Management of Children With Sickle Cell Disease: A Comprehensive Review of the Literature

@article{Kavanagh2011ManagementOC,
  title={Management of Children With Sickle Cell Disease: A Comprehensive Review of the Literature},
  author={Patricia L. Kavanagh and Philippa G Sprinz and Samuel R. Vinci and Howard Bauchner and C. Jason Wang},
  journal={Pediatrics},
  year={2011},
  volume={128},
  pages={e1552 - e1574}
}
OBJECTIVE: Sickle cell disease (SCD) affects 70 000 to 100 000 people in the United States, and 2000 infants are born with the disease each year. The purpose of this study was to review the quality of the literature for preventive interventions and treatment of complications for children with SCD to facilitate the use of evidence-based medicine in clinical practice and identify areas in need of additional research. METHODS: We searched the Ovid Medline database and the Cochrane Library for… 

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References

SHOWING 1-10 OF 370 REFERENCES
Hydroxyurea for Sickle Cell Disease: A Systematic Review for Efficacy and Toxicity in Children
TLDR
Hydroxyurea reduces hospitalization and increases total and fetal hemoglobin levels in children with severe sickle cell anemia and there was inadequate evidence to assess the efficacy of hydroxyurea in other groups.
Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease.
Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of
Retrospective Review of Osteoarticular Infections in a Pediatric Sickle Cell Age Group
TLDR
This study suggests that an ill-appearing patient with a fever >38.2°C, pain, and swelling should prompt the physician to aspirate or biopsy the area and not rely on diagnostic studies that were found to be unreliable.
Hydroxyurea for sickle cell disease in children and for prevention of cerebrovascular events: the Belgian experience.
TLDR
The results confirm the benefit of HU, even in very young children, and its possible role in primary stroke prevention and its role in the prevention of cerebrovascular events.
Malignancy in patients with sickle cell disease
TLDR
The International Association of Sickle Cell Nurses and Physician Assistants identified 52 cases of cancer among 16,613 patients with SCD followed at 52 institutions, and three patients received hydroxyurea before the diagnosis of malignancy.
Contribution of sickle cell disease to the occurrence of developmental disabilities: A population-based study
TLDR
Children with SCD have increased risk for DD associated with stroke; thus, aggressive interventions are needed to prevent stroke in these children.
Septic complications after splenectomy for sickle cell sequestration crisis
TLDR
There was no increase in infections or episodes of sepsis in SCD patients who underwent splenectomy, although infections are common in children with SCD, and there were no operative deaths.
Liver involvement in white patients with sickle-cell disease.
TLDR
The data showed that liver involvement in sickle-cell disease is clinically irrelevant, reflecting the fact that the clinical expression of Sickle- cell disease in Sicilian patients is moderate.
Five years of experience with hydroxyurea in children and young adults with sickle cell disease.
TLDR
Long-term chronic treatment with HU for patients with SCD appears feasible, effective, and devoid of any major toxicity; in patients with a history of stroke, HU may be a valid alternative to chronic transfusion support.
Therapeutic challenges in childhood sickle cell disease Part 2: a problem‐orientated approach
TLDR
The major clinical problems arising in children with severesickle cell disease are stroke, recurrent acute chest syn-drome and frequent, debilitating vaso-occlusive crises and an evidence-based, problem-orientated approach to the current management of these problems in children is provided.
...
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