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1.
Causes and Risk Factors of Pediatric Spontaneous Intracranial Hemorrhage-A Systematic Review
Ciochon UM, Bindslev JBB, Hoei-Hansen CE, Truelsen TC, Larsen VA, Nielsen MB, Hansen AE
Diagnostics (Basel, Switzerland). 2022;12(6)
Abstract
Previous studies suggest that the most common cause of spontaneous intracerebral hemorrhage in children and adolescents is arteriovenous malformations (AVMs). However, an update containing recently published data on pediatric spontaneous intracranial hemorrhages is lacking. The aim of this study is to systematically analyze the published data on the etiologies and risk factors of pediatric spontaneous intracranial hemorrhage. This systematic review was performed in compliance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. A search in PubMed, Embase, Scopus, Web of Science and Cochrane Library was conducted aiming for articles published in year 2000 and later, containing data on etiology and risk factors of spontaneous intracranial hemorrhages in unselected cohorts of patients aged between 1 month and 18 years. As a result, forty studies were eligible for data extraction and final analysis. These included 7931 children and adolescents with 4009 reported etiologies and risk factors. A marked variety of reported etiologies and risk factors among studies was observed. Vascular etiologies were the most frequently reported cause of pediatric spontaneous intracranial hemorrhages (n = 1727, 43.08% of all identified etiologies or risk factors), with AVMs being the most common vascular cause (n = 1226, 70.99% of all vascular causes). Hematological and systemic causes, brain tumors, intracranial infections and cardiac causes were less commonly encountered risk factors and etiologies.
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Coagulation in pediatric extracorporeal membrane oxygenation: A systematic review of studies shows lack of standardized reporting
Drop J, Van Den Helm S, Monagle P, Wildschut E, de Hoog M, Gunput STG, Newall F, Dalton HJ, MacLaren G, Ignjatovic V, et al
Research and practice in thrombosis and haemostasis. 2022;6(2):e12687
Abstract
OBJECTIVES Extracorporeal membrane oxygenation (ECMO) involves complex coagulation management and frequent hemostatic complications. ECMO practice between centers is variable. To compare results between coagulation studies, standardized definitions and clear documentation of ECMO practice is essential. We assessed how study population, outcome definitions, and ECMO-, coagulation-, and transfusion-related parameters were described in pediatric ECMO studies. DATA SOURCES Embase, Medline, Web of Science, Cochrane Library and Google Scholar. STUDY SELECTION English original studies of pediatric ECMO patients describing hemostatic tests or outcome. DATA EXTRACTION Eligibility was assessed following PRISMA guidelines. Study population, outcome and ECMO-, coagulation, and transfusion parameters were summarized. DATA SYNTHESIS A total of 107 of 1312 records were included. Study population parameters most frequently included (gestational) age (79%), gender (60%), and (birth) weight (59%). Outcomes, including definitions of bleeding (29%), thrombosis (15%), and survival (43%), were described using various definitions. Description of pump type, oxygenator and cannulation mode occurred in 49%, 45%, and 36% of studies, respectively. The main coagulation test (53%), its reference ranges (49%), and frequency of testing (24%) were the most prevalent reported coagulation parameters. The transfusion thresholds for platelets, red blood cells, and fibrinogen were described in 27%, 18%, and 18% of studies, respectively. CONCLUSIONS This systematic review demonstrates a widespread lack of detail or standardization of several parameters in coagulation research of pediatric ECMO patients. We suggest several parameters that might be included in future coagulation studies. We encourage the ECMO community to adopt and refine this list of parameters and to use standardized definitions in future research.
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A scoping review of transition interventions for young adults with sickle cell disease
Viola A, Porter J, Shipman J, Brooks E, Valrie C
Pediatric blood & cancer. 2021;:e29135
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Abstract
Standardized programming for individuals with sickle cell disease (SCD) transitioning from pediatric to adult-centered care does not currently exist, resulting in high rates of mortality and morbidity. This scoping review examines and evaluates the current literature on SCD transition programs and interventions. Eligible studies described an existing program for individuals with SCD aged 12-29 years preparing to transition. The Evidence Project risk-of-bias tool was used to assess article quality. We identified 30 eligible articles, of which, only two were randomized controlled trials. Many studies have incomplete reports of feasibility information, such as completion rates, patient characteristics, and attrition; all studies were limited to a single institution; and most studies were rated high for risk of bias. Progress has been made in designing and gathering initial evaluation data for SCD transition programs; however, there is a need for higher quality studies, consistent assessment, and better dissemination of programs.
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Metrics of shock in pediatric trauma patients: A systematic search and review
Alberto, E. C., McKenna, E., Amberson, M. J., Tashiro, J., Donnelly, K., Thenappan, A. A., Tempel, P. E., Ranganna, A. S., Keller, S., Marsic, I., et al
Injury. 2021;52(10):3166-3172
Abstract
INTRODUCTION Shock-index (SI) and systolic blood pressure (SBP) are metrics for identifying children and adults with hemodynamic instability following injury. The purpose of this systematic review was to assess the quality of these metrics as predictors of outcomes following pediatric injury. MATERIALS AND METHODS We conducted a literature search in Pubmed, SCOPUS, and CINAHL to identify studies describing the association between shock metrics on the morbidity and mortality of injured children and adolescents. We used the data presented in the studies to calculate the sensitivity and specificity for each metric. This study was registered with Prospero, protocol CRD42020162971. RESULTS Fifteen articles met the inclusion criteria. seven studies evaluated SI or SIPA score, an age-corrected version of SI, as predictors of outcomes following pediatric trauma, with one study comparing SIPA score and SBP and one study comparing SI and SBP. The remaining eight studies evaluated SBP as the primary indicator of shock. The median sensitivity for predicting mortality and need for blood transfusion was highest for SI, followed by SIPA, and then SBP. The median specificity for predicting these outcomes was highest for SBP, followed by SIPA, and then SI. CONCLUSIONS Common conclusions were that high SIPA scores were more specific than SI and more sensitive than SBP. SIPA score had better discrimination for severely injured children compared to SI and SBP. An elevated SIPA was associated with a greater need for blood transfusion and higher in-hospital mortality. SIPA is specific enough to exclude most patients who do not require a blood transfusion.
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Evidence-based interventions implemented in low-and middle-income countries for sickle cell disease management: A systematic review of randomized controlled trials
Gyamfi J, Ojo T, Epou S, Diawara A, Dike L, Adenikinju D, Enechukwu S, Vieira D, Nnodu O, Ogedegbe G, et al
PloS one. 2021;16(2):e0246700
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Editor's Choice
Abstract
BACKGROUND Despite ~90% of sickle cell disease (SCD) occurring in low-and middle-income countries (LMICs), the vast majority of people are not receiving evidence-based interventions (EBIs) to reduce SCD-related adverse outcomes and mortality, and data on implementation research outcomes (IROs) and SCD is limited. This study aims to synthesize available data on EBIs for SCD and assess IROs. METHODS We conducted a systematic review of RCTs reporting on EBIs for SCD management implemented in LMICs. We identified articles from PubMed/Medline, Global Health, PubMed Central, Embase, Web of Science medical subject heading (MeSH and Emtree) and keywords, published from inception through February 23, 2020, and conducted an updated search through December 24, 2020. We provide intervention characteristics for each study, EBI impact on SCD, and evidence of reporting on IROs. MAIN RESULTS 29 RCTs were analyzed. EBIs identified included disease modifying agents, supportive care agents/analgesics, anti-malarials, systemic treatments, patient/ provider education, and nutritional supplements. Studies using disease modifying agents, nutritional supplements, and anti-malarials reported improvements in pain crisis, hospitalization, children's growth and reduction in severity and prevalence of malaria. Two studies reported on the sustainability of supplementary arginine, citrulline, and daily chloroquine and hydroxyurea for SCD patients. Only 13 studies (44.8%) provided descriptions that captured at least three of the eight IROs. There was limited reporting of acceptability, feasibility, fidelity, cost and sustainability. CONCLUSION EBIs are effective for SCD management in LMICs; however, measurement of IROs is scarce. Future research should focus on penetration of EBIs to inform evidence-based practice and sustainability in the context of LMICs. CLINICAL TRIAL REGISTRATION This review is registered in PROSPERO #CRD42020167289.
PICO Summary
Population
Children and adults with sickle cell disease (SCD) in 14 low- and middle-income countries, (30 studies).
Intervention
Evidence-based interventions including: disease modifying agents, supportive care agents/analgesics, anti-malarials, systemic treatments (e.g., red blood cell transfusions), patient/provider education, and nutritional supplements.
Comparison
Placebo or comparator intervention
Outcome
Studies using disease modifying agents, nutritional supplements, and anti-malarials reported improvements in pain crisis, hospitalization, children's growth and reduction in severity and prevalence of malaria. Two studies reported on the sustainability of supplementary arginine, citrulline, and daily chloroquine and hydroxyurea for SCD patients. Only 13 studies (44.8%) provided descriptions that captured at least three of the eight implementation research outcomes. There was limited reporting of acceptability, feasibility, fidelity, cost and sustainability.
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Use of electronic self-administered bleeding assessment tool in diagnosis of paediatric bleeding disorders
Kaur D, Kerlin BA, Stanek JR, O'Brien SH
Haemophilia : the official journal of the World Federation of Hemophilia. 2021
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Editor's Choice
Abstract
INTRODUCTION In the era of electronic medical records, pen-and-paper-based physician-administered bleeding assessment tools (BAT) remain under-utilized in the clinical setting, as they are noted to be time-consuming. AIM: The current study reviews the use of an electronic self-administered bleeding assessment tool (eBAT) prospectively in a paediatric haematology clinic and in comparison with a physician administered BAT (pBAT). MATERIALS AND METHODS This was reviewed and approved in the current form because the aims statement includes the method regarding comparison of 2 groups. So no additional section required. RESULTS A total of 94 BAT response pairs were available for analysis. The median time required for patients or parents to complete the eBAT was 8 min, with less than a third of the patients requiring over 10 min. The median bleeding scores noted in this study were 4 for both the BATs, with strong positive correlation between the eBAT and the physician administered bleeding questionnaire. The eBAT had a sensitivity of 93.8% (95% CI 82.8%-98.7%), a specificity of 34.8% (95% CI 21.4%-50.3%), a positive predictive value (PV) of 60.0% (95% CI 54.5%-65.2%) and a negative PV of 84.2% (95% CI 62.5%-94.5%) for identifying a bleeding disorder. CONCLUSIONS Findings indicate that eBAT is a valid and time-efficient screening tool for evaluating patients' bleeding symptoms, which can improve clinical applicability of BATs by reducing time for bleeding history review.
PICO Summary
Population
Paediatric haematology patients (n=94).
Intervention
Electronic self-administered bleeding assessment tool (eBAT), (n= 94).
Comparison
Physician administered BAT (pBAT), (n= 94).
Outcome
The median time required for patients or parents to complete the eBAT was 8 min, with less than a third of the patients requiring over 10 min. The median bleeding scores noted in this study were 4 for both the BATs, with strong positive correlation between the eBAT and the physician administered bleeding questionnaire. The eBAT had a sensitivity of 93.8%, a specificity of 34.8%, a positive predictive value (PV) of 60.0%, and a negative PV of 84.2% for identifying a bleeding disorder.
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Neonatal Uterine Bleedings: An Ignored Sign but a Possible Cause of Early-Onset Endometriosis - A Systematic Review
Dekker J, Hooijer I, Ket JCF, Vejnović A, Benagiano G, Brosens I, Mijatovic V
Biomedicine hub. 2021;6(1):6-16
Abstract
OBJECTIVE Based on the hypothesis that neonatal uterine bleedings (NUB), occurring mostly in the first week after birth, could represent a pathogenetic mechanism for early-onset endometriosis, this systematic review (SR) was undertaken to evaluate the prevalence and screening strategies used to assess and quantify NUB. DESIGN Both a SR and a sample literature search in PubMed and Embase were conducted to gather information on NUB prevalence and screening techniques. This was performed by an information specialist. Only full-text articles regarding the assessment of NUB in neonates in the first 2 weeks after birth were included. No limit on language or publication data was used. MATERIALS AND METHODS The SR was registered in PROSPERO (CRD42019138121). Data was first assessed for eligibility on title and abstract by 2 blinded review authors. Any disagreements were discussed with a third reviewer if necessary. Subsequently, full-text articles were read and assessed for quality using the Cochrane Collaboration Handbook. RESULTS Out of 1,988 articles in the systematic search, 10 relevant articles were selected, of which 8 were identified through the systematic search and 2 were found through other sources. The sample search of 4,445 articles did not bring up relevant articles. Results were not comparable due to the heterogeneity of screening techniques, although data showed consensus. The prevalence of visible bleeding ranged from 3.3 to 53.8% and the prevalence of occult bleeding from 25.4 to 96.7%. The occurrence was the highest between the 3rd and 7th day postpartum (PP) and the bleeding lasted for 3-4 days on average. Various screening techniques for detecting NUB were found in the literature, including the use of hemoglobin detection devices (such as Hemastix) in the vaginal vestibulum, comparison of diapers with stains of known volume, colposcopy, and ultrasonography. CONCLUSION The reported prevalence of NUB varies considerably, with a consistent occurrence between the 3rd and the 7th day PP. Literature to assess NUB is dated. The techniques are poorly described and heterogeneous. Future research should focus on prospective cohort studies in order to attempt to correlate NUB cases to (early-onset) endometriosis.
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Challenges in Maintaining the Hemostatic Balance in Children Undergoing Extracorporeal Membrane Oxygenation: A Systematic Literature Review
Drop JGF, Wildschut ED, Gunput STG, de Hoog M, van Ommen CH
Frontiers in pediatrics. 2020;8:612467
Abstract
Background: Despite advances in technology and clinical experience, the incidence of hemostatic complications, including bleeding and thrombosis, remains high in children supported with extracorporeal membrane oxygenation (ECMO). These hemostatic complications are important to prevent, since they are associated with increased morbidity and mortality. This systematic literature review aims to outline the most important risk factors for hemostatic complications in children undergoing ECMO treatment, to summarize the reported alternative anticoagulant drugs used in pediatric ECMO and to describe studied associations between coagulation tests and hemostatic complications. Methods: A literature search was performed in Embase, Medline, Web of Science Core Collection, Cochrane Central Register of Controlled Trials, and Google Scholar in February 2020. Included studies were studies evaluating children (<18 years old) treated with ECMO, and studies evaluating risk factors for hemostatic complications, alternative anticoagulants, or the association between coagulation tests and hemostatic complications. Results: Out of 1,152 articles, 35 studies were included. Thirteen out of 49 risk factors were investigated in three or more studies. Most consistent results were found regarding ECMO duration and pH. However, evidence for risk factors was equivocal in the majority of studies, which is explained by the variability of populations studied, definitions of hemostatic complications, ECMO circuits, anticoagulation protocols, transfusion triggers and monitoring of anticoagulation. Five studies described alternative anticoagulants, including bivalirudin (n = 3), argatroban (n = 1) and FUT (n = 1). Higher anti-factor Xa levels were associated with less clotting events in one of nine studies, investigating the association between tests and hemostatic complications. Two studies revealed an association between anti-factor Xa assay-based protocols and a decreased number of transfusions, bleedings and need for circuit change. Conclusion: Studies regarding risk factors showed conflicting results and a few retrospective studies reported the use of new anticoagulants and data on coagulation tests in relation to hemostatic complications. To decrease hemostatic complications in ECMO children, prospective multicenter studies are needed with clear bleeding and thrombotic definitions, and the best possible standardization of ECMO circuits used, anticoagulation protocols, and transfusion triggers.
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Prevalence of neonatal hyperbilirubinaemia and its association with glucose-6-phosphate dehydrogenase deficiency and blood-type incompatibility in sub-Saharan Africa: a systematic review and meta-analysis
Aynalem YA, Mulu GB, Akalu TY, Shiferaw WS
BMJ paediatrics open. 2020;4(1):e000750
Abstract
BACKGROUND Hyperbilirubinaemia is a silent cause of newborn disease and death worldwide. However, studies of the disease in sub-Saharan Africa are highly variable with respect to its prevalence. Hence, this study aimed to estimate the overall magnitude of neonatal hyperbilirubinaemia and its association with glucose-6-phosphate dehydrogenase (G6PD) deficiency and blood-type incompatibility in sub-Saharan Africa. METHODS PubMed, Scopus, Google Scholar and the Cochrane Review were systematically searched online to retrieve hyperbilirubinaemia-related articles. All observational studies reported the prevalence of hyperbilirubinaemia in sub-Saharan Africa were included for analysis and excluded if the study failed to determine the desired outcome. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed. Heterogeneity across the included studies was evaluated using the inconsistency index (I(2)). Subgroup and meta- regression analysis were also done. Publication bias was examined by funnel plot and the Egger's regression test. The random-effect model was fitted to estimate the pooled prevalence of neonatal hyperbilirubinaemia. The meta-analysis was performed using the STATA V.14 software. RESULTS A total of 30 486 studies were collected from the different databases and 10 articles were included for the final analysis. The overall magnitude of neonatal hyperbilirubinaemia was 28.08% (95% CI20.23 to 35.94, I(2)=83.2) in sub-Saharan Africa. Neonates with G6PD deficiency (OR 2.42, 95% CI 1.64 to 3.56, I(2)=37%) and neonates that had a blood type that was incompatible with their mother's (OR 3.3, (95% CI 1.96 to 5.72, I(2)=84%) were more likely to develop hyperbilirubinaemia. CONCLUSION The failure to prevent and screen G6PD deficiency and blood-type incompatibility with their mother's results in high burden of neonatal hyperbilirubinaemia in sub-Saharan Africa. Therefore, early identification and care strategies should be developed to the affected neonates with G6PD deficiency and blood-type incompatibility with their mother's to address long-term medical and scholastic damages among those exposed to hyperbilirubinaemia.
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Efficacy and toxicities of low-temperature plasma radiofrequency ablation for the treatment of laryngomalacia in neonates and infants: a prospective randomized controlled trial
Xu H, Chen F, Zheng Y, Li X
Annals of translational medicine. 2020;8(21):1366
Abstract
BACKGROUND Laryngomalacia is the most common cause of stridor in neonates and infants, and supraglottoplasty is the mainstay of surgical treatment. Although low-temperature plasma radiofrequency ablation (LTP-RFA) using coblation technology has been used for treating laryngomalacia, it is still lack of high-quality clinical evidence. Therefore, we conduct this prospective randomized study to clearly define the role of LTP-RFA for the treatment of laryngomalacia in neonates and infants. METHODS Between Jan 2017 and Dec 2019, a total of 89 children with laryngomalacia were included for analysis. All patients were initially stratified according to the severity of laryngomalacia. Patients with severe laryngomalacia were randomly assigned to receive LTP-RFA or traditionally surgical supraglottoplasty, while patients with moderate laryngomalacia were assigned to LTP-RFA or observation. The primary end point was the efficacy and toxicities of LTP-RFA by assessing the changes of clinical score and visual analogue scale (VAS) symptom score. The total score was the combination of clinical score with VAS score. RESULTS Of the 89 children, 40 children presented with severe laryngomalacia, and the remaining 49 children were diagnosed as moderate laryngomalacia. The median age was 68 days (range, 19 to 337 days). For children with severe laryngomalacia, our results showed that LTP-RFA treatment significantly reduced the operative time (5.55±1.66 vs. 18.7±5.31 min, P<0.001), length of hospital stay (6.71±1.15 vs. 7.95±1.55 days, P=0.008) and the amount of intraoperative hemorrhage (1.71±1.79 vs. 4.90±1.82, P<0.001) when compared to traditionally surgical supraglottoplasty, while the treatment efficacy was comparable between LTP-RFA and traditionally surgical supraglottoplasty in terms of changed total score (P=0.322), changed clinical score (P=0.135) and changed VAS symptom score (P=0.559). Additionally, for children with moderate laryngomalacia, LTP-RFA treatment significantly improved the symptom evaluated by total score (P<0.001), clinical score (P<0.001) and VAS symptom score (P<0.001) in comparison with the observation group. Post-operative pneumonia was observed in 10 patients. No surgical related death was reported. CONCLUSIONS The present study indicated that LTP-RFA was an effective treatment option for both severe and moderate laryngomalacia in neonates and infants with a low intraoperative complication. Long-term outcomes of LTP-RFA for laryngomalacia would be reported in further studies.