation into prognostic and therapeutic significance of this finding. To reduce the risk of MYH9-RD misdiagnosis, we recommend assessing mean platelet diameter and granulocyte inclusions in patients with unexplained proteinuria and refractory thrombocytopenia.https://www.frontiersin.org/articles/10.3389/fped.2025.1502727/fullproteinuriathrombocytopenialiver dysfunctionFSGSMYH9-RDchild
spellingShingle Dan-Feng Xie
Dan-Feng Xie
Lin Zhu
Lin Zhu
Xiao-Meng Wang
Xiao-Meng Wang
Yun Li
Yun Li
Ping Zhou
Ping Zhou
Novel variant in MYH9 in a child with proteinuria and thrombocytopenia: a case report and literature review
Frontiers in Pediatrics
proteinuria
thrombocytopenia
liver dysfunction
FSGS
MYH9-RD
child
title Novel variant in MYH9 in a child with proteinuria and thrombocytopenia: a case report and literature review
title_full Novel variant in MYH9 in a child with proteinuria and thrombocytopenia: a case report and literature review
title_fullStr Novel variant in MYH9 in a child with proteinuria and thrombocytopenia: a case report and literature review
title_full_unstemmed Novel variant in MYH9 in a child with proteinuria and thrombocytopenia: a case report and literature review
title_short Novel variant in MYH9 in a child with proteinuria and thrombocytopenia: a case report and literature review
title_sort novel variant in myh9 in a child with proteinuria and thrombocytopenia a case report and literature review
topic proteinuria
thrombocytopenia
liver dysfunction
FSGS
MYH9-RD
child
url https://www.frontiersin.org/articles/10.3389/fped.2025.1502727/full
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