Effect of chronic transfusion therapy on progression of neurovascular pathology in pediatric patients with sickle cell anemia

2011 ◽  
Vol 47 (2) ◽  
pp. 125-128 ◽  
Author(s):  
Sarah Bishop ◽  
M. Gisele Matheus ◽  
Miguel R. Abboud ◽  
Ian D. Cane ◽  
Robert J. Adams ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pediatric Patients ◽  
Transfusion Therapy

Pial synangiosis for moyamoya syndrome in children with sickle cell anemia: a comprehensive review of reported cases

2014 ◽  
Vol 36 (1) ◽  
pp. E12 ◽  
Author(s):  
Benjamin C. Kennedy ◽  
Michael M. McDowell ◽  
Peter H. Yang ◽  
Caroline M. Wilson ◽  
Sida Li ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Brain Ischemia ◽  
Sickle Cell Anemia ◽  
Transcranial Doppler ◽  
Pediatric Patients ◽  
Significant Risk ◽  
Moyamoya Syndrome ◽  
Transfusion Therapy ◽  
Comprehensive Review

Object Pediatric patients with sickle cell anemia (SCA) carry a significant risk of developing moyamoya syndrome (MMS) and brain ischemia. The authors sought to review the safety and efficacy of pial synangiosis in the treatment of MMS in children with SCA by performing a comprehensive review of all previously reported cases in the literature. Methods The authors retrospectively reviewed the clinical and radiographic records in 17 pediatric patients with SCA treated at the Morgan Stanley Children's Hospital of New York (MSCHONY) who developed radiological evidence of MMS and underwent pial synangiosis between 1996 and 2012. The authors then added any additional reported cases of pial synangiosis for this population in the literature for a combined analysis of clinical and radiographic outcomes. Results The combined data consisted of 48 pial synangiosis procedures performed in 30 patients. Of these, 27 patients (90%) presented with seizure, stroke, or transient ischemic attack, whereas 3 (10%) were referred after transcranial Doppler screening. At the time of surgery, the median age was 12 years. Thirteen patients (43%) suffered an ischemic stroke while on chronic transfusion therapy. Long-term follow-up imaging (MR angiography or catheter angiography) at a mean of 25 months postoperatively was available in 39 (81%) treated hemispheres. In 34 (87%) of those hemispheres there were demonstrable collateral vessels on imaging. There were 4 neurological events in 1590 cumulative months of follow-up, or 1 event per 33 patient-years. In the patients in whom complete data were available (MSCHONY series, n = 17), the postoperative stroke rate was reduced more than 6-fold from the preoperative rate (p = 0.0003). Conclusions Pial synangiosis in patients with SCA, MMS, and brain ischemia appears to be a safe and effective treatment option. Transcranial Doppler and/or MRI screening in asymptomatic patients with SCA is recommended for the diagnosis of MMS.

Population Pharmacokinetic and Exposure–Response Analyses of Prasugrel in Pediatric Patients with Sickle Cell Anemia

2017 ◽  
Vol 57 (2) ◽  
pp. 243-254 ◽  
Author(s):  
Brian A. Moser ◽  
Elizabeth S. LaBell ◽  
Emmanuel Chigutsa ◽  
Joseph A. Jakubowski ◽  
David S. Small
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pediatric Patients ◽  
Population Pharmacokinetic ◽  
Exposure Response

Hepatic Iron Overload in Children With Sickle Cell Anemia on Chronic Transfusion Therapy

2009 ◽  
Vol 31 (5) ◽  
pp. 309-312 ◽  
Author(s):  
Kathy Brown ◽  
Charu Subramony ◽  
Warren May ◽  
Gail Megason ◽  
Hua Liu ◽  
...  
Keyword(s):  
Iron Overload ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Hepatic Iron ◽  
Transfusion Therapy ◽  
Hepatic Iron Overload

Unexpected Anemia and Reticulocytopenia in an Adolescent With Sickle Cell Anemia Receiving Chronic Transfusion Therapy

2015 ◽  
Vol 37 (7) ◽  
pp. e438-e440
Author(s):  
Emily R. Blauel ◽  
Lily T. Grossmann ◽  
Madhav Vissa ◽  
Scott T. Miller
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Transfusion Therapy

STUDIES OF CONGENITAL HEMOLYTIC SYNDROMES

PEDIATRICS ◽  
1958 ◽  
Vol 22 (5) ◽  
pp. 910-922
Author(s):  
Marion E. Erlandson ◽  
Irving Schulman ◽  
Gertrude Stern ◽  
Carl H. Smith
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Fetal Hemoglobin ◽  
Severe Form ◽  
Intermediate Form ◽  
Transfusion Therapy ◽  
Effective Synthesis ◽  
Cooley's Anemia ◽  
Compensation Index

Rates of destruction of erythrocytes and of effective production of erythrocytes and hemoglobin have been determined in 10 patients with homozygous Cooley's anemia. The method employed was based upon survival of Cr51-labeled cells in patients in whom a state of equilibrium of erythrocytes was present. While a marked hemohytic defect is present, this defect does not, by itself, determine the degree of anemia present. Rates of effective production of erythrocytes are increased above normal but are not increased to the same degree found in patients with other hemolytic diseases. Rates of effective synthesis of hemoglobin were found to be less than those obtained for production of erythrocytes. The rates of production of fetal hemoglobin in these patients are remarkably elevated but cannot be directly correlated with the rate of destruction of erythrocytes, rate of production of erythrocytes, or the degree of anemia present. The hemolytic defect in patients with intermediate Cooley's anemia was comparable to that in the majority of the patients with the severe form of disease. However, the most marked hemolytic defects were among patients with the severe and not with the intermediate form of disease. Production of erythrocytes and hemoglobin did not differ significantly in the two forms of this disease. Results in two splenectomized patients did not differ significantly from results in the non-splenectomized group of patients. However, since pre-splenectomy data were not available, no statement may be made as to possible individual benefit derived from the operation. The final status of each patient is determined by the particular balance obtained between rates of destruction and production. Neither production nor destruction alone determines the degree of anemia. The compensation index, as a measure of final status in each patient, was lowest in the severe form of Cooley's anemia. It is presumed to be lower still in many patients who could not be studied because transfusion therapy was in progress. The compensation index is somewhat higher in patients with intermediate Cooley's anemia and in two splenectomized individuals not requiring frequent transfusions. Values in these patients approach the higher levels found in patients with sickle cell anemia and congenital spherocytosis.

scholarly journals Myth: Blood transfusion is effective for sickle cell anemia—associated priapism

CJEM ◽  
2006 ◽  
Vol 8 (02) ◽  
pp. 119-122 ◽  
Author(s):  
Andrew L. Merritt ◽  
Christopher Haiman ◽  
Sean O. Henderson
Keyword(s):  
Blood Transfusion ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Conventional Therapy ◽  
Case Reports ◽  
Complete Information ◽  
Effective Therapy ◽  
Transfusion Therapy ◽  
Medline Search ◽  
Neurologic Sequelae

ABSTRACTObjective:Priapism is a recognized complication of sickle cell anemia (SCA). When initial conventional treatments fail, simple or exchange blood transfusion has been advocated as a secondary intervention. However, recent literature suggests this may not be an effective therapy and may have significant neurologic sequelae. This paper reviews and summarizes the effectiveness and risks of blood transfusion compared with conventional priapism therapy.Methods:All relevant papers identified from a MEDLINE search were systematically examined for data related to the use of blood transfusion in the setting of priapism due to SCA. The effectiveness of conventional therapy was compared with transfusion therapy using the outcome of “time to detumescence” (TTD). In addition, papers documenting adverse neurologic sequela were reviewed and summarized.Results:Forty-two case reports were identified containing complete information with regard to patient age and TTD. The mean TTD was 8.0 days with conventional therapy (n= 16) and 10.8 days with blood transfusion therapy (n= 26). Adverse neurologic sequelae from blood transfusion therapy was described in 9 cases, with long term outcomes ranging from complete resolution to severe residual deficits.Conclusion:The current literature does not support the contention that blood transfusion is an effective therapy in the treatment of priapism due to SCA, as defined by an acceleration of TTD. In fact, numerous reports suggest that serious neurologic sequelae may result from this treatment. We feel the routine use of this therapy cannot be recommended.

scholarly journals Evaluation of caries-associated virulence of biofilms from Candida albicans isolated from saliva of pediatric patients with sickle-cell anemia

2014 ◽  
Vol 22 (6) ◽  
pp. 484-489 ◽  
Author(s):  
Fernanda Lourenção BRIGHENTI ◽  
Amanda Coelho MEDEIROS ◽  
Bruno Mello MATOS ◽  
Zulene Eveline Abreu RIBEIRO ◽  
Cristiane Yumi KOGA-ITO
Keyword(s):  
Candida Albicans ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pediatric Patients

scholarly journals Hydroxyurea reduces cerebral metabolic stress in patients with sickle cell anemia

Blood ◽  
2019 ◽  
Vol 133 (22) ◽  
pp. 2436-2444 ◽  
Author(s):  
Melanie E. Fields ◽  
Kristin P. Guilliams ◽  
Dustin Ragan ◽  
Michael M. Binkley ◽  
Amy Mirro ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Metabolic Stress ◽  
Brain Magnetic Resonance Imaging ◽  
Border Zone ◽  
Oxygen Extraction Fraction ◽  
Transfusion Therapy ◽  
Whole Brain ◽  
Disease Modifying Therapy ◽  
Disease Modifying

Abstract Chronic transfusion therapy (CTT) prevents stroke in selected patients with sickle cell anemia (SCA). We have shown that CTT mitigates signatures of cerebral metabolic stress, reflected by elevated oxygen extraction fraction (OEF), which likely drives stroke risk reduction. The region of highest OEF falls within the border zone, where cerebral blood flow (CBF) nadirs; OEF in this region was reduced after CTT. The neuroprotective efficacy of hydroxyurea (HU) remains unclear. To test our hypothesis that patients receiving HU therapy have lower cerebral metabolic stress compared with patients not receiving disease-modifying therapy, we prospectively obtained brain magnetic resonance imaging scans with voxel-wise measurements of CBF and OEF in 84 participants with SCA who were grouped by therapy: no disease-modifying therapy, HU, or CTT. There was no difference in whole-brain CBF among the 3 cohorts (P = .148). However, whole-brain OEF was significantly different (P < .001): participants without disease-modifying therapy had the highest OEF (median 42.9% [interquartile range (IQR) 39.1%-49.1%]), followed by HU treatment (median 40.7% [IQR 34.9%-43.6%]), whereas CTT treatment had the lowest values (median 35.3% [IQR 32.2%-38.9%]). Moreover, the percentage of white matter at highest risk for ischemia, defined by OEF greater than 40% and 42.5%, was lower in the HU cohort compared with the untreated cohort (P = .025 and P = .034 respectively), but higher compared with the CTT cohort (P = .018 and P = .029 respectively). We conclude that HU may offer neuroprotection by mitigating cerebral metabolic stress in patients with SCA, but not to the same degree as CTT.

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