Factors associated with abnormal cerebral blood flow in Egyptian children with sickle cell disease

Background: Transcranial Doppler (TCD) is a well-established tool for cerebrovascular assessment. Estimating the flow velocity across the intracranial arteries helps to identify children with sickle cell anaemia who are at risk for stroke. Objective: Our aim is to correlate TCD findings with clinical condition in children with sickle cell disease (SCD) to determine the value of TCD assessment as a predictive tool for stroke in SCD and to identify any association of TCD findings with disease severity, transfusion therapy and treatment administered. Methods: Eighty-five paediatric SCD patients aged from 3 years to 18 years of both genders who were followed up at the Hematology Clinic of New Children’s Hospital at Cairo University were included in this cross-sectional observational study. All our participants underwent routine laboratory investigations and TCD assessments. Results: Oof the 85 patients, two patients (2.3%) died before completing the TCD study and eventually 83 patients were included in the analysis. Seventeen (20.5%) patients had abnormal TCD findings, seven (8.4%) patients showed high-risk findings and 10 (12.1%) patients had conditional flow pattern. Logistic linear regression analysis confirmed that annual frequency of blood transfusion and hydroxyurea (HU) dose were associated with a decreased risk of abnormal TCD findings. Conclusion: The current study demonstrates that our TCD data reproduce the findings of other studies and that it is very likely the results from large trials are applicable for Egyptian children. The annual frequency of blood transfusion and HU dose were associated with a decreased frequency of abnormal TCD findings.

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Abstract TP402: Sickle Cell Disease in Pediatric Stroke - Inpatient Resource Utilization in the Decade Following STOP

Stroke ◽

10.1161/str.48.suppl_1.tp402 ◽

2017 ◽

Vol 48 (suppl_1) ◽

Author(s):

J. Michael Taylor ◽

Paul Horn ◽

Heidi Sucharew ◽

Todd A Abruzzo ◽

Jane Khoury

Keyword(s):

Sickle Cell Disease ◽

Blood Transfusion ◽

Sickle Cell ◽

Care Utilization ◽

Cell Disease ◽

Common Procedure ◽

Transfusion Therapy ◽

Stroke Population ◽

Clinical Classification Software ◽

Inpatient Database

Background: Sickle cell disease (SCD) is an important risk factor for stroke in children. Natural history studies demonstrate that greater than 10% of hemoglobin SS patients suffered ischemic stroke prior to age 20 years. In 1998, the Stroke Prevention Trial in Sickle Cell Anemia (STOP) successfully demonstrated the role for routine transfusion therapy in reducing stroke in at risk SCD patients. Fullerton and colleagues then found that first time stroke in SCD decreased in Californian children in the 2 years following STOP. We investigated the stroke rate and health care utilization of children with SCD for two calendar years in the decade following publication of the STOP trial using a national inpatient database. Methods: The 2000 and 2009 Kids’ Inpatient Database (KID) were used for analysis. SCD and stroke cases were identified by ICD-9 codes 282.6x, 430, 431, 432.9, 434.X1, 434.9, 435.9. We queried the KID procedural clinical classification software for utilization of services pertinent to SCD and stroke; transfusion, MRI, and cerebral angio. Results: In 2000, SCD was a discharge diagnosis in 34,294 children and 158 (0.46%) children had SCD and stroke. By 2009, discharges with SCD rose to 37,082 children with 212 (0.57%) children carrying both diagnoses. In 2000 and 2009, AIS is the most common stroke type at 83%, males account for 53% of stroke and black race was reported by 92% of SCD and stroke subjects. Procedure utilization is higher in the SCD and stroke population than in SCD without stroke (Figure 1). Blood transfusion is the most common procedure in both study years, significantly higher in stroke subjects. Conclusion: For pediatric inpatients with SCD, blood transfusion and diagnostic cerebrovascular procedures were significantly more common in the cohort with comorbid stroke. In the decade after STOP, children hospitalized with SCD and stroke represented less than 0.6% of the total inpatient SCD population.

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Retinal microvascular analysis using Fundus Fluorescein Angiography in Egyptian sickle cell disease patients

QJM ◽

10.1093/qjmed/hcab113.067 ◽

2021 ◽

Vol 114 (Supplement_1) ◽

Author(s):

Nayera H El Sherif ◽

Mahmoud A Kenny ◽

Waheed S Elhalfawy

Keyword(s):

Sickle Cell Disease ◽

Blood Transfusion ◽

Fluorescein Angiography ◽

Sickle Cell ◽

Large Sample Size ◽

Cell Disease ◽

Fundus Fluorescein Angiography ◽

Transfusion Therapy ◽

Hydroxyurea Treatment ◽

Trans Cranial Doppler

Abstract Background Sickle cell disease can affect retina of eye via vaso-occulsive changes that occur in micro-vessels of retina which could be analysed by using Fundus Fluorescein Angiography. Aim To analyze macular microvascular alternation in patients with SCD by Fundus Fluorescein Angiography (FFA) and to assess the role of potentially contributory Clinico-pathological factors including Trans-Cranial Doppler, genotypes, hydroxyurea, transfusion therapy and finally iron overload state on the development of macular alterations. Method This was across-sectional study which included 30 Sickle cell disease patients randomly recruited from the Paediatric Haematology clinic, children Hospital, Ain Shams University, Cairo, Egypt. Complete blood count (CBC), Trans-Cranial Doppler (TCD) and Fundus Fluorescein Angiography. Results In our study, there were 30 patients with mean age (14.1± 4.02), 5 patients had abnormal/conditional Trans-Cranial, 15 patients had Vaso-occlusive crises, 11 patients were on regular simple blood transfusion; all 30 studied sickle cell disease patients had normal Fundus Fluorescein Angiography and eye examination and only one patient hadabnormal visual acuity;A 29 years oldgirl who had five attacks of cerebral strokes last year, on regular simple blood transfusion and Hydroxyurea treatment with abnormal TCD and recurrent Vaso-occlusive crises in last two years, Although her vision is hand movement yet Fundus Fluorescein Angiography was normal. Conclusion we didn’t find any Retinal microvascular alternation in our studied SCD patients using Fundus Fluorescein Angiography, we related our results to the fact that our studied SCD patients were young and all our studied patients were on hydroxyurea therapy with fair compliance, further studies using large sample size are warranted in order to illustrate the utility of Fundus Fluorescein Angiography (FFA) as a tool for better detection of sickle retinopathy.

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Automated exchange compared to manual and simple blood transfusion attenuates rise in ferritin level after 1 year of regular blood transfusion therapy in chronically transfused children with sickle cell disease

Transfusion ◽

10.1111/trf.15982 ◽

2020 ◽

Vol 60 (11) ◽

pp. 2508-2516

Author(s):

Shannon Kelly ◽

Mark Rodeghier ◽

Michael R. DeBaun

Keyword(s):

Sickle Cell Disease ◽

Blood Transfusion ◽

Sickle Cell ◽

Ferritin Level ◽

Cell Disease ◽

Transfusion Therapy

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How I treat and manage strokes in sickle cell disease

Blood ◽

10.1182/blood-2014-09-551564 ◽

2015 ◽

Vol 125 (22) ◽

pp. 3401-3410 ◽

Cited By ~ 64

Author(s):

Adetola A. Kassim ◽

Najibah A. Galadanci ◽

Sumit Pruthi ◽

Michael R. DeBaun

Keyword(s):

Sickle Cell Disease ◽

Blood Transfusion ◽

Sickle Cell ◽

Exchange Transfusion ◽

Management Strategies ◽

Cell Disease ◽

Transfusion Therapy ◽

Hematopoietic Stem ◽

Neuro Imaging

Abstract Neurologic complications are a major cause of morbidity and mortality in sickle cell disease (SCD). In children with sickle cell anemia, routine use of transcranial Doppler screening, coupled with regular blood transfusion therapy, has decreased the prevalence of overt stroke from ∼11% to 1%. Limited evidence is available to guide acute and chronic management of individuals with SCD and strokes. Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Initial management of a focal neurologic deficit includes evaluation by a multidisciplinary team (a hematologist, neurologist, neuroradiologist, and transfusion medicine specialist); prompt neuro-imaging and an initial blood transfusion (simple followed immediately by an exchange transfusion or only exchange transfusion) is recommended if the hemoglobin is >4 gm/dL and <10 gm/dL. Standard therapy for secondary prevention of strokes and silent cerebral infarcts includes regular blood transfusion therapy and in selected cases, hematopoietic stem cell transplantation. A critical component of the medical care following an infarct is cognitive and physical rehabilitation. We will discuss our strategy of acute and long-term management of strokes in SCD.

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Chronic Blood Transfusion Therapy Practices to Treat Strokes in Children with Sickle Cell Disease

Journal of the American Academy of Nurse Practitioners ◽

10.1111/j.1745-7599.2005.0046.x ◽

2005 ◽

Vol 17 (7) ◽

pp. 277-282 ◽

Cited By ~ 8

Author(s):

Terianne Lindsey ◽

Nutrena Watts-Tate ◽

Elaine Southwood ◽

Julie Routhieaux ◽

Janice Beatty ◽

...

Keyword(s):

Sickle Cell Disease ◽

Blood Transfusion ◽

Sickle Cell ◽

Cell Disease ◽

Transfusion Therapy

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Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease

Blood ◽

10.1182/blood-2010-01-261123 ◽

2011 ◽

Vol 117 (3) ◽

pp. 772-779 ◽

Cited By ~ 154

Author(s):

Monica L. Hulbert ◽

Robert C. McKinstry ◽

JoAnne L. Lacey ◽

Christopher J. Moran ◽

Julie A. Panepinto ◽

...

Keyword(s):

Magnetic Resonance ◽

Sickle Cell Disease ◽

Blood Transfusion ◽

Sickle Cell ◽

Stroke Prevention ◽

Secondary Stroke Prevention ◽

Cell Disease ◽

Cerebral Infarcts ◽

Eligibility Criteria ◽

Transfusion Therapy

Abstract Children with sickle cell disease (SCD) and strokes receive blood transfusion therapy for secondary stroke prevention; despite this, approximately 20% experience second overt strokes. Given this rate of second overt strokes and the clinical significance of silent cerebral infarcts, we tested the hypothesis that silent cerebral infarcts occur among children with SCD being transfused for secondary stroke prevention. A prospective cohort enrolled children with SCD and overt strokes at 7 academic centers. Magnetic resonance imaging and magnetic resonance angiography of the brain were scheduled approximately every 1 to 2 years; studies were reviewed by a panel of neuroradiologists. Eligibility criteria included regularly scheduled blood transfusion therapy. Forty children were included; mean pretransfusion hemoglobin S concentration was 29%. Progressive cerebral infarcts occurred in 45% (18 of 40 children) while receiving chronic blood transfusion therapy; 7 had second overt strokes and 11 had new silent cerebral infarcts. Worsening cerebral vasculopathy was associated with new cerebral infarction (overt or silent; relative risk = 12.7; 95% confidence interval, 2.65-60.5, P = .001). Children with SCD and overt strokes receiving regular blood transfusion therapy experience silent cerebral infarcts at a higher rate than previously recognized. Additional therapies are needed for secondary stroke prevention in children with SCD.

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Anti-Erythrocyte Allo-Immunization to Sickle Cell Disease Patients Followed in Transfusion Therapy Unit of the National Blood Transfusion Center of Abidjan Côte D’Ivoire

International Journal of Immunology ◽

10.11648/j.iji.20170501.11 ◽

2017 ◽

Vol 5 (1) ◽

pp. 1 ◽

Cited By ~ 2

Author(s):

Sekongo Y. M.

Keyword(s):

Sickle Cell Disease ◽

Blood Transfusion ◽

Sickle Cell ◽

Cote D'ivoire ◽

Côte D’Ivoire ◽

Cell Disease ◽

Transfusion Therapy ◽

Cote D’Ivoire ◽

Transfusion Center ◽

Blood Transfusion Center

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The prevalence of human immunodeficiency and of hepatitis B viral infections is not increased in patients with sickle cell disease in Tanzania

BMC Infectious Diseases ◽

10.1186/s12879-021-06726-z ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Grace Shayo ◽

Irene Makundi ◽

Lucio Luzzatto

Keyword(s):

Sickle Cell Disease ◽

Blood Transfusion ◽

Hepatitis B ◽

Sickle Cell ◽

Viral Infections ◽

Cross Sectional Study ◽

Dar Es Salaam ◽

Categorical Variables ◽

Cell Disease ◽

Cross Sectional

Abstract Background Tanzania ranks as the fourth country in the world with respect to the number of sickle cell disease (SCD) births; it is also endemic to the human immunodeficiency virus (HIV) and the hepatitis B virus (HBV). This study was done to determine the prevalence of HIV and HBV infections among SCD patients in Dar es Salaam, Tanzania. Methods A multicenter hospital-based descriptive cross sectional study was carried out among participants aged ≥ 16 years with a proven diagnosis of SCD. Socio-demographic and clinical data were recorded. Blood samples were drawn for HIV and HBV diagnosis. All categorical variables were summarized into frequencies. Results There were 185/325 (56.9 %) females. The mean age (SD) was 23.0 ± 7.5 years. The prevalence of HIV was 1.8 %; the prevalence of HBV was 1.2 %. Conclusions The prevalence of both HIV and HBV in SCD patients is no greater than in the general population of Dar es Salaam or Tanzania. For associations, a large study would be needed. From a detailed blood transfusion history of SCD patients we found no evidence that HIV or HBV infection was transmitted through blood transfusion.

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Alloimmunization in Egyptian Children with Sickle Cell Disease

QJM ◽

10.1093/qjmed/hcab113.023 ◽

2021 ◽

Vol 114 (Supplement_1) ◽

Author(s):

Mohsen Saleh Elalfy ◽

Mahmoud Adel Kenny ◽

Fatima Zahrra Abdelkarim Saed ◽

Fatma Soliman Elsayed Ebeid

Keyword(s):

Sickle Cell Disease ◽

Blood Transfusion ◽

Sickle Cell ◽

Complete Blood Count ◽

Blood Group Antigens ◽

Cell Disease ◽

Antibody Screening ◽

Egyptian Children ◽

Significant Difference ◽

Detailed Medical History

Abstract Background Transfusion is critical in the management of sickle cell disease (SCD) complications. The resultant alloimmunization to RBC group antigens is a major complication of allogeneic blood transfusion and generally presents significant challenges in the management of SCD patients. Aim To measure the frequency of the occurrence of the alloimmune markers in sickle cell disease and to investigate its predicators. Subjects and methods This cross-sectional study included 50 children and young adults with SCD, all patients were subjected to detailed medical history thorough clinical examination. Laboratory investigations included complete blood count, markers of hemolysis and serum ferritin. The D, C, c, E, e, M, NJKa and JKb antigens were typed using monoclonal antisera. Fya, Fyb, Jka, Jkb, S, s and Anti Lua antigens were typed by commercially prepared polyclonal anti- human sera. Antibody screening was performed using Ortho screening panel. Results The study Sickle SS was the most common type of SCD representing more than half of the recruited patients. Frequency of antibody positive screening among SCD was 16%. Comparison between SCD patients with positive and those with negative antibody screening showed that higher frequency of positive antibody screening in those with SB+ than those SS and SB0. Patient with positive antibodies screening were significantly older when they received their first blood transfusion and had lower transfusion index (p = 0.037) than those with negative antibodies screening (p=0.013). There were no significant difference between patients with positive and those with negative antibodies screening as regards age, gender, transfusion frequency and as regards comorbidities; painful crisis (p=0.117), stroke (p=0.398), ACS (p=0.363). Conclusion The distribution of the blood group antigens in patients with SCD has important clinical significance especially those who often require regular blood transfusion and who may have developed multiple antibodies. The resultant alloimmunization is serious necessitates early recognition and management.

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