Pathophysiology and treatment of stroke in sickle-cell disease: present and future

Switzer, Jeffrey A.; Hess, David C.; Nichols, Fenwick T.; Adams, Robert J.

doi:10.1016/s1474-4422(06)70469-0

Cited by 246 publications

(192 citation statements)

References 139 publications

Supporting

Mentioning

173

Contrasting

Unclassified

Order By: Relevance

“…61,66 SCD-associated morbidity and mortality in young adults is largely due to as yet unpreventable complications such as priapism, avascular necrosis, chronic pulmonary impairment, hypertension, stroke and recurrent venoocclusive crises. 58,[67][68][69][70] The only curative approach for SCD is HSCT. Historically, the indication for HSCT in SCD was mainly based on SCD-associated morbidity: the sicker the child, the stronger the indication.…”

Section: Sickle Cell Diseasementioning

confidence: 99%

Hematopoietic stem cell transplantation in thalassemia major and sickle cell disease: indications and management recommendations from an international expert panel

et al. 2014

View full text Add to dashboard Cite

Section: Sickle Cell Diseasementioning

confidence: 99%

Hematopoietic stem cell transplantation in thalassemia major and sickle cell disease: indications and management recommendations from an international expert panel

et al. 2014

View full text Add to dashboard Cite

“…5,26 Similar to MRA, TCD reveals largevessel disease whereas the mechanism of silent infarction in SCD seems to be essentially microvascular. 27 The benefit of transfusion in the prevention of silent cerebral infarction in children with SCD seems to depend on the patient's cerebrovascular status. 28 Analysis of the STOP trial revealed that transfusion therapy lowers the risk Figure 1.…”

Section: Discussionmentioning

confidence: 99%

Discontinuing prophylactic transfusions increases the risk of silent brain infarction in children with sickle cell disease: data from STOP II

Abboud

Yim

Musallam

et al. 2011

Blood

View full text Add to dashboard Cite

In the STOP II trial, discontinuation of prophylactic transfusions in high risk children with sickle cell disease (SCD) resulted in a high rate of reversion to abnormal blood-flow velocities on transcranial Doppler (TCD) ultrasonography and strokes. We analyzed data from STOP II to determine the effect of discontinuing transfusions on the development or progression of silent brain infarcts on magnetic resonance imaging (MRI). At study entry, 21 of 79 (27%) patients had evidence of silent infarcts. There were no statistically significant differences in baseline characteristics between patients with normal brain MRI or silent infarcts at study entry. At study end, 3 of 37 (8.1%) patients in the continued-transfusion group developed new brain MRI lesions compared with 11 of 40 (27.5%) in the transfusion-halted group (P ‫؍‬ .03). The total number of lesions remained essentially unchanged decreasing from 25 to 24 in the continuedtransfusion group while increasing from 27 to 45 in transfusion-halted patients. Thus, discontinuation of transfusions in children with SCD and abnormal TCD who revert to low-risk increases the risk of silent brain infarction. Together with data from STOP, these findings demonstrate that transfusions prevent the development of silent infarcts in patients with SCD and abnormal TCD but normal MRA. (Blood. 2011;118(4):894-898) IntroductionEstimates of the prevalence of silent brain infarcts, in children with sickle cell disease (SCD) range from 17% to 35%. [1][2][3][4] Despite the terminology, 'silent infarcts' are clinically significant given their association with subsequent overt stroke and neurocognitive deficits in school-aged children. 2,[5][6][7][8][9] Better understanding of the associated risk factors and efforts to optimize prevention remain essential. Reported risk factors for silent infarction are raised white blood-cell count, anemia, severe vasculopathy, and the SEN ␤-globin haplotype. 4,[10][11] Transfusions have been successfully used to prevent initial or recurrent strokes in SCD. [12][13][14][15][16] To prevent first strokes, the Stroke Prevention Trial in Sickle Cell Anemia (STOP) used prophylactic red-cell transfusions in children who were identified by transcranial Doppler (TCD) ultrasonography as being at high risk for stroke. This strategy reduced the incidence of stroke among such children from 10% per year to Ͻ 1% per year. 15 The STOP study led to recommendations for TCD screening and prophylactic transfusion for children with abnormal velocities on ultrasonography. 17 Among patients with MRI evidence of silent infarction and raised velocities by TCD enrolled in STOP, transfusion also diminished the risk of new silent infarcts. 18 Despite the reduced risk of stroke, the potentially indefinite duration of transfusion aroused concern about adverse effects, especially iron overload. In the Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP II) Trial, discontinuation of transfusions in patients whose TCD velocities had normalized (normal TCD) resulted in a hig...

show abstract

“…Sickle cell disease (SCD) is a genetic disorder caused by the substitution of valine for glutamic acid at the sixth position of the amino acid beta chain of the haem molecule 1 and characterized by the possession of sickle cell hemoglobin 2 . Hence, sickle cell hemoglobin is a type of hemoglobin occurring in the erythrocytes of sickle cell disease patients 3 .…”

Section: Asian Journal Of Medical Sciences4(2013)mentioning

confidence: 99%

In vitro effect of Allopurinol on sickling rate and uric acid level in sickle cell erythrocyte.

Johnkennedy

2013

Asian J Med Sci

View full text Add to dashboard Cite

Objective: Allopurinol is a drug used for the prevention of gout. The in vitro effect of allopurinol on human plasma and erythrocyte of sickle cell haemoglobin(HbSS) were investigated on uric acid level and sickling rate respectively. Methods:The determination was carried out on samples from 9-25years attending General Hospital Owerri. The plasma uric acid level and sickling rate were determined prior to and after incubation with 100mg standard dose of allopurinol. Results:The plasma uric acid level was significantly decreased (p<0.05) when compared with the control while sickling rate was significantly increased when compared with the control (p <0.05).The results obtained demonstrated that allopurinol could hasten sickling rate and decrease uric acid level. Conclusion:These observations suggest that allopurinol concentration in the circulation might induce marked increased in erythrocyte sickling in vivo and reduced the antioxidant ability of uric acid level in sickle cell disease. Consequently, slow recovery from a sickling crisis. This may be detrimental in the management of sickle cell disease

show abstract

Pathophysiology and treatment of stroke in sickle-cell disease: present and future

Cited by 246 publications

References 139 publications

Hematopoietic stem cell transplantation in thalassemia major and sickle cell disease: indications and management recommendations from an international expert panel

Hematopoietic stem cell transplantation in thalassemia major and sickle cell disease: indications and management recommendations from an international expert panel

Discontinuing prophylactic transfusions increases the risk of silent brain infarction in children with sickle cell disease: data from STOP II

In vitro effect of Allopurinol on sickling rate and uric acid level in sickle cell erythrocyte.

Contact Info

Product

Resources

About