Erdheim–Chester Disease With MAP2K (p.K57N) Mutation Diagnosed With Kidney Biopsy

This case study presents Erdheim–Chester disease (ECD) diagnosed by kidney biopsy in a middle-aged woman who needed hemodialysis. She was admitted with dyspnea, congestive heart failure, and acute kidney injury. A kidney biopsy revealed histiocytosis renal infiltration and molecular mutation (MAP2K...

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Main Authors: Marina López-Martínez, Fernando Martínez-Valle, Alejandra Gabaldón, Juan Carlos León-Román, María Teresa Salcedo, María José Soler
Format: Article
Language:English
Published: American College of Physicians 2024-08-01
Series:Annals of Internal Medicine: Clinical Cases
Online Access:https://www.acpjournals.org/doi/10.7326/aimcc.2023.1497
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author Marina López-Martínez
Fernando Martínez-Valle
Alejandra Gabaldón
Juan Carlos León-Román
María Teresa Salcedo
María José Soler
author_facet Marina López-Martínez
Fernando Martínez-Valle
Alejandra Gabaldón
Juan Carlos León-Román
María Teresa Salcedo
María José Soler
author_sort Marina López-Martínez
collection DOAJ
description This case study presents Erdheim–Chester disease (ECD) diagnosed by kidney biopsy in a middle-aged woman who needed hemodialysis. She was admitted with dyspnea, congestive heart failure, and acute kidney injury. A kidney biopsy revealed histiocytosis renal infiltration and molecular mutation (MAP2K p.K57N) in the mitogen-activated protein kinase (MAPK) pathway, confirming ECD. Corticosteroid treatment resulsted in recovered renal function after 3 months. To our knowledge, this is the first well-documented case of this association. In patients with acute kidney injury and histiocytosis infiltration in the kidney, ECD should be considered, with steroids being a plausible treatment of histiocytosis infiltration in the kidney.
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issn 2767-7664
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publishDate 2024-08-01
publisher American College of Physicians
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series Annals of Internal Medicine: Clinical Cases
spelling doaj-art-6e5dd0d09a664c96be5e2a2ed8cee9d22025-08-20T02:11:43ZengAmerican College of PhysiciansAnnals of Internal Medicine: Clinical Cases2767-76642024-08-013810.7326/aimcc.2023.1497Erdheim–Chester Disease With MAP2K (p.K57N) Mutation Diagnosed With Kidney BiopsyMarina López-Martínez0Fernando Martínez-Valle1Alejandra Gabaldón2Juan Carlos León-Román3María Teresa Salcedo4María José Soler51Department of Nephrology, Vall d'Hebron University Hospital, Barcelona, Spain2Department of Internal Medicine, Vall d'Hebron University Hospital, Barcelona, Spain3Department of Pathology, Vall d'Hebron University Hospital, Barcelona, Spain1Department of Nephrology, Vall d'Hebron University Hospital, Barcelona, Spain3Department of Pathology, Vall d'Hebron University Hospital, Barcelona, Spain1Department of Nephrology, Vall d'Hebron University Hospital, Barcelona, SpainThis case study presents Erdheim–Chester disease (ECD) diagnosed by kidney biopsy in a middle-aged woman who needed hemodialysis. She was admitted with dyspnea, congestive heart failure, and acute kidney injury. A kidney biopsy revealed histiocytosis renal infiltration and molecular mutation (MAP2K p.K57N) in the mitogen-activated protein kinase (MAPK) pathway, confirming ECD. Corticosteroid treatment resulsted in recovered renal function after 3 months. To our knowledge, this is the first well-documented case of this association. In patients with acute kidney injury and histiocytosis infiltration in the kidney, ECD should be considered, with steroids being a plausible treatment of histiocytosis infiltration in the kidney.https://www.acpjournals.org/doi/10.7326/aimcc.2023.1497
spellingShingle Marina López-Martínez
Fernando Martínez-Valle
Alejandra Gabaldón
Juan Carlos León-Román
María Teresa Salcedo
María José Soler
Erdheim–Chester Disease With MAP2K (p.K57N) Mutation Diagnosed With Kidney Biopsy
Annals of Internal Medicine: Clinical Cases
title Erdheim–Chester Disease With MAP2K (p.K57N) Mutation Diagnosed With Kidney Biopsy
title_full Erdheim–Chester Disease With MAP2K (p.K57N) Mutation Diagnosed With Kidney Biopsy
title_fullStr Erdheim–Chester Disease With MAP2K (p.K57N) Mutation Diagnosed With Kidney Biopsy
title_full_unstemmed Erdheim–Chester Disease With MAP2K (p.K57N) Mutation Diagnosed With Kidney Biopsy
title_short Erdheim–Chester Disease With MAP2K (p.K57N) Mutation Diagnosed With Kidney Biopsy
title_sort erdheim chester disease with map2k p k57n mutation diagnosed with kidney biopsy
url https://www.acpjournals.org/doi/10.7326/aimcc.2023.1497
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