Managing sickle cell disease

Claster, Susan; Vichinsky, Elliott

doi:10.1136/bmj.327.7424.1151

Cited by 154 publications

(137 citation statements)

References 32 publications

(31 reference statements)

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“…The use of hydroxyurea has decreased the frequency of crises and hospitalization and increased the fetal hemoglobin level in some patients (6). At present, sickle cell anemia can be cured by bone marrow transplantation when there is a histocompatible donor (7). However, only a small fraction of patients in the United States have suitable donors (8).…”

mentioning

confidence: 99%

Correction of the sickle cell mutation in embryonic stem cells

Chang

Ye²,

Kan³

2006

Proc. Natl. Acad. Sci. U.S.A.

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Sickle cell anemia is one of the most common genetic diseases worldwide. Patients often suffer from anemia, painful crises, infections, strokes, and cardiopulmonary complications. Although current management has improved the quality of life and survival of patients, cure can be achieved only with bone marrow transplantation when histocompatible donors are available. The ES cell technology suggests that a therapeutic cloning approach may be feasible for treatment of this disease. Using a transgenic͞knockout sickle cell anemia mouse model, which harbors 240 kb of human DNA sequences containing the ␤ S -globin gene, we prepared ES cells from blastocysts that had the sickle cells anemia genotype and carried out homologous recombination with DNA constructs that contained the ␤ A -globin gene. We obtained ES cells in which the ␤ S was corrected to the ␤ A sequence. Hematopoietic cells differentiated from these ES cells produced both hemoglobin A and hemoglobin S. This approach can be applied to human ES cells to correct the sickle mutation as well as ␤-thalassemia mutations.correction of mutation ͉ homologous recombination ͉ sickle cell anemia ͉ hematopoietic differentiation ͉ ␤-thalassemia

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mentioning

confidence: 99%

Correction of the sickle cell mutation in embryonic stem cells

Chang

Ye²,

Kan³

2006

Proc. Natl. Acad. Sci. U.S.A.

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“…37 When started early it has significantly reduced the incidence of bacteremia by pneumococcus. 31,21 Some studies report 84% reduction is sepsis by pneumococcus in children given the correct prophylaxis with penicillin. 39 Recommendations are to start at 4 months or as soon as a diagnosis of sickle-cell anemia is made, with maintenance until 4 years 3,13,34 (some authors suggest continuing until 5 years 37,40 ).…”

Section: Antipneumococcalmentioning

confidence: 99%

“…Acute chest syndrome is the second most common cause of hospital admissions for all age groups of individuals with sickle-cell anemia. 21 In acute chest syndrome, the use of antibiotics does not generally trigger a rapid improvement and progress is often unfavorable. 3 …”

Section: Airwaysmentioning

confidence: 99%

“…Laboratory tests may reveal leukocytosis (above 15,000/mm 3 ) and increased erythrocyte sedimentation rate. 24 Differential diagnosis between osteomyelitis and bone infarction is difficult, 21 since their clinical signs are similar. High fever with shivering and an appearance of intoxication increase the suspicion of osteomyelitis, and vigilance must be maintained in such cases.…”

Section: Osteo-articular Systemmentioning

confidence: 99%

“…20 Infections caused by atypical bacteria may occur at any age. 21 Secondary infection may occur in areas of the pulmonary parenchyma that have suffered infarction in acute chest syndrome, 22 which presents with clinical manifestations similar to pneumonia, such as fever, coughing, dyspnea and chest pain. Acute chest syndrome is the second most common cause of hospital admissions for all age groups of individuals with sickle-cell anemia.…”

Section: Airwaysmentioning

confidence: 99%

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Anemia falciforme e infecções

Nuzzo¹,

Fonseca²

2004

J. Pediatr. (Rio J.)

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Objective: To discuss the high prevalence of sickle cell disease in our environment and the increased morbidity and mortality as a result of infection associated with this condition. Sources of data:Review of MEDLINE from 1986 to 2003. We found around 600 references about the subject. Thirty-five journal articles were reviewed, in addition to chapters in two text books. Summary of the findings:We discuss general information concerning sickle cell disease as well as a few topics about the most frequently observed infections in these patients. Drug prophylaxis and immunizations are also covered.Conclusions: This review hopes to provide the pediatric community with information concerning the association between sickle cell disease and infections, so as to minimize the occurrence of complications.

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Sickle Cell Disease

2012

Handbook of Pediatric Hematology and Oncology

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Managing sickle cell disease

Cited by 154 publications

References 32 publications

Correction of the sickle cell mutation in embryonic stem cells

Correction of the sickle cell mutation in embryonic stem cells

Anemia falciforme e infecções

Sickle Cell Disease

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