International Consensus Document (ICON): Common Variable Immunodeficiency Disorders.

The International Collaboration in Asthma, Allergy and Immunology initiated an international coalition among the American Academy of Allergy, Asthma & Immunology; the European Academy of Allergy and Clinical Immunology; the World Allergy Organization; and the American College of Allergy, Asthma & Immunology on common variable immunodeficiency. An author group was formed and then divided into individual committees. Within the committee, teams of authors were subgrouped to generate content for specific sections of the document.

A Candida albicans Strain Expressing Mammalian Interleukin-17A Results in Early Control of Fungal Growth during Disseminated Infection.

Candida albicans is normally a commensal fungus of the human mucosae and skin, but it causes life-threatening systemic infections in hospital settings in the face of predisposing conditions, such as indwelling catheters, abdominal surgery, or antibiotic use. Immunity to C. albicans involves various immune parameters, but the cytokine interleukin-17A (IL-17A) (also known as IL-17) has emerged as a centrally important mediator of immune defense against both mucosal and systemic candidiasis.

Integrin binding by Borrelia burgdorferi P66 facilitates dissemination but is not required for infectivity.

P66, a Borrelia burgdorferi surface protein with porin and integrin-binding activities, is essential for murine infection. The role of P66 integrin-binding activity in B. burgdorferi infection was investigated and found to affect transendothelial migration.

Infant with nasolacrimal sinonasal myxoma: Diffusion MRI features.

We report the imaging features of a rare sinonasal myxoma situated over the right nasolacrimal duct in a 5-month-old male. We emphasize the importance of including sinonasal myxomas in the list of differential diagnostic possibilities when encountering a nasolacrimal gland mass in an infant, and describe the CT and MRI characteristics of this rare entity.

Pediatric surgeons' attitudes toward regionalization of neonatal surgical care.

Background/purpose: Research has suggested that high-risk pediatric surgical patients have better outcomes when treated in resource-rich children's environments. Surgical neonates are a particularly high-risk population and some suggest that regionalization might be a strategy to improve clinical outcomes in neonatal surgical patients. We conducted a national survey of pediatric surgeons in the United States to explore their attitudes toward regionalization of neonatal surgical care.

Technical performance score is associated with outcomes after the Norwood procedure.

Objectives: The technical performance score (TPS) has been reported in a single center study to predict the outcomes after congenital cardiac surgery. We sought to determine the association of the TPS with outcomes in patients undergoing the Norwood procedure in the Single Ventricle Reconstruction trial.

Methods: We calculated the TPS (class 1, optimal; class 2, adequate; class 3, inadequate) according to the predischarge echocardiograms analyzed in a core laboratory and unplanned reinterventions that occurred before discharge from the Norwood hospitalization.

Factors associated with neurodevelopment for children with single ventricle lesions.

Objective: To measure neurodevelopment at 3 years of age in children with single right-ventricle anomalies and to assess its relationship to Norwood shunt type, neurodevelopment at 14 months of age, and patient and medical factors.

Study Design: All subjects in the Single Ventricle Reconstruction Trial who were alive without cardiac transplant were eligible for inclusion. The Ages and Stages Questionnaire (ASQ, n = 203) and other measures of behavior and quality of life were completed at age 3 years.

Higher doses of low-molecular-weight heparin (enoxaparin) are needed to achieve target anti-Xa concentrations in critically ill children*.

Objectives: To demonstrate that low-molecular-weight heparin (enoxaparin) can be used in critically ill pediatric patients to achieve target anti-factor Xa concentrations and determine appropriate dosing corrected for age and illness severity.

Design: Retrospective cohort study.

Setting: Single tertiary level PICU.

Transplantation-free survival and interventions at 3 years in the single ventricle reconstruction trial.

Background: In the Single Ventricle Reconstruction (SVR) trial, 1-year transplantation-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a modified Blalock-Taussig shunt (MBTS). At 3 years, we compared transplantation-free survival, echocardiographic right ventricular ejection fraction, and unplanned interventions in the treatment groups.

Methods And Results: Vital status and medical history were ascertained from annual medical records, death indexes, and phone interviews.

The pain frequency-severity-duration scale as a measure of pain: preliminary validation in a pediatric chronic pain sample.

Typically, pain is measured by intensity and sensory characteristics. Although intensity is one of the most common dimensions of pain assessment, it has been suggested that measuring pain intensity in isolation is only capturing part of the pain experience and may not lead to an accurate measurement of how pain impacts a child's daily functioning. The current study aimed to develop a measure that would capture pain intensity along with frequency and duration in a clinical sample of youth diagnosed with chronic pain.

Bronchogenic cyst in the intradiaphragmatic location.

Bronchogenic cysts are congenital foregut malformations thought to develop due to abnormal budding of tracheal diverticulumand proximal bronchial structures during embryologic development. The cyst is lined by ciliated pseudostratified columnar epithelium and the wall contains cartilage and layers of smooth muscle. These lesions most commonly are seen in the mediastinum, lung, or pleural spaces.

Changes in cerebral oxygen saturation correlate with S100B in infants undergoing cardiac surgery with cardiopulmonary bypass.

Objectives: The relationship of cerebral saturation measured by near-infrared spectroscopy with serum biomarker of brain injury S100B was investigated in infants undergoing cardiac surgery with cardiopulmonary bypass.

Design: Prospective cohort study.

Setting: Single-center children's hospital.

In utero diagnosis of long QT syndrome by magnetocardiography.

Background: The electrophysiology of long QT syndrome (LQTS) in utero is virtually unstudied. Our goal here was to evaluate the efficacy of fetal magnetocardiography (fMCG) for diagnosis and prognosis of fetuses at risk of LQTS.

Methods And Results: We reviewed the pre/postnatal medical records of 30 fetuses referred for fMCG because of a family history of LQTS (n=17); neonatal/childhood sudden cardiac death (n=3), or presentation of prenatal LQTS rhythms (n=12): 2° atrioventricular block, ventricular tachycardia, heart rate < 3(rd) percentile.

Single-ventricle infant home monitoring programs: outcomes and impact.

Purpose Of Review: Patients with single-ventricle, shunt-dependent physiology are at increased risk for interstage death due to the inherent instability of parallel circulation. Enhanced surveillance and early identification of deteriorating physiology via interstage home monitoring result in significant reduction in mortality. These programs are an important focus of improving outcomes for patients with single-ventricle heart disease.

Interstage mortality after the Norwood procedure: Results of the multicenter Single Ventricle Reconstruction trial.

Objective: For infants with single ventricle malformations undergoing staged repair, interstage mortality is reported at 2% to 20%. The Single Ventricle Reconstruction trial randomized subjects with a single morphologic right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt (MBTS) or a right ventricle-to-pulmonary artery shunt (RVPAS). The aim of this analysis was to explore the associations of interstage mortality and shunt type, and demographic, anatomic, and perioperative factors.

Effectiveness of clinical guidelines for deep vein thrombosis prophylaxis in reducing the incidence of venous thromboembolism in critically ill children after trauma.

Background: Historically, 6% of critically ill children developed clinically apparent venous thromboembolism (VTE) after trauma at our Level I pediatric trauma center. We hypothesized that implementation of clinical guidelines for thrombosis prophylaxis incorporating both VTE risk and bleeding risk would reduce VTE incidence without increased bleeding.

Methods: VTE, both clinically apparent and those only detected by guideline-directed screening, were prospectively identified for all children admitted to the intensive care unit after trauma during three time periods: preimplementation of guidelines for VTE thromboprophylaxis (PRE; April 1, 2006-June 30, 2007), the intervening period (ROLL OUT; July 1, 2007-November 4, 2008), and postguideline implementation (POST; November 5, 2008-June 1, 2010).

Feeding, growth, nutrition, and optimal interstage surveillance for infants with hypoplastic left heart syndrome.

Improvement in operative survival of patients with hypoplastic left heart syndrome has led to increasing emphasis on prevention of interstage mortality. Many centres have improved interstage results through programmes of home monitoring following discharge after the Norwood (Stage 1) operation. Experience with heightened interstage surveillance has identified failure to thrive during infancy as a modifiable risk factor for this population, one that has been linked to concerning outcomes at subsequent palliative surgeries.

Incidence and risk factors for venous thromboembolism in critically ill children after trauma.

Background: Venous thromboembolism (VTE) causes major morbidity in adults after trauma, occurring in up to 50% of patients without prophylaxis. The incidence of VTE after trauma is lower in children. No study has measured the incidence of and risk factors for VTE in critically ill children after trauma.

Gastrointestinal complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease.

A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention.

Medical management of the failing Fontan.

The Fontan operation accomplishes complete separation of systemic venous blood from pulmonary venous circulation in patients with single ventricle anatomy. Operative survival since the first description of the Fontan operation is excellent in the current era through modifications in surgical techniques, identification of patient-specific risk factors, and advances in postoperative care. Improved early outcomes have also resulted in a decline in late mortality for patients who have undergone staged palliation with the Fontan operation.

Prevalence of heparin-dependent platelet antibodies in children after cardiopulmonary bypass.

Objective: To determine the prevalence of heparin-dependent platelet antibodies (HDPA) in children requiring heparin for >5 days after cardiopulmonary bypass surgery.

Design: Prospective, observational study.

Setting: Tertiary care pediatric intensive care unit.

Detection of Pseudomonas aeruginosa type III antibodies in children with tracheostomies.

Pseudomonas aeruginosa is often cultured from the airways of children with tracheostomies. P. aeruginosa produces exotoxin A (ETA) and type III cytotoxins.