GSTM1 and Liver Iron Content in Children with Sickle Cell Anemia and Iron Overload Article Swipe
YOU?
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· 2019
· Open Access
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· DOI: https://doi.org/10.3390/jcm8111878
Chronic blood transfusions in patients with sickle cell anemia (SCA) cause iron overload, which occurs with a degree of interpatient variability in serum ferritin and liver iron content (LIC). Reasons for this variability are unclear and may be influenced by genes that regulate iron metabolism. We evaluated the association of the copy number of the glutathione S-transferase M1 (GSTM1) gene and degree of iron overload among patients with SCA. We compared LIC in 38 children with SCA and ≥12 lifetime erythrocyte transfusions stratified by GSTM1 genotype. Baseline LIC was measured using magnetic resonance imaging (MRI), R2*MRI within 3 months prior to, and again after, starting iron unloading therapy. After controlling for weight-corrected transfusion burden (mL/kg) and splenectomy, mean pre-chelation LIC (mg/g dry liver dry weight) was similar in all groups: GSTM1 wild-type (WT) (11.45, SD±6.8), heterozygous (8.2, SD±4.52), and homozygous GSTM1 deletion (GSTM1-null; 7.8, SD±6.9, p = 0.09). However, after >12 months of chelation, GSTM1-null genotype subjects had the least decrease in LIC compared to non-null genotype subjects (mean LIC change for GSTM1-null = 0.1 (SD±3.3); versus −0.3 (SD±3.0) and −1.9 (SD±4.9) mg/g liver dry weight for heterozygous and WT, respectively, p = 0.047). GSTM1 homozygous deletion may prevent effective chelation in children with SCA and iron overload.
Related Topics
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.3390/jcm8111878
- https://www.mdpi.com/2077-0383/8/11/1878/pdf?version=1572952746
- OA Status
- gold
- Cited By
- 4
- References
- 27
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W2983211437
Raw OpenAlex JSON
- OpenAlex ID
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https://openalex.org/W2983211437Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.3390/jcm8111878Digital Object Identifier
- Title
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GSTM1 and Liver Iron Content in Children with Sickle Cell Anemia and Iron OverloadWork title
- Type
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articleOpenAlex work type
- Language
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enPrimary language
- Publication year
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2019Year of publication
- Publication date
-
2019-11-05Full publication date if available
- Authors
-
Latika Puri, Jonathan M. Flanagan, Guolian Kang, Juan Ding, Wenjian Bi, Beth McCarville, Ralf B. Loeffler, Aaryani Tipirneni‐Sajja, Martha Villavicencio, Kristine R. Crews, Claudia M. Hillenbrand, Jane S. HankinsList of authors in order
- Landing page
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https://doi.org/10.3390/jcm8111878Publisher landing page
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https://www.mdpi.com/2077-0383/8/11/1878/pdf?version=1572952746Direct link to full text PDF
- Open access
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YesWhether a free full text is available
- OA status
-
goldOpen access status per OpenAlex
- OA URL
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https://www.mdpi.com/2077-0383/8/11/1878/pdf?version=1572952746Direct OA link when available
- Concepts
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Medicine, Anemia, Dietary iron, Sickle cell anemia, Internal medicine, Gastroenterology, Iron deficiency, DiseaseTop concepts (fields/topics) attached by OpenAlex
- Cited by
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4Total citation count in OpenAlex
- Citations by year (recent)
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2023: 1, 2022: 1, 2021: 1, 2020: 1Per-year citation counts (last 5 years)
- References (count)
-
27Number of works referenced by this work
- Related works (count)
-
10Other works algorithmically related by OpenAlex
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