Coexistence or a related condition: an infant with retinoblastoma and Gaucher disease

Gaucher disease (GD) is the most prevalant lysosomal lipid storage disease that results from loss of function of acid β-glucosidase due to mutations in the glucocerebrosidase gene. Common features of all types of GD include hepatosplenomegaly, cytopenia, and various patterns of bone and lung involv...

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Main Authors: Burcu Berberoğlu-Ateş, Ali Varan, Hülya Demir, Canan Akyüz, Aysel Yüce
Format: Article
Language:English
Published: Hacettepe University Institute of Child Health 2019-06-01
Series:The Turkish Journal of Pediatrics
Subjects:
Online Access:https://turkjpediatr.org/article/view/718
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author Burcu Berberoğlu-Ateş
Ali Varan
Hülya Demir
Canan Akyüz
Aysel Yüce
author_facet Burcu Berberoğlu-Ateş
Ali Varan
Hülya Demir
Canan Akyüz
Aysel Yüce
author_sort Burcu Berberoğlu-Ateş
collection DOAJ
description Gaucher disease (GD) is the most prevalant lysosomal lipid storage disease that results from loss of function of acid β-glucosidase due to mutations in the glucocerebrosidase gene. Common features of all types of GD include hepatosplenomegaly, cytopenia, and various patterns of bone and lung involvement. Retinoblastoma is a malignant tumor of the developing retina that occurs in children, typically before the age of five. Retinoblastoma develops from cells that have cancer-predisposing variants in both copies of RB1. The association between GD and retinoblastoma has not been reported until now. Here we report the case that was diagnosed with, retinoblastoma at the age of 2 months and then GD at the age of 11 months. Although there are controversies concerning the association between GD and cancer; malignancies should be kept in mind during GD patients follow up.
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issn 0041-4301
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language English
publishDate 2019-06-01
publisher Hacettepe University Institute of Child Health
record_format Article
series The Turkish Journal of Pediatrics
spelling doaj-art-94b40517c24c426780cb052e2e8e5f492025-08-20T03:00:53ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212019-06-0161310.24953/turkjped.2019.03.022Coexistence or a related condition: an infant with retinoblastoma and Gaucher diseaseBurcu Berberoğlu-Ateş0Ali Varan1Hülya Demir2Canan Akyüz3Aysel Yüce4Departments of Pediatric Gastroenterology, Hepatology and Nutrition Hacettepe University Faculty of Medicine, Ankara, Turkey.Departments of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.Departments of Pediatric Gastroenterology, Hepatology and Nutrition Hacettepe University Faculty of Medicine, Ankara, Turkey.Departments of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.Departments of Pediatric Gastroenterology, Hepatology and Nutrition Hacettepe University Faculty of Medicine, Ankara, Turkey. Gaucher disease (GD) is the most prevalant lysosomal lipid storage disease that results from loss of function of acid β-glucosidase due to mutations in the glucocerebrosidase gene. Common features of all types of GD include hepatosplenomegaly, cytopenia, and various patterns of bone and lung involvement. Retinoblastoma is a malignant tumor of the developing retina that occurs in children, typically before the age of five. Retinoblastoma develops from cells that have cancer-predisposing variants in both copies of RB1. The association between GD and retinoblastoma has not been reported until now. Here we report the case that was diagnosed with, retinoblastoma at the age of 2 months and then GD at the age of 11 months. Although there are controversies concerning the association between GD and cancer; malignancies should be kept in mind during GD patients follow up. https://turkjpediatr.org/article/view/718Gaucher diseasecancerretinoblastoma
spellingShingle Burcu Berberoğlu-Ateş
Ali Varan
Hülya Demir
Canan Akyüz
Aysel Yüce
Coexistence or a related condition: an infant with retinoblastoma and Gaucher disease
The Turkish Journal of Pediatrics
Gaucher disease
cancer
retinoblastoma
title Coexistence or a related condition: an infant with retinoblastoma and Gaucher disease
title_full Coexistence or a related condition: an infant with retinoblastoma and Gaucher disease
title_fullStr Coexistence or a related condition: an infant with retinoblastoma and Gaucher disease
title_full_unstemmed Coexistence or a related condition: an infant with retinoblastoma and Gaucher disease
title_short Coexistence or a related condition: an infant with retinoblastoma and Gaucher disease
title_sort coexistence or a related condition an infant with retinoblastoma and gaucher disease
topic Gaucher disease
cancer
retinoblastoma
url https://turkjpediatr.org/article/view/718
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