East Mediterranean region sickle cell disease mortality trial: retrospective multicenter cohort analysis of 735 patients

2016 ◽  
Vol 95 (6) ◽  
pp. 993-1000 ◽  
Author(s):  
Pelin Kardaş Karacaoglu ◽  
Suheyl Asma ◽  
Aslı Korur ◽  
Soner Solmaz ◽  
Nurhilal Turgut Buyukkurt ◽  
...  
Transfusion ◽  
2015 ◽  
Vol 56 (1) ◽  
pp. 107-114 ◽  
Author(s):  
Robert Sheppard Nickel ◽  
Jeanne E. Hendrickson ◽  
Ross M. Fasano ◽  
Erin K. Meyer ◽  
Anne M. Winkler ◽  
...  

1972 ◽  
Vol 4 (3) ◽  
pp. 307-313
Author(s):  
F. E. A. Lesi ◽  
Ekpo E. E. Bassey

A pilot survey has been made of Nigerian families at risk of producing SS haemoglobin homozygotes. Genotypes of parents and off-spring were investigated in twenty-five families. There is a suggestion that more SS children are produced where the first born suffers from haemoglobin SS disease. Mortality among offspring was higher in the study population than in the general population.


2021 ◽  
Vol 43 ◽  
pp. S29
Author(s):  
DC Stocco ◽  
FLS Santos ◽  
VEF Costa ◽  
ALL Morais ◽  
GC Santis ◽  
...  

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 28-29
Author(s):  
Jennifer G. Davila ◽  
Sarah H. O'Brien ◽  
Joseph R Stanek

Background: Evolving pediatric data, including case reports, case series, and combined adult and adolescent cohort studies, suggest an increased prevalence of venous thromboembolism (VTE) in children with Sickle Cell Disease (SCD) who undergo central venous catheter (CVC) placement (Ko RH and Thornburg C, Front Pediatr, 2017). Additionally, VTE has been shown to affect patients with SCD at a younger age compared to African-American controls (Stein PD, et al, Am J Med, 2006). Anecdotally, pediatric hematologists acknowledge the VTE risk in pediatric patients with SCD and have been using thromboprophylaxis in their patients despite the lack of standardized guidelines. The goal of this retrospective cohort study is to assess the utilization of pharmacologic and non-pharmacologic thromboprophylaxis in adolescent patients with SCD using a large pediatric database. Methods: The data source for this multicenter cohort study was the Pediatric Health Information Systems (PHIS), an administrative database that contains clinical and resource utilization data for inpatient, ambulatory surgery, emergency department, and observation unit patient encounters for 49 freestanding children's hospitals in the US. ICD-9-CM and ICD-10-CM codes were used to identify subjects, including SCD genotype (SS, SC and SB0 thal) and other comorbidities (acute chest syndrome, vaso-occlusive crisis, osteomyelitis, and bacteremia). We included subjects, aged 13-21 years, admitted to any PHIS hospital between 01/01/2010 - 12/31/2019. Subjects with a history of VTE were excluded. Supply billing codes were used to identify CVC placement and mechanical prophylaxis. Pharmaceutical billing codes were used to identify anticoagulant use. Results: 6,903 unique patients (3,147 male) met inclusion criteria with a median age of 17.2 years (13.0-21.9 years). These patients comprised a total of 33,298 admissions, with 2,434 (7.3%) admissions identified as receiving pharmacologic or mechanical prophylaxis (Table 1). 4.3% of the total admissions received mechanical prophylaxis and 3.3% received pharmacologic prophylaxis. Enoxaparin was the most commonly used anticoagulant, utilized in 91.3% of admissions in which pharmacologic anticoagulation was prescribed. The use of thromboprophylaxis was noted to increase over the study period, with significant increases observed in both pharmacologic and mechanical prophylaxis(p<.0001; Figure 1). The percentage of unique patients receiving thromboprophylaxis at each participating institution is shown in Figure 2. WConclusions: Use of both pharmacologic and mechanical thromboprophylaxis in hospitalized adolescent patients with SCD has increased in the last decade. However, there is variability amongst pediatric hospitals regarding the use and preferred method of thromboprophylaxis. Prospective cohort studies are needed to determine VTE risk factors in adolescents and children with SCD and the efficacy of prophylaxis regimens. These studies will help guide the development of VTE prophylaxis regimens. Disclosures Davila: ATHN: Other: Grant Funding; Spire Learning: Speakers Bureau. O'Brien:Bristol Myers Squibb: Consultancy, Membership on an entity's Board of Directors or advisory committees. OffLabel Disclosure: The use of Direct Oral Anticoagulants and Enoxaparin in children


2019 ◽  
Vol 53 (10) ◽  
pp. 991-996
Author(s):  
Alyssa M. Claudio ◽  
Lindsey Foltanski ◽  
Tracie Delay ◽  
Ashley Britell ◽  
Ashley Duckett ◽  
...  

Background: Acute chest syndrome (ACS) is an acute complication of sickle cell disease (SCD). Historically, the most common pathogens were Chlamydophila pneumoniae, Mycoplasma pneumoniae, and respiratory syncytial virus. Pediatric patients receiving guideline-adherent therapy experienced fewer ACS-related and all-cause 30-day readmissions compared with those receiving nonadherent therapy. This has not been evaluated in adults. Objectives: The primary objectives were to characterize antibiotic use and pathogens. The secondary objective was to assess the occurrence of readmissions associated with guideline-adherent and clinically appropriate treatment compared with regimens that did not meet those criteria. Methods: A retrospective cohort analysis was conducted for adults with SCD hospitalized between August 1, 2014, and July 31, 2017, with pneumonia (PNA) or ACS. The study was approved by the institutional review board. Results: A total of 139 patients with 255 hospitalizations were reviewed. Among 41 respiratory cultures, 3 organisms were isolated: Cryptococcus neoformans, Pseudomonas aeruginosa, and budding yeast. Respiratory panels were collected on 121 admissions, with 17 positive for 1 virus; all were negative for Chlamydophila pneumoniae and M pneumoniae. There were significantly more ACS-/PNA-related 7-day readmissions from patients on guideline-adherent regimens compared with nonadherent regimens (3.7% vs 0%; P = 0.04). Conclusion and Relevance: These findings challenge existing knowledge regarding the most common pathogens in adults with SCD with ACS or PNA. Routine inclusion of a macrolide may not be necessary. Future studies focused on pathogen characterization with standardized assessment are necessary to determine appropriate empirical therapy in this population.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 7-7 ◽  
Author(s):  
Riten Kumar ◽  
Joseph R Stanek ◽  
Susan E Creary ◽  
Sarah O'Brien

Abstract Background: A hypercoagulable state resulting in increased venous thrombo-embolism (VTE) has been described in adults with sickle cell disease (SCD). Similar data for children is lacking. Previously, in a single-institution, retrospective study of 414 pediatric patients with SCD followed at Nationwide Children's Hospital (NCH) between 2009 and 2015, we identified central venous catheters (CVC) as an independent risk factor for VTE [OR (± 95%CI): 10.3 (1.1-92.2)]. 12/414 (2.9%) subjects developed VTE over the course of the study1. The objective of this retrospective, multicenter cohort study was to describe risk factors associated with VTE in children with SCD across children's hospitals in the United States (US). Methods: This study was deemed to be exempt by the Institutional Review Board at NCH. Data source for this multicenter cohort study was the Pediatric Health Information Systems (PHIS), an administrative database that contains clinical and resource utilization data for inpatient, ambulatory surgery, emergency department and observation unit patient encounters for 49 free standing children's hospitals in the US. Data quality and reliability are assured through a joint effort between Children's Hospital Association and participating institutions2. ICD-9-CM codes were used to identify subjects. Eligible subjects were <21 years of age, were admitted to one of the PHIS hospitals between 01/01/2009 and 12/31/2013 and had at least 2 SCD specific ICD-9 discharge codes. VTE and comorbid conditions of interest (congenital heart disease, cancer, chronic renal disease, obesity, inflammatory bowel disease etc) were also identified using ICD-9 codes. Supply codes were used to identify CVC placement and pharmaceutical billing codes to identify oral contraceptive use. Logistic regression analysis was used to study association between unique patient characteristics and VTE. Due to the low event rate of VTE, logistic regression models were corrected using the Firth method. Variables found to be significant (p-value < 0.05) on univariate analysis were entered into a multivariable model. All data were summarized and presented using descriptive statistics. All statistical analyses were performed using SAS software, version 9.3 (SAS Institute, Cary, NC). Results: A total of 8941 unique subjects (4359 female) met inclusion criteria with a mean age (± 95%CI) of 7.28 (7.14-7.42) years. 159 subjects (96 female) developed VTE during the study period. Mean age (± 95%CI) at VTE diagnosis was 14.73 (13.84-15.63) years. No increase in VTE diagnosis was appreciated over the course of the study. On multivariable analysis, any CVC placement [OR (± 95%CI): 8.9 (6.45-12.3); p<0.0001], chronic renal disease [5.19 (1.48-18.19); p=0.01], female gender [1.59 (1.15-2.20); p=0.005), and older age at admission [1.10 (1.07-1.12); p<0.0001] were identified as risk factors associated with VTE diagnosis. Patients with SCD and VTE were more likely to be admitted to the intensive care unit (1.61 (0.99-2.62); p=0.05), though VTE diagnosis had no impact on mortality [1.49 (0.44-5.10); p=0.5]. Conclusion: Rate of VTE in children with SCD admitted to children's hospitals in the US is around 1.8%. CVC use is associated with a nearly 9-fold increased risk of VTE diagnosis. Additionally, chronic renal disease, female gender and older age at admission were also associated with VTE diagnosis. Prospective cohort studies are needed to confirm these findings and develop risk prediction models for VTE in children with SCD. Such studies will help develop and validate evidence based VTE prophylactic regimens for children with SCD. Reference: 1. Woods G, Sharma R, Creary S, et al. Venous thrombo-embolism (VTE) in children with sickle cell disease (SCD): an institutional experience. Journal of Thrombosis and Haemostasis. 2015;13:58-58. 2. Witmer CM, Lambert MP, O'Brien SH, Neunert C. Multicenter Cohort Study Comparing U.S. Management of Inpatient Pediatric Immune Thrombocytopenia to Current Treatment Guidelines. Pediatr Blood Cancer. 2016;63(7):1227-1231. Disclosures No relevant conflicts of interest to declare.


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