Trends In Mortality Rates and Age of Death In Sickle Cell Disease (SCD): 1979–2005

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 736-736
Author(s):  
Sophie Lanzkron ◽  
Carlton Haywood
Keyword(s):  
Sickle Cell Disease ◽  
Mortality Rate ◽  
Cause Of Death ◽  
Sickle Cell ◽  
Mortality Rates ◽  
Age At Death ◽  
Cell Disease ◽  
Time Period ◽  
The Mean ◽  
Age Of Death

Abstract Abstract 736 While improvements in care have resulted in significant decreases in mortality for children with SCD, it is unclear if similar decreases in mortality rates have occurred in adults with the disease. The purpose of this research is to describe mortality rates and trends in age of death for those with SCD over the last 3 decades. We used the National Center for Health Statistics Multiple Cause of Death (MCOD) files to examine age at death and calculate mortality rates from 1979–2005. ICD9 and ICD10 codes for SCD were used as appropriate to identify sickle cell related deaths. Mortality rates were calculated as deaths per 100,000 African American population. The number of African Americans each year was determined using available census data. Trends in mortality rates were examined using negative binomial regression and age of death was examined using t-tests and linear regression. After excluding certificates with codes for sickle trait and those with multiple sickle codes we identified 16,654 sickle-related deaths. The age range was 0 to 107 years. Mean age of death was significantly different for men (33.4, 95% CI [33.0, 33.7]) and women (36.9, 95% CI [36.5, 37.4]). SCD was the most common listed underlying cause of death (COD) at 62.8%. Infection was the second most common COD (5.9%). Controlling for sex and the presence of infection as COD, the mean age of death increased significantly by 0.08 years (p<0.001) each year over the time period studied, with men on average dying 4.3 years earlier than women (p<0.001). The mean age of death in 2005 was 43 yrs for women and 37 yrs for men. Those with COD of pulmonary hypertension, stroke and renal disease had a significantly older age of death than those without those diagnoses, while having infection as the underlying COD was associated with a younger age at death. The overall mortality rate increased by 0.7% (p<0.001) each year over the time period studied. Mortality rates for adults and children over time are shown in Figure 1. The adult (>19 yrs) mortality rate increased by 1% (p<0.001) each year over the time period studied. The pediatric mortality rate decreased by 3% (p<0.001) each year over the time period studied. When controlling for the pediatric mortality rate the adult mortality rate increased by 1.6% (p<0.001) each year. This data confirms prior studies showing a significant decrease in mortality for children with sickle cell disease over the last 30 yrs. The mortality rate for the adult population appears to be steadily increasing over the same time period. It seems unlikely that this is due merely to an influx of younger patients surviving to adulthood. Further investigation as to the cause of the increasing mortality rate in adults is needed. Disclosures: No relevant conflicts of interest to declare.

Sickle Cell Disease Mortality in the United States: Age at Death and Contributing Causes.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 81-81 ◽  
Author(s):  
Carlton Haywood ◽  
Sophie Lanzkron
Keyword(s):  
Sickle Cell Disease ◽  
Cause Of Death ◽  
Sickle Cell ◽  
Causes Of Death ◽  
College Education ◽  
Age At Death ◽  
Cell Disease ◽  
Time Period ◽  
Icd 10 ◽  
The U.S

Abstract BACKGROUND: In the early 1990’s, the Cooperative Study of Sickle Cell Disease (CSSCD) estimated a median life expectancy of 42 years for males, and 48 years for females with sickle cell anemia. We used death certificate data from the late 1990’s and early 2000’s to examine age at death and contributing causes of death for persons with sickle cell disease (SCD). METHODS: We used the National Center for Health Statistics Multiple Cause of Death (MCOD) files to examine age at death and contributing causes of death for persons in the U.S. with SCD during the years 1999 to 2004. The MCOD files contain data from all death certificates filed in the U.S. Each observation in the data has listed an underlying (primary) cause of death, as well as up to 20 conditions thought to contribute to the death. We used ICD-10 codes D570-D578 to identify all deaths attributed to SCD during the time period under study. Records with the ICD-10 code for sickle cell trait (D573) were excluded from further analyses. We used the Clinical Classification Software provided by the Healthcare Cost and Utilization Project to collapse all listed ICD-10 codes into smaller categories. Analyses of age at death were conducted using t-tests, median tests, ANOVA, and multiple linear regression as appropriate. RESULTS: From 1999 to 2004, there were 4553 deaths in the U.S. attributed to SCD (mean = 759/yr, sd = 42.6). SCD was listed as the primary cause in 65% of the deaths. 95% of the deaths were attributed to HbSS disease, and approximately 1% of the deaths were attributed to double heterozygous sickle cell disorders (SC/SD/SE/Thal). 50.4% of the deaths were among males. 64% of the decedents had a high school education or less. 54% of the decedents lived in the South. 68% of the decedents died as inpatients in a hospital. The mean age at death for the time period was 38.2 years (sd = 15.6). There was no change in the mean age at death during the time period. Females were older than males at death (39.4 vs. 36.9, p < 0.0001). Those with HbSS were younger than those with a double heterozygous disorder (38 vs. 47, p < 0.02). Having SCD listed as the primary cause of death was associated with younger age at death (36.8 vs. 40.7, p < 0.0001). Decedents with at least some college education were older at death than those with high school educations or less (40.9 vs. 37.0 p < 0.0001). There were no regional differences in mean age at death. In a multivariate model of age at death with the predictors gender, region, education, and whether or not SCD was listed as the primary cause of death, being female and having some college education remained associated with older age at death, while having SCD listed as the primary cause of death remained associated with younger age at death. Septicemia, pulmonary heart disease, liver disease and renal failure were among the top contributing causes of death for adults, while septicemia, acute cerebrovascular disease and pneumonia were among the top contributing causes of death for kids. CONCLUSIONS: Persons dying from SCD during 1999 to 2004 experienced ages at death that are not improved over those reported by the CSSCD, suggesting the continued need for societal efforts aimed at improving the quality of care for SCD, especially among adults with the condition. Educational attainment is associated with age at death among the SCD population, though it is not possible from the cross-sectional nature of this data to determine the causal directionality of this association.

scholarly journals Sickle Cell Disease Mortality in Brazil: Real-World Evidence

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3025-3025
Author(s):  
Rodolfo D Cançado ◽  
Fernando Ferreira Costa ◽  
Clarisse Lobo ◽  
Celina Borges Migliavaca ◽  
Maicon Falavigna ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Sickle Cell Disease ◽  
Mortality Rate ◽  
General Population ◽  
Life Expectancy ◽  
Cause Of Death ◽  
Sickle Cell ◽  
Age At Death ◽  
Cell Disease ◽  
Related Mortality

Abstract Background: Sickle cell disease (SCD) is a group of inherited chronic multisystem disorders, being one of the most common hereditary diseases in Brazil.[1] In a modelled US cohort, life expectancy of individuals with SCD was estimated to be 22 years lower compared to the general population (54 versus 76 years, respectively).[2] The life expectancy at birth in Brazil, for the general population, is 77 years.[3] However, little is known about the impact of SCD in mortality in Brazil. The aim of this study was to better understand the patters of mortality of SCD individuals, compared to the general population, using real-world data from Brazilian public databases. Methods: We retrieved data from all registered deaths in Brazil from 2015 to 2019 from the Brazilian public database of the Mortality Information System (Sistema de Informações sobre Mortalidade - SIM). To identify records from individuals with SCD, we selected records with an ICD-10 code referring to SCD (D57, D57.0, D57.1, D57.2 or D57.8) in any field related to cause of death. Using descriptive statistics, we explored the sex, race, age at death, and cause of death recorded in the registries. We also estimated the crude mortality rate of individuals with SCD (per 100.000 inhabitants) and compared the rates from each year with Pearson's correlation. Sensitivity analyses were conducted considering only registries with SCD as the primary (underlying) cause of death. All analyses were conducted with R, version 4.1.0. Results: A total of 6,553,132 deaths were registered in Brazil, from which 3,320 records were from individuals with SCD. Among them, the median age at death was 32.0 (IQR 19.0 - 46.0, Figure 1a), 37 years younger than the general population (median 69.0; IQR 53.0 - 81.0, Figure 1b). Considering only records with SCD as the primary cause of death, we identified 2,603 records. Among them, the median age at death was 30.0 (IQR 19.0 - 45.0). Similar results are observed in subgroup analyses according to sex and race (Table 1). Among registries from individuals with SCD, most were brown or black (79%) and women (52%). In comparison, in the general population, 45% of registries were from brown or black individuals, and 44% were from women. Over the five years evaluated, the crude annual mortality rate of SCD population was 0.32 per 100.000 inhabitants of the general population; considering only deaths with SCD as a primary cause, the mortality rate was 0.25 per 100.000 inhabitants. We did not observe substantial changes or statistical trends in the yearly mortality rate over the period evaluated (from 0.30 to 0.34; Pearson's r = -0.21; p = 0.735). The most common causes of death among individuals with SCD related mortality were sepsis (24.2%) and respiratory failure (7.9%). Conclusions: In Brazil, SCD is associated with a reduction of approximately 37 years on median age at death, in comparison with the general population; data was consistent when stratified by sex and race. This result is similar compared to two other studies: one considering the US context and one evaluating a Brazilian cohort from a reference local center, observed a loss of more than 20 years in the life expectancy of SCD patients at birth.[3, 4] As expected, the main causes of deaths were septicaemias and respiratory failure.[4, 5] Possible misdiagnoses, inaccurate completion of death certificates and possible confounding due to regional differences not accounted in these analyses are the main limitations of our study. References: 1- Lervolino, Luciana Garcia et al. Prevalence of sickle cell disease and sickle cell trait in national neonatal screening studies. Revista Brasileira de Hematologia e Hemoterapia. 2011;33(1):49-54. 2- IBGE - Instituto Brasileiro de Geografia e Estatística. Projeção da População do Brasil e das Unidades da Federação. Available in: https://www.ibge.gov.br/apps/populacao/projecao/. Accessed on July 13th, 2021. 3- Lubeck D, Agodoa I, Bhakta N, et al. Estimated Life Expectancy and Income of Patients with Sickle Cell Disease Compared with Those Without Sickle Cell Disease. JAMA Network Open. 2019;2(11):e1915374. 4- Lobo, CLC, Nascimento, EM, Jesus, LJC, et al. Mortality in children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil. Hematology, Transfusion and Cell Therapy. 2018;40(1):37-42. 5- Santo AH. Sickle cell disease related mortality in Brazil, 2000-2018. Hematology, Transfusion and Cell Therapy. 2020. Figure 1 Figure 1. Disclosures Cançado: Novartis: Consultancy. Costa: Novartis: Consultancy. Lobo: Novartis: Consultancy. Migliavaca: Novartis: Consultancy. Falavigna: Novartis: Consultancy. Filho: Novartis: Current Employment. Bueno: Novartis: Current Employment. Pinto: Novartis: Consultancy; Global Blood therapeutics (GBT): Consultancy; EMS, Brazil: Consultancy.

Does Treatment with Hydroxyurea and Opioids Affect Age of Death in Sickle Cell Disease Patients?

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4808-4808
Author(s):  
Charlene M. Flahiff ◽  
Jude C Jonassaint ◽  
Charles R. Jonassaint ◽  
Soheir S. Adam ◽  
Marilyn J. Telen ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Treatment Modality ◽  
Group Analysis ◽  
Treatment Modalities ◽  
Age At Death ◽  
Cell Disease ◽  
Number Of Patients ◽  
Living Group ◽  
Age Of Death

Abstract Hydroxyurea (HU) is used to treat sickle cell disease and has been shown to decrease painful episodes (Charache, 1995) and possibly vaso-occlusive episodes associated morbidity and mortality (Steinberg, 2003). Opioids are often prescribed in adult patients for daily management of their chronic pain. The aim of the current study was to determine the age at death and the effect of treatment with HU and/or opioids prior to death in patients who died from sickle cell disease (SCD) related complications and to compare these parameters to those in our current patients population. Methods: Age, daily treatment with opioids, and HU treatment were determined for 185 patients currently followed at the Duke Comprehensive Sickle Cell Center (DCSCC) and for 50 patients who died between 2002 and 2008 due to SCD complications and who were regularly followed at the DCSCC for their care. The two cohorts, living and deceased patients were divided based on their treatment modality into the following 4 groups: opioid only, HU only, both drugs, and neither drug. Non-parametric chi-square test was performed to determine whether the treatment modality distribution was different in the deceased group compared to the living group. Analysis of variance was done to determine the relationship between treatment group and age at death. Results and Discussion: The distribution of treatment modalities in the deceased group was significantly different than that of the living group. The opioids only group had the largest number of patients in the deceased cohort (44%), and this percentage was almost twice that of the living group (25%). (Fig. 1) Moreover, 72 % of the deceased patients were treated with opioids vs. only 53 % of the living patients, perhaps because the sicker patients are often treated with daily opioids. However, the age of death in the opioids only group was 44 ± 15.5 years. (Fig. 2) In the living group, treatment with both drugs or with no drugs were equivalent (28%), while in the deceased group, more patients were treated with both drugs (28%) compared to no drug treatment (18%). The HU only group had the lowest number of deaths (10%), and the percentage of patients in this group was nearly half that the one of the living group (19%). This group also was the oldest at death (58 ±16 years). Age at death was also significantly higher in this group than in each of the other 3 groups (p&lt;0.05). The low use of HU in the deceased cohort, along with the higher age of death, support the reported effectiveness of this drug in reducing morbidity associated with SCD. The age of the living patients receiving treatment with both drugs was the same as that of the deceased. Similarly the age of the non treated living patients was comparable with that of the deceased group. Interestingly, the interaction of the 2 therapeutic interventions (HU and opiates) had a significant effect on the age at death (p=0.003). We conclude that opioid treatment, either alone or in conjunction with HU, appears to have a significant effect on the age at death and warrants further investigation.

scholarly journals Mortality Rates and Age at Death from Sickle Cell Disease: U.S., 1979–2005

2013 ◽  
Vol 128 (2) ◽  
pp. 110-116 ◽  
Author(s):  
Sophie Lanzkron ◽  
C. Patrick Carroll ◽  
Carlton Haywood
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Mortality Rates ◽  
Age At Death ◽  
Cell Disease

Mortality in Sickle Cell Patient Transitioning from Pediatric to Adult Program: 10 Years Grady Comprehensive Sickle Cell Center Experience.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1426-1426 ◽  
Author(s):  
Si mbo O Aduloju ◽  
Sheila Palmer ◽  
James R. Eckman
Keyword(s):  
Young Adults ◽  
Sickle Cell Disease ◽  
Mortality Rate ◽  
Sudden Death ◽  
Iron Overload ◽  
Organ Failure ◽  
Cause Of Death ◽  
Sickle Cell ◽  
Cell Disease ◽  
Adult Care

Abstract Over the last three decades there has been improvement in survival in children with sickle cell disease. Overall survival from birth to age 18 of 86% and 95% has been reported in children with sickle cell anemia (HbSS) and sickle hemoglobin C (Hb SC) disease respectively. These encouraging results are secondary to initiation of preventive measures like newborn screening, penicillin prophylaxis, immunization and stroke prevention and other supportive therapies. Ballas (Blood2004;104 supl:Abstr 3743) reported high death rate for young adults with the disease. To determine the death rates and cause of death in our population, we did a retrospective review of our patients who had recently transitioned into adult care. Mortality rate and circumstances of death in a 10 year period of transition from pediatric to adult program in Georgia Comprehensive Sickle Cell Center at Grady Memorial Hospital between 1996 and 2006 were determined. Total number of deaths was obtained from the clinical database. Death, autopsy and medical records were reviewed to determine the cause and circumstances of death. Between 1996 and 2006, 387 young adults with sickle cell disease (HbSS, HbSC, HbS beta thalassemia) transitioned to the adult program at age 18. Twenty two (5.8%) patients died during their first 10 years of transition. Mean age at death was 23.4 (range 21.6–26) and male to female ratio was 1.2 to 1. Ten patients (45%) died due to chronic organ failure all due to chronic iron overload (end stage liver disease in 8 and congestive heart failure in 2). Eight patients (36%) died due to complications of acute vaso-occlusive crisis (sudden death in 4, acute chest/multi-organ failure in 4) while 4 patients (18%) died of non-sickle related causes. Three of four who died of sudden death has documented pulmonary hypertension. Our mortality rate is lower than those reported by Ballas with the most common cause of death being complications of iron overload in chronically transfused patients. Specific interventions targeted at improving management of iron overload need to be developed for the increasing numbers of patients on chronic transfusion transitioning into adult care.

Effect of active prenatal management on pregnancy outcome in sickle cell disease in an African setting

Blood ◽  
2000 ◽  
Vol 96 (5) ◽  
pp. 1685-1689
Author(s):  
Mohamed C. Rahimy ◽  
Annick Gangbo ◽  
Roslyn Adjou ◽  
Chantal Deguenon ◽  
Stephanie Goussanou ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Mortality Rate ◽  
Maternal Mortality ◽  
Pregnancy Outcome ◽  
Sickle Cell ◽  
Mortality Rates ◽  
Cell Disease ◽  
Maternal Mortality Rate ◽  
Prenatal Management ◽  
African Setting

Sickle cell disease (SCD) is associated with an increased risk of medical complications during pregnancy. In sub-Saharan Africa, fetal and maternal mortality rates are particularly high. This study evaluated the effect of an active prenatal management program on pregnancy outcome in patients with SCD in an African setting. Pregnant women with SCD attending the National Teaching Hospital in Cotonou (The Republic of Benin, West Africa) were recruited before the 28th week of gestation. Management was based on providing information and education about SCD and improving nutritional status, malaria prevention, early detection of bacterial infections, and restricted use of blood transfusion. Maternal and fetal mortality rates and SCD-related morbidity were the principal variables assessed. One hundred and eight patients (42 SS and 66 SC) with 111 fetuses were included in the study. Thirteen fetal deaths (from 9 SS and 4 SC mothers) were recorded and 2 deaths of SC mothers. The maternal mortality rate of 1.8% was comparable with the overall maternal mortality rate for this maternity unit (1.2%). Few SCD-related events were recorded. Plasmodium falciparum malaria infection was the major cause of morbidity. Sixty-three patients (19 SS and 44 SC) successfully completed their pregnancy (58.3%) without requiring transfusion. Providing pregnant SCD patients with relevant medical care based on simple cost-effective approaches can have a positive impact on SCD-associated morbidity and mortality in an otherwise difficult setting in Africa.

Effect of active prenatal management on pregnancy outcome in sickle cell disease in an African setting

Blood ◽  
2000 ◽  
Vol 96 (5) ◽  
pp. 1685-1689 ◽  
Author(s):  
Mohamed C. Rahimy ◽  
Annick Gangbo ◽  
Roslyn Adjou ◽  
Chantal Deguenon ◽  
Stephanie Goussanou ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Mortality Rate ◽  
Maternal Mortality ◽  
Pregnancy Outcome ◽  
Sickle Cell ◽  
Mortality Rates ◽  
Cell Disease ◽  
Maternal Mortality Rate ◽  
Prenatal Management ◽  
African Setting

Abstract Sickle cell disease (SCD) is associated with an increased risk of medical complications during pregnancy. In sub-Saharan Africa, fetal and maternal mortality rates are particularly high. This study evaluated the effect of an active prenatal management program on pregnancy outcome in patients with SCD in an African setting. Pregnant women with SCD attending the National Teaching Hospital in Cotonou (The Republic of Benin, West Africa) were recruited before the 28th week of gestation. Management was based on providing information and education about SCD and improving nutritional status, malaria prevention, early detection of bacterial infections, and restricted use of blood transfusion. Maternal and fetal mortality rates and SCD-related morbidity were the principal variables assessed. One hundred and eight patients (42 SS and 66 SC) with 111 fetuses were included in the study. Thirteen fetal deaths (from 9 SS and 4 SC mothers) were recorded and 2 deaths of SC mothers. The maternal mortality rate of 1.8% was comparable with the overall maternal mortality rate for this maternity unit (1.2%). Few SCD-related events were recorded. Plasmodium falciparum malaria infection was the major cause of morbidity. Sixty-three patients (19 SS and 44 SC) successfully completed their pregnancy (58.3%) without requiring transfusion. Providing pregnant SCD patients with relevant medical care based on simple cost-effective approaches can have a positive impact on SCD-associated morbidity and mortality in an otherwise difficult setting in Africa.

scholarly journals Survival of children with sickle cell disease

Blood ◽  
2004 ◽  
Vol 103 (11) ◽  
pp. 4023-4027 ◽  
Author(s):  
Charles T. Quinn ◽  
Zora R. Rogers ◽  
George R. Buchanan
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Survival Data ◽  
Childhood Mortality ◽  
Age At Death ◽  
Cell Disease ◽  
Inception Cohort ◽  
Free Survival ◽  
Hemoglobin C ◽  
The Mean

Abstract Contemporary survival data are not available for children with sickle cell disease (SCD). The few previous childhood SCD cohort studies do not reflect the benefits of modern therapy. We defined an inception cohort of newborns with sickle cell anemia (SS), sickle-β°-thalassemia (S β°), sickle-hemoglobin C disease (SC), or sickle-β+-thalassemia (Sβ+) who were identified by newborn screening and followed for up to 18 years. The incidence of death and stroke were calculated. Overall survival, SCD-related survival (considering only SCD-related deaths), and strokefree survival were determined. The 711 subjects provided 5648 patient-years of observation. Twenty-five subjects died; mean age at death was 5.6 years. Five patients died from infection. Thirty had at least one stroke. Among SS and Sβ° subjects (n = 448), the overall rates of death and stroke were 0.59 and 0.85/100 patient-years. Survival analysis of SS and Sβ° subjects predicted the cumulative overall, SCD-related, and stroke-free survival to be 85.6%, 93.6%, and 88.5% by 18 years of age. No SCD-related deaths or strokes occurred in SC or Sβ+ subjects (n = 263). Childhood mortality from SCD is decreasing, the mean age at death is increasing, and a smaller proportion of deaths are from infection.

scholarly journals Prevalence and Predisposing Factors of Atrial Fibrillation in a Multi-Ethnic Society: The Impact of Racial Differences in Bahrain

2011 ◽  
Vol 4 ◽  
pp. OJCS.S8032 ◽  
Author(s):  
Taysir Garadah ◽  
Saleh Gabani ◽  
Mohamed Al Alawi ◽  
Ahmed Abu-Taleb
Keyword(s):  
Atrial Fibrillation ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Middle Eastern ◽  
Predisposing Factors ◽  
Cell Disease ◽  
Clinical Events ◽  
History Of ◽  
The Mean ◽  
One Year

Background The prevalence and epidemiological data of atrial fibrillation (AF) among multi-ethnic populations is less well studied worldwide. Aim Evaluation of the prevalence and predisposing factors of AF in patients who were admitted to acute medical emergencies (ER) in Bahrain over the period of one year. Methods Two hundred and fifty three patients with onset of AF were studied. The mean difference of biochemical data and clinical characteristics between Middle Eastern (ME) and sub continental (SC) patients was evaluated. The odds ratio of different predisposing factors for the development of clinical events in AF patients was assessed using multiple logistic regression analysis. Results Out of 7,450 patients that were admitted to ER over one year, 253 had AF based on twelve leads Electrocardiogram (ECG), with prevalence of 3.4%. In the whole study, the mean age was 59.45 ± 18.27 years, with 164 (65%) male. There were 150 ME patients (59%), and 107 (41%) SC, 55 (22%) were Indian (IND) and 48 (19%) were South Asian (SA). In the whole study clinical presentation was of 48% for palpitation, pulmonary edema was of 14%, angina pectoris on rest of 12%, 10% had embolic phenomena, 6% had dizziness, and 7% were asymptomatic. The odds ratio of different variables for occurrence of clinical events in the study was positive of 2.2 for history of hypertension, 1.8 for sickle cell disease, 1.2 for high body mass index (BMI) >30, 1.1 for mitral valve disease. The ME patients, compared with SC, were older, had significantly higher body mass index, higher history of rheumatic valve disease, sickle cell disease with high level of uric acid and lower hemoglobin. The history of hypertension, DM and smoking was higher among the SC patients. The rate of thyroid disease was equal in both groups. Conclusion The prevalence of atrial fibrillation was 3.4% with male predominance of 65%. Patients of sub continental origin were younger with a significantly high history of hypertension and ischemic heart disease. The patients of Middle Eastern origin had significantly high rate of rheumatic heart disease, and sickle cell disease. The history of hypertension was the most important independent clinical predictor of adverse events in patients presented with AF.

scholarly journals Ischemic Stroke in Children and Young Adults with Sickle Cell Disease (SCD) in the Post-STOP Era

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 68-68 ◽  
Author(s):  
Janet L. Kwiatkowski ◽  
Julie Kanter ◽  
Heather J. Fullerton ◽  
Jenifer Voeks ◽  
Ellen Debenham ◽  
...  
Keyword(s):  
Ischemic Stroke ◽  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Stroke Prevention ◽  
Cell Disease ◽  
Clinical Series ◽  
The Mean

Abstract Background: The Stroke Prevention Trial in Sickle Cell Anemia (STOP) and Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) established routine transcranial Doppler ultrasound (TCD) screening with indefinite chronic red cell transfusions (CRCT) for children with abnormal TCD as standard of care. To identify children at high-risk of stroke, annual TCD screening is recommended from ages 2 to 16 years, with more frequent monitoring if the result is not normal. A reduction in stroke incidence in children with SCD has been reported in several clinical series and analyses utilizing large hospital databases when comparing rates before and after the publication of the STOP study in 1998. We sought to determine the rate of first ischemic stroke in a multicenter cohort of children who had previously participated in the STOP and/or STOP 2 trials and to determine whether these strokes were screening or treatment failures. Subjects and Methods: Between 1995 and 2005, STOP and STOP 2 (STOP/2) were conducted at 26 sites in the US and Canada. These studies included 3,835 children, ages 2 to 16 y with SCD type SS or S-beta-0-thalassemia. Participation in STOP/2 ranged from a single screening TCD to randomization. STOP 2 also had an observational arm for children on CRCT for abnormal TCD whose TCD had not reverted to normal. The Post-STOP study was designed to follow-up the outcomes of children who participated in one or both trials. 19 of the 26 original study sites participated in Post-STOP, contributing a total of 3,539 (92%) of the STOP/2 subjects. After exit from STOP/2, these children received TCD screening and treatment according to local practices. Data abstractors visited each clinical site and obtained retrospective data from STOP/2 study exit to 2012-2014 (depending on site) including follow-up TCD and brain imaging results, clinical information, and laboratory results. Two vascular neurologists, blinded to STOP/2 status and prior TCD and neuroimaging results, reviewed source records to confirm all ischemic strokes, defined as a symptomatic cerebral infarction; discordant opinions were resolved through discussion. For the first Post-STOP ischemic stroke, prior TCD result and treatment history subsequently were analyzed. Results: Of the 3,539 subjects, follow-up data were available for 2,850 (81%). Twelve children who had a stroke during STOP or STOP2 were excluded from these analyses resulting in data on 2,838 subjects. The mean age at the start of Post-STOP was 10.5 y and mean duration of follow-up after exiting STOP/2 was 9.1 y. A total of 69 first ischemic strokes occurred in the Post-STOP observation period (incidence 0.27 per 100 pt years). The mean age at time of stroke was 14.4±6.2 (median 13.8, range 3.5-28.9) y. Twenty-five of the 69 patients (36%) had documented abnormal TCD (STOP/2 or Post-STOP) prior to the stroke; 15 (60%) were receiving CRCT and 9 (36%) were not (treatment data not available for 1 subject). Among the 44 subjects without documented abnormal TCD, 29 (66%) had not had TCD re-screen in the Post-STOP period prior to the event; 7 of these 29 (24%) were 16 y or older at the start of Post-STOP, which is beyond the recommended screening age. Four of the 44 (9%) patients had inadequate TCD in Post-STOP (1 to 10.7 y prior to event). Six (14%) had normal TCD more than a year before the event (1.2 - 4 y); all but one of these children were younger than 16 y at the time of that TCD. Only 5 (11%) had a documented normal TCD less than 1 year prior to the event. Conclusions: In the Post-STOP era, the rate of first ischemic stroke was substantially lower than that reported in the Cooperative Study of Sickle Cell Disease, prior to implementation of TCD screening. Many (39%) of the Post-STOP ischemic strokes were associated with a failure to re-screen according to current guidelines, while only 11% occurred in children who had had recent low-risk TCD. Among those known to be at high risk prior to stroke, treatment refusal or inadequate treatment may have contributed. While TCD screening and treatment are effective at reducing ischemic stroke in clinical practice, significant gaps in screening and treatment, even at sites experienced in the STOP protocol, remain to be addressed. Closing these gaps should provide yet further reduction of ischemic stroke in SCD. Disclosures No relevant conflicts of interest to declare.

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