scholarly journals Epidemiology of Stroke in Sickle Cell Disease

2021 ◽  
Vol 10 (18) ◽  
pp. 4232
Author(s):  
Fenella Jane Kirkham ◽  
Ikeoluwa A. Lagunju
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Maximum Velocity ◽  
Optimal Dose ◽  
Haemorrhagic Stroke ◽  
Quantitative Mri ◽  
Cell Disease ◽  
Neurological Conditions ◽  
Magnetic Resonance Imaging Mri ◽  
Penicillin Prophylaxis

Sickle cell disease is the most common cause of stroke in childhood, both ischaemic and haemorrhagic, and it also affects adults with the condition. Without any screening or preventative treatment, the incidence appears to fall within the range 0.5 to 0.9 per 100 patient years of observation. Newborn screening with Penicillin prophylaxis and vaccination leading to reduced bacterial infection may have reduced the incidence, alongside increasing hydroxyurea prescription. Transcranial Doppler screening and prophylactic chronic transfusion for at least an initial year has reduced the incidence of stroke by up to 10-fold in children with time averaged mean of the maximum velocity >200 cm/s. Hydroxyurea also appears to reduce the incidence of first stroke to a similar extent in the same group but the optimal dose remains controversial. The prevention of haemorrhagic stroke at all ages and ischaemic stroke in adults has not yet received the same degree of attention. Although there are fewer studies, silent cerebral infarction on magnetic resonance imaging (MRI), and other neurological conditions, including headache, epilepsy and cognitive dysfunction, are also more prevalent in sickle cell disease compared with age matched controls. Clinical, neuropsychological and quantitative MRI screening may prove useful for understanding epidemiology and aetiology.

Quantitative MRI of the brain in children with sickle cell disease reveals abnormalities unseen by conventional MRI

1998 ◽  
Vol 8 (3) ◽  
pp. 535-543 ◽  
Author(s):  
R. Grant Steen ◽  
Wilburn E. Reddick ◽  
Raymond K. Mulhern ◽  
James W. Langston ◽  
Robert J. Ogg ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Quantitative Mri ◽  
Cell Disease ◽  
Conventional Mri ◽  
The Brain

scholarly journals Quantitative MRI Analysis of Craniofacial Bone Marrow in Patients with Sickle Cell Disease

2012 ◽  
Vol 34 (3) ◽  
pp. 622-627 ◽  
Author(s):  
E.J. Elias ◽  
J.H. Liao ◽  
H. Jara ◽  
M. Watanabe ◽  
R.N. Nadgir ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Quantitative Mri ◽  
Cell Disease ◽  
Craniofacial Bone

scholarly journals Penicillin prophylaxis in children with sickle cell disease in Brent.

BMJ ◽  
1991 ◽  
Vol 302 (6783) ◽  
pp. 989-990 ◽  
Author(s):  
D Cummins ◽  
R Heuschkel ◽  
S C Davies
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Penicillin Prophylaxis

scholarly journals Quantitative MRI analysis of salivary glands in sickle cell disease

2012 ◽  
Vol 41 (8) ◽  
pp. 630-636 ◽  
Author(s):  
J Liao ◽  
N Saito ◽  
A Ozonoff ◽  
H Jara ◽  
M Steinberg ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Salivary Glands ◽  
Sickle Cell ◽  
Quantitative Mri ◽  
Cell Disease

scholarly journals Prevalence of Severe Bacterial Infection in Febrile Children with Sickle CELL Disease in the Era of Pneumococcal Conjugate Vaccine 13

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3665-3665
Author(s):  
Gloria Contreras-Yametti ◽  
Custodio Haidee ◽  
Hamayun Imran
Keyword(s):  
Sickle Cell Disease ◽  
Conjugate Vaccine ◽  
Sickle Cell ◽  
Pneumococcal Conjugate Vaccine ◽  
Univariate Analysis ◽  
Cell Disease ◽  
Pneumococcal Infections ◽  
Patient Disposition ◽  
Penicillin Prophylaxis ◽  
Febrile Events

Abstract Introduction The incidence of invasive pneumococcal infections in patients with sickle cell disease (SCD) decreased after introduction of penicillin prophylaxis and pneumococcal conjugate vaccine (PCV). However, the decrease in pneumococcal infections alone may not necessarily mean an overall decrease in severe bacterial infections (SBI). In a previous publication, we reported a 0.4 % prevalence of pneumococcal bacteremia following introduction of PCV 13. In the current study, we aimed to define the prevalence of SBI and hospitalization in febrile patients in the same cohort in the later years. Methods We performed a retrospective study of patients with SCD <18 years old presenting with fever to University of South Alabama Children's and Women's Hospital from January 2014 to June 2017. SBI was defined as: bacteremia, pneumonia, pyelonephritis, meningitis, osteomyelitis and abscess (superficial and deep). Univariate analysis and multivariate logistic regression were used to determine factors associated with patient disposition as well as presence of SBI. Results There were 258 febrile events in 120 patients resulting in 187 (72%) admissions (figure 1). SBI was seen in 12% of admissions with uncomplicated community acquired pneumonia being the most common. The prevalence of bacteremia was 1.6% with single cases of pneumococcus, E. coli, and H. influenzae bacteremia. Younger age, high fever, and splenectomy were associated with hospitalization (p<0.05). However, only C reactive protein was associated with SBI (p<0.02). Viral infection was diagnosed in 80% of outpatients but 87% were given antibiotics. Among inpatients, all received parenteral antibiotics, and 67% were assessed to have viral illness, although only 23% had a virus identified. Pneumococcal vaccination status was satisfactory in 77% of our sample while compliance rate with penicillin prophylaxis was >85% in both inpatient and outpatient groups. Conclusion Although majority of febrile events were due to viral infections, 3 of four febrile episodes in our cohort resulted in hospitalization. A small proportion of patients had SBI and a much smaller proportion had bacteremia. These findings support early virus identification which can have implications on patient discharge disposition and antibiotic use. Further studies looking at risk stratification of febrile patients with SCD are needed to encourage outpatient management without compromising safety. Figure 1. Figure 1. Disclosures Imran: Novo Nordask: Speakers Bureau.

scholarly journals Outcomes of Splenectomy in Children with Sickle Cell Disease

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1382-1382
Author(s):  
Peter B. Soh ◽  
Abdul H. Siddiqui
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Doppler Ultrasonography ◽  
Beta Thalassemia ◽  
Blood Transfusions ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Operative Complications ◽  
The Mean ◽  
Penicillin Prophylaxis

Abstract Background Children with splenic sequestration, related to sickle cell disease, are managed acutely with blood transfusions followed by an elective splenectomy to prevent recurrent episodes. The objective of this study was to review the outcomes of splenectomy in children with sickle cell disease as a single center experience. Methods A retrospective chart review of children with sickle cell disease who had splenectomy between 1999 and 2014 at the University of South Alabama was performed. Data on demographics, sequestration episodes, post-operative complications, bacteremia, transcranial doppler ultrasonography and death were collected. Results A total of 52 patients (36 with Hemoglobin SS, 7 with Hemoglobin SC, 5 with S-Beta Thalassemia Plus, and 4 with S-Beta Thalassemia Zero) received splenectomy during the study period. Mean age at first splenic sequestration event was 39 months. The mean age of splenectomy was 5 years (Minimum: 18 months; Maximum: 18 years). There were 24 males and 28 females. Over 95 percent of patients were on penicillin prophylaxis. In only 73 percent of patients, proof of completed vaccination including pneumococcal polysaccharide, pneumococcal conjugate and meningococcal conjugate vaccines, could be found. Average post-splenectomy follow-up was 7.4 years. The post-operative complications included fever in 4 patients, acute chest syndrome in 4 patients, lobar pneumonia in 2 patients, pleural effusion in 1 patient, atelectasis on chest radiograph in 2 patients and surgical wound abscess in 1 patient. One patient had an intra-abdominal bleed which required reoperation. The average number of hospitalizations for vaso-occlusive pain crises was 3.3 per year prior to splenectomy and 2.2 per year during the 2 years following splenectomy (p=0.04). Mean platelet count before splenectomy was 267/m3 compared to 533/m3 at 1 year after splenectomy (p<0.05). Differences in mean white blood cell and reticulocyte counts before and after splenectomy were not statistically significant. Only 2 of the patients had culture proven bacteremia, but both of them occurred prior to their splenectomy. One of the patients grew Staphylococcus hominis and the other grew Staphylococcus lugdunensis. No true bacteremia were reported in patients after splenectomy. None of the patients developed stroke while four (~8%) patients developed critical transcranial doppler ultrasonography velocities (≥200 cm/s) and were started on chronic blood transfusions. The mean time-average maximum velocity before splenectomy was 127 cm/sec and increased to 151 cm/sec at 2 years after splenectomy (p=0.002). Among the splenectomized patients, 18 (35%) of them had been started on Hydroxyurea. Discussion Our results indicate that with proper vaccination and penicillin prophylaxis, the risk of infection after splenectomy can be controlled. The mean hemoglobin levels did not change after splenectomy but our patients had fewer hospitalizations for pain crises after splenectomy. The cerebral blood flow velocity increased after splenectomy. This might imply that more patients will require chronic blood transfusions for stroke prevention after splenectomy. We conclude that splenectomy is a safe and effective modality for management of life threatening splenic sequestrations in children with sickle cell disease. Disclosures No relevant conflicts of interest to declare.

Penicillin Prophylaxis in Children with Sickle Cell Disease

2010 ◽  
Vol 15 (3) ◽  
pp. 152-159 ◽  
Author(s):  
Mary Petrea Cober ◽  
Stephanie J. Phelps
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pneumococcal Infection ◽  
Cell Disease ◽  
Older Children ◽  
American Academy Of Pediatrics ◽  
Increased Risk ◽  
Potential Problems ◽  
Life Threatening ◽  
Penicillin Prophylaxis

Abstract Children who have sickle cell disease and are under the age of five years are at increased risk of life-threatening pneumococcal infection due to absent or non-functional spleens and a decreased immune response. To prevent pneumococcal infection, the American Academy of Pediatrics recommends the use of penicillin prophylaxis in children with sickle cell disease under the age of five and in older children who have had a previous severe pneumococcal infection or have functional/surgical asplenia. These recommendations are based on two landmark studies, the first evaluating the effectiveness of penicillin prophylaxis and the second evaluating the duration of prophylaxis. Although the mortality rate from infection has been reduced following penicillin prophylaxis, altered immunologic response and penicillin-resistant S. pneumoniae remain a concern. This paper will review the literature that supports the use of penicillin prophylaxis, potential problems associated with prolonged therapy and recommendations for prophylaxis.

Evaluation of Transcranial Doppler in 1135 Sickle Cell Disease Children From Brazil: What Is the Time Averaged Maximum Velocity for High Risk of Stroke Among HbSC Patients?

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1003-1003
Author(s):  
Camilo Vieira ◽  
Isa Lyra ◽  
Marilda Goncalves
Keyword(s):  
Blood Flow ◽  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Internal Carotid ◽  
Cerebral Arteries ◽  
Maximum Velocity ◽  
Cell Disease ◽  
Middle Cerebral Arteries ◽  
The Mean

Abstract Abstract 1003 Introduction. Sickle cell disease (SCD) is an autosomal recessive hereditary disorder, characterized by the presence of hemoglobin S (HbS), and a clinical multisystem involvement. Stroke is the most disabling complication of SCD and has an incidence around 11% and 2% in sickle cell anemia (HbSS) and SCD patients before the age of 20 respectively. The Transcranial Doppler (TCD) is a noninvasive and safe diagnostic technique to monitor the cerebral mean blood flow velocities of SCD identifying those at risk for developing stroke, enabling the prophylactic treatment with chronic transfusion regime. Despite the high incidence of stroke in HbSC patients when compared with the pediatric population without HbSC, few studies have evaluated flow velocities by TCD in this genotype. Values used for risk stratification of TCD were obtained from HbSS or HbS/βthalassemia patients; therefore, theoretically, these cannot be extrapolated to HbSC patients. Aim. The aim of this study is to compare, by TDC, characteristics of cerebral blood flow among patients with HbSS, HbSC and HbS/βthalassemia. Patients and Methods. A cross-sectional study was performed from May 2011 to April 2011 in 1135 SCD patients aged from 2 and 16 years, at seven Brazilian states. Patients were submitted to a TCD screening (using a single device Doppler, probe 2Mhz model Ezdop), being excluded those with a prior stroke event or under chronic transfusion regimen. Time averaged maximum velocity (Tamm) in the middle cerebral arteries and distal internal carotid was obtained according to STOP protocol. Patients were stratified by SCD genotype. The study was approved by the research board from the State Government and all parents or guardians provided written informed consent. Results and Discussion. Females represented 46.3% (525) of the sample, and the mean age of 7.2±4.1 years. The Tamm at ACI/ACM was obtained at left and right respectively. In subjects with HbSS the velocity was of 131.7 cm/s and 130.7 cm/s; in patients with HbS/βthalassemia of 115.2 cm/s and 122.1 cm/s, and HbSC patients of 99.3 cm/s and 98.1 cm/s. Results of TDC were normal in 80.2% of SCD patients, conditional in 9.9%, 7.2% abnormal, 1.8% inconclusive, and 0.9% with low speed. The number of abnormal test, representing patients with a high risk for the occurrence of stroke among HbSS and β0thalassemia patients was 9.3%. This value was similar to the international literature that varies from 9.1% to 12.5%. One of the few studies that evaluated patients with HbSC through TCD was developed by Rees et al (2008) that analyzed 47 TCD from HbSC patients. The Tamm average found in the middle cerebral arteries was 94cm/s [12]. In this study, HbSC patients had an average Tamm of the middle cerebral arteries of 98.7 cm/s. Compared to patients with HbSS and Hb/βthalassemia, mean Tamm was significantly lower. Using rates of stroke risk in individuals with HbSS and HbS/βthalassemia, only 1.1% of HbSC patients would present high risk of stroke. However, differences in the mean Tamm in the middle cerebral arteries in HbSS patients (131.2 cm/s), HbS/βthalassemia (118.7 cm/s), and HbSC (98.7 cm/s), p<0.05, values suggest specific risk for HbSC patients was established. The average Tamm of middle cerebral artery/internal carotid distal in HbSC patients was 98.7 cm s with a standard deviation of 18.3 cm/s. Establishing standard deviations for HbSC patients, values above 135.3 cm/s could be considered high values for this population. In this case, considering as a cutoff point for HbSC patients speeds greater than 135.3 cm/s, 17 (6.2%) of individuals in our study would show high values. In the study of Rees, the Tamm who represented the 98th percentile was 128cm/s. Conclusion. Approximately 9 to 10% of HbSS and HbS/βthalassemia individuals in our sample have a high risk for stroke occurrence. However, data from HbSC patients' needs to be studied prospectively in order to establish if there is different TCD velocities values for an increased risk for stroke, contributing to preventive measures implementation. Disclosures: No relevant conflicts of interest to declare.

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