Varied Age of First Presentation of Sickle Cell Disease: Case Presentations and Review
Sickle cell disease is a multisystem condition characterized by hemolytic anemia and vasoocclusion. Not only are the symptoms of the first presentation but also the ages of presentation are very variable. Following three case reports, different causes of possible late presentation are discussed. Man...
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| Format: | Article |
| Language: | English |
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Wiley
2021-01-01
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| Series: | Case Reports in Medicine |
| Online Access: | http://dx.doi.org/10.1155/2021/8895020 |
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| author | Alexis Claeys Susanne Van Steijn Lydia Van Kesteren Elizabet Damen Machiel Van Den Akker |
| author_facet | Alexis Claeys Susanne Van Steijn Lydia Van Kesteren Elizabet Damen Machiel Van Den Akker |
| author_sort | Alexis Claeys |
| collection | DOAJ |
| description | Sickle cell disease is a multisystem condition characterized by hemolytic anemia and vasoocclusion. Not only are the symptoms of the first presentation but also the ages of presentation are very variable. Following three case reports, different causes of possible late presentation are discussed. Many factors are responsible for the age at which sickle cell disease is diagnosed: doctor’s delay (unfamiliarity with the disease), patient’s delay (education and financial position of the parents, cultural factors), high- versus low-resource country (availability of newborn screening), fetal hemoglobin, reticulocyte count, and genetic modulators, such as SCD genotype, alpha-thalassemia, fetal hemoglobin concentration, and G6PD deficiency. The individual course of sickle cell disease depends on (epi) genetic and environmental properties and the underlying interactions. In further studies, the role of each factor should be evaluated more deeply, and its use as a marker of disease severity or activity should be assessed. |
| format | Article |
| id | doaj-art-e610329b27984719a136fd7221f180d9 |
| institution | Kabale University |
| issn | 1687-9627 1687-9635 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Medicine |
| spelling | doaj-art-e610329b27984719a136fd7221f180d92025-02-03T06:43:35ZengWileyCase Reports in Medicine1687-96271687-96352021-01-01202110.1155/2021/88950208895020Varied Age of First Presentation of Sickle Cell Disease: Case Presentations and ReviewAlexis Claeys0Susanne Van Steijn1Lydia Van Kesteren2Elizabet Damen3Machiel Van Den Akker4Pediatric Department, ZNA Paola Children’s Hospital, Antwerp, BelgiumPediatric Department, ZNA Paola Children’s Hospital, Antwerp, BelgiumPediatric Department, ZNA Paola Children’s Hospital, Antwerp, BelgiumPediatric Hematology, Oncology Unit, UZ Brussel, Jette, BelgiumPediatric Department, ZNA Paola Children’s Hospital, Antwerp, BelgiumSickle cell disease is a multisystem condition characterized by hemolytic anemia and vasoocclusion. Not only are the symptoms of the first presentation but also the ages of presentation are very variable. Following three case reports, different causes of possible late presentation are discussed. Many factors are responsible for the age at which sickle cell disease is diagnosed: doctor’s delay (unfamiliarity with the disease), patient’s delay (education and financial position of the parents, cultural factors), high- versus low-resource country (availability of newborn screening), fetal hemoglobin, reticulocyte count, and genetic modulators, such as SCD genotype, alpha-thalassemia, fetal hemoglobin concentration, and G6PD deficiency. The individual course of sickle cell disease depends on (epi) genetic and environmental properties and the underlying interactions. In further studies, the role of each factor should be evaluated more deeply, and its use as a marker of disease severity or activity should be assessed.http://dx.doi.org/10.1155/2021/8895020 |
| spellingShingle | Alexis Claeys Susanne Van Steijn Lydia Van Kesteren Elizabet Damen Machiel Van Den Akker Varied Age of First Presentation of Sickle Cell Disease: Case Presentations and Review Case Reports in Medicine |
| title | Varied Age of First Presentation of Sickle Cell Disease: Case Presentations and Review |
| title_full | Varied Age of First Presentation of Sickle Cell Disease: Case Presentations and Review |
| title_fullStr | Varied Age of First Presentation of Sickle Cell Disease: Case Presentations and Review |
| title_full_unstemmed | Varied Age of First Presentation of Sickle Cell Disease: Case Presentations and Review |
| title_short | Varied Age of First Presentation of Sickle Cell Disease: Case Presentations and Review |
| title_sort | varied age of first presentation of sickle cell disease case presentations and review |
| url | http://dx.doi.org/10.1155/2021/8895020 |
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