These ATS-sponsored technical standards describe the equipment, personnel, competencies, and special procedures associated with FAE in children.

Bronchiolitis obliterans (BO) in children is a relatively rare diagnosis. The increase in lung and bone marrow transplantation in children, however, has led to a heightened interest in BO, as this is one of the important complications of those procedures. This article will discuss BO as an entity that can follow any of several illnesses or toxic exposures, in addition to following allogeneic lung or bone marrow transplantation. The complex and incompletely understood pathology, pathogenesis, and molecular pathology involved in BO remain the subject of ongoing investigations. As the prognosis for BO is uncertain and treatment is often unsuccessful, the continued need for the recognition of surrogate markers for BO in patients at risk and the development of better forms of therapy are paramount. This review will describe our current understanding of BO, and will call attention to those research areas that require continuing efforts in order to prevent or treat this entity.

Background: Advanced cystic fibrosis lung disease (ACFLD) is common, is associated with reduced quality of life, and remains the most frequent cause of death in individuals with cystic fibrosis (CF). These consensus guidelines provide recommendations to the CF community on management of both common and unique issues that arise when individuals reach a state of ACFLD. Methods:The CF Foundation assembled a multidisciplinary expert panel consisting of three workgroups: Pulmonary management; Management of comorbid conditions; Symptom management and psychosocial issues. Topics were excluded if the management considerations did not differ in ACFLD from in the overall CF population or if already addressed in other published guidelines. Recommendations were based on a systematic literature review combined with expert opinion when appropriate. Results:The committee formulated twenty-three recommendation statements specific to ACFLD that address the definition of ACFLD, pulmonary and intensive care unit management, management of selected comorbidities, symptom control, and psychosocial issues.

Elevated TRV, a surrogate marker for PHTN, occurs in children with SCD and is associated with low hemoglobin, elevated reticulocyte count, and cerebral vasculopathy. Appropriate screening by echocardiography can lead to detection and treatment that may reduce TRV and potentially reverse the disease process, prevent the increased morbidity and mortality associated with PHTN.

NAC recipients maintained their lung function while placebo recipients declined (24 week FEV1 treatment effect=150 mL, p<0.02). However no effect on HNE activity and other selected biomarkers of neutrophilic inflammation were detected. Further studies on mechanism and clinical outcomes are warranted.

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Risk factors for PFI include Grade A2 rejection, repeated acute rejection, CMV-positive donor, tacrolimus-based regimen and pre-transplant colonization.

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