Diagnosis and treatment of pediatric acquired aplastic anemia (AAA): An initial survey of the North American Pediatric Aplastic Anemia Consortium (NAPAAC)

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/106711/1/pbc24875.pd

Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update

In 2010, the congenital diaphragmatic hernia (CDH) EURO Consortium published a standardized neonatal treatment protocol. Five years later, the number of participating centers has been raised from 13 to 22. In this article the relevant literature is updated, and consensus has been reached between the members of the CDH EURO Consortium. Key updated recommendations are: (1) planned delivery after a gestational age of 39 weeks in a high-volume tertiary center; (2) neuromuscular blocking agents to be avoided during initial treatment in the delivery room; (3) adapt treatment to reach a preductal saturation of between 80 and 95% and postductal saturation >70%; (4) target PaCO2 to be between 50 and 70 mm Hg; (5) conventional mechanical ventilation to be the optimal initial ventilation strategy, and (6) intravenous sildenafil to be considered in CDH patients with severe pulmonary hypertension. This article represents the current opinion of all consortium members in Europe for the optimal neonatal treatment of CDH

Adenovirus-Associated Virus Vector-Mediated Gene Transfer in Hemophilia B

NIHR (RP-PG-0310-1001), the Medical Research Council, the Katharine Dormandy Trust, the U.K. Department of Health, NHS Blood and Transplant, the NIHR Biomedical Research Centers (to University College London Hospital and University College London), the ASSISI Foundation of Memphis, the American Lebanese Syrian Associated Charities, the Howard Hughes Medical Institute, the National Heart, Lung, and Blood Institute (HL094396), the Royal Free Hospital Charity Special Trustees Fund 35, the Royal Free Hospital NHS Trust, and St. Jude Children’s Research Hospita

The impact of quality and duration of enoxaparin therapy on recurrent venous thrombosis in children

Background: Venous thromboembolism (VTE) and recurrent venous thromboembolism (rVTE) are rare, but significant problems in pediatrics. Current recommendations for anticoagulant therapy arise from adult literature, and there is little data on clinical outcomes following therapeutic low-molecular-weight heparin in children. Method: All patients \u3c19 years of age that were diagnosed with a VTE or right atrial thrombus via standard imaging methods at St. Jude Children\u27s Research Hospital were retrospectively identified from January 2004 through August 2008. Demographic characteristics, coexisting clinic conditions, description of anticoagulant therapy, and record of rVTE were chronicled following a comprehensive chart review. Descriptive statistics of clinical characteristics and anticoagulation are presented. Results: Venous thrombosis was identified in 149 children with 21% (31/149) developing a rVTE. Coexisting clinical conditions were identified in 93% of children at initial diagnosis with 48% (71/149) of patients having a coexisting malignancy. Seventy-seven percent (114/149) of children received anticoagulant therapy with UFH (10/114) or enoxaparin (104/114). Neither duration of enoxaparin therapy (\u3e6, 3-6, \u3c3 months) (P=0.61), nor quality of therapy (≥75% of time on anticoagulation spent with an anti-FXa of 0.5-1.0U/ml) (P=1.0) were found to be protective against rVTE. Conclusion: Anticoagulation with enoxaparin based on adult literature may be suboptimal in preventing rVTE in pediatric populations. Future prospective randomized controlled trials in pediatrics using clinical outcomes with anticoagulant therapy are urgently needed. © 2011 Wiley Periodicals, Inc