Description of a national, multi-center registry of patients with sickle cell disease and SARS-CoV-2 infection: Data from the Pediatric COVID-19 United States Registry.

Children with sickle cell disease (SCD) are at risk of complications from viral infections, including SARS-CoV-2. We present the clinical characteristics and outcomes of pediatric patients with SCD from the Pediatric COVID-19 United States Registry who developed acute COVID-19 due to SARS-CoV-2 infection (n = 259) or multisystem inflammatory syndrome in children (MIS-C; n = 4). Nearly half of hospitalized children with SCD and SARS-CoV-2 infection required supplemental oxygen, though children with SCD had fewer intensive care (ICU) admissions compared to the general pediatric and immunocompromised populations.

The Continued Rise of Venous Thromboembolism Across US Children's Hospitals.

Objectives: In 2009, a large multicenter study demonstrated that the rate of pediatric venous thromboembolism (VTE) across US children's hospitals had significantly increased from 2001 to 2007. The objective of this study was to evaluate the rate of pediatric VTE from 2008 to 2019 using similar methodology.

Methods: A retrospective cohort study using the Pediatric Health Information System (PHIS) database.

The ASH-ASPHO Choosing Wisely Campaign: 5 hematologic tests and treatments to question.

Choosing Wisely is a medical stewardship and quality-improvement initiative led by the American Board of Internal Medicine Foundation in collaboration with leading medical societies in the United States. The American Society of Hematology (ASH) has been an active participant in the Choosing Wisely project. In 2019, ASH and the American Society of Pediatric Hematology/Oncology (ASPHO) formed a joint task force to solicit, evaluate, and select items for a pediatric-focused Choosing Wisely list.

The ASH-ASPHO Choosing Wisely Campaign: 5 hematologic tests and treatments to question.

Choosing Wisely is a medical stewardship and quality-improvement initiative led by the American Board of Internal Medicine Foundation in collaboration with leading medical societies in the United States. The American Society of Hematology (ASH) has been an active participant in the Choosing Wisely project. In 2019, ASH and the American Society of Pediatric Hematology/Oncology (ASPHO) formed a joint task force to solicit, evaluate, and select items for a pediatric-focused Choosing Wisely list.

Utilization of an Electronic Medical Record-integrated Dashboard Improves Identification and Treatment of Anemia and Iron Deficiency in Pediatric Inflammatory Bowel Disease.

Background: Iron deficiency (ID) and anemia are one of the most common extraintestinal manifestations of inflammatory bowel disease (IBD), usually complicating the course both in ulcerative colitis and Crohn's disease. Despite their high prevalence and significant impact on patients, this particular aspect is still underestimated by clinicians. Although guidelines have been recently published to address this problem, these recommendations do not address pediatric specific concerns and do not provide guidance as to how implement these guidelines in clinical practice.

The impact of extended half-life factor concentrates on prophylaxis for severe hemophilia in the United States.

With licensure of extended half-life (EHL) factor products and the changing landscape of available hemophilia products, patients and providers have options for less treatment-intense prophylaxis. The impact of these products in clinical practice to date remains understudied. We aimed to quantify the use of EHL products in prophylaxis in the US using the ATHN-dataset, a database of 145 ATHN-affiliated hemophilia treatment centers (HTCs).

Utility of repeat testing in the evaluation for von Willebrand disease in pediatric patients.

Background: Von Willebrand disease (VWD) is the most common inherited bleeding disorder and is caused by quantitative and qualitative defects in von Willebrand factor (VWF). The laboratory diagnosis of VWD in pediatric patients is complicated by VWF interassay and intra-assay variability, stress-induced elevations in VWF levels, and a lack of significant bleeding history with which to correlate test results.

Objective: Guidelines recommend repeat testing in patients with a high suspicion of VWD and unclear laboratory assay results; however, no studies have evaluated the utility of repeat VWF testing in pediatric patients.

Primary thromboprophylaxis in hospitalized children: A multi-center retrospective analysis.

Background/objectives: Hospital acquired venous thromboembolism in children is associated with significant morbidity/mortality. Prevention strategies include sequential compression devices and prophylactic anticoagulation but these interventions carry risk and are poorly studied in children. Objectives were to evaluate primary thromboprophylaxis use in hospitalized children over time and the associated bleeding risk.

How I approach managing student athletes at risk for bleeding.

Frequently, pediatric hematologists need to provide guidance regarding sports participation for children with congenital coagulopathies, immune thrombocytopenia, and those receiving anticoagulation. Although sports participation has clear health and psychosocial benefits, it can be associated with harm secondary to bleeding from injury. Decision-making for sports involvement should be individualized, patient centered, and well informed.

Pediatric Hospital Acquired Venous Thromboembolism.

Pediatric hospital acquired venous thromboembolism (HA-VTE) is an increasing problem with an estimated increase from 5.3 events per 10,000 pediatric hospital admissions in the early 1990s to a current estimate of 30-58 events per 10,000 pediatric hospital admissions. Pediatric HA-VTE is associated with significant morbidity and mortality.

Utility of the immature platelet fraction in pediatric immune thrombocytopenia: Differentiating from bone marrow failure and predicting bleeding risk.

Background: Differentiating childhood immune thrombocytopenia (ITP) from other cause of thrombocytopenia remains a diagnosis of exclusion. Additionally factors that predict bleeding risk for those patients with ITP are currently not well understood. Previous small studies have suggested that immature platelet fraction (IPF) may differentiate ITP from other causes of thrombocytopenia and in combination with other factors may predict bleeding risk.

A survey of pediatric hematology/oncology specialists regarding management of central line associated venous thrombosis.

Central venous catheters (CVCs) account for the largest proportion of thrombotic events in pediatric patients. Questions remain regarding adequate treatment and prevention methods. We surveyed pediatric hematology/oncology specialists, using hypothetical cases to assess management strategies for acute CVC thrombosis and secondary prevention.

Antithrombin Concentrate Use in Pediatric Extracorporeal Membrane Oxygenation: A Multicenter Cohort Study.

Objective: To describe antithrombin concentrate use and to compare thrombotic and hemorrhagic outcomes throughout the hospital stay in pediatric subjects who received extracorporeal membrane oxygenation in a Pediatric Health Information System-participating children's hospital.

Design: Retrospective, multi-center, cohort study.

Setting: Forty-three free-standing children's hospitals participating in Pediatric Health Information System.

Multicenter Cohort Study Comparing U.S. Management of Inpatient Pediatric Immune Thrombocytopenia to Current Treatment Guidelines.

Background: Recent pediatric immune thrombocytopenia (ITP) guidelines have significantly altered and are encouraging an observational approach for patients without significant bleeding regardless of their platelet count.

Procedure: This retrospective multicenter cohort study utilized the Pediatric Health Information Systems (PHIS) administrative database. Subjects were 6 months to 18 years of age, admitted to a PHIS hospital between January 1, 2008 and September 30, 2014, with a primary diagnosis code for ITP.

Spontaneous Subclavian Vein Thrombosis in a Healthy Adolescent Cheerleader: A Case of Paget-Schroetter Syndrome.

We present the case of a healthy 13-year-old female adolescent who developed acute progressive swelling and pain in her right upper extremity that was secondary to an acute deep venous thrombosis of her right subclavian vein. Dynamic imaging revealed subclavian vein compression at the junction of the first rib and proximal third of the clavicle consistent with Paget-Schroetter syndrome, also known as effort-related thrombosis. The compressive etiology of her thrombus was most likely related to her cheerleading activity, in which she served as the pyramid base.

Validation Study of the Composite Score to Identify Von Willebrand Disease in Children.

Background: The diagnosis of type 1 von Willebrand disease (VWD) presents a diagnostic challenge in children. In fact, 25% or more of children with VWD may be diagnosed only after they experience postoperative bleeding. We previously described a 4-variable composite score that has 92.

Hematologic manifestations of systemic disease (including iron deficiency, anemia of inflammation and DIC).

A complete blood cell count (CBC) is a frequent test sent to aid in the diagnostic evaluation of ill patients. Not uncommonly hematologic abnormalities may be the first sign of an underlying systemic disorder. The astute clinician needs to understand how systemic disease can affect the CBC to direct further diagnostic investigations.

Thrombotic events in critically ill children with myocarditis.

Background: Children with myocarditis have multiple risk factors for thrombotic events, yet the role of antithrombotic therapy is unclear in this population. We hypothesised that thrombotic events in critically ill children with myocarditis are common and that children with myocarditis are at higher risk for thrombotic events than children with non-inflammatory dilated cardiomyopathy.

Methods: This is a retrospective chart review of all children presenting to a single centre cardiac intensive care unit with myocarditis from 1995 to 2008.

Haemorrhagic stroke in term and late preterm neonates.

Objective: Few data regarding causes and outcomes of haemorrhagic stroke (HS) in term neonates are available. We characterised risk factors, mechanism and short-term outcomes in term and late preterm neonates with acute HS.

Design: Prospective cohort.

Antithrombin concentrate use in children: a multicenter cohort study.

Objective: To describe the off-label use of antithrombin concentrate in tertiary care pediatric hospitals across the US.

Study Design: This is a retrospective, multicenter, cohort study of 4210 admissions of children younger than 18 years of age who received antithrombin concentrate between 2002 and 2011 within the Pediatric Health Information System administrative database. An on-label admission was defined as an admission with an International Classification of Diseases diagnostic code for a primary hypercoagulable state; admissions without this code were classified as off-label.

Off-label recombinant factor VIIa use and thrombosis in children: a multi-center cohort study.

Objective: To describe the off-label use of recombinant factor VIIa (rFVIIa) in tertiary care pediatric hospitals across the United States and to assess thrombotic events.

Study Design: A retrospective multi-center cohort study using the Pediatric Health Information System administrative database. Children 18 years of age or younger who received rFVIIa between 2000 and 2007 were included.

The clinical management of hemophilia and head trauma: a survey of current clinical practice among pediatric hematology/oncology physicians.

Background: Determining the appropriate evaluation for a pediatric patient with hemophilia and head trauma is a diagnostic challenge with no neuroimaging guidelines and limited clinical evidence to direct care.

Procedure: A questionnaire, with two case scenarios, was emailed to members of the American Society of Pediatric Hematology/Oncology. The case scenarios involved asymptomatic toddlers with severe hemophilia who had either fallen from a height (case 1) or from standing (case 2).