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Background and objectives Shared decision making (SDM) prior to non-urgent tracheostomy in a child with chronic respiratory failure (CRF) is often recommended, but has proven challenging to implement in practice. We hypothesize that utilization of the microsystem model for analysis of the complex ecosystem in which SDM occurs will yield insights that enable formation of a reproducible, measurable SDM process. Methods Retrospective chart review of a case series of children with CRF in whom a SDM process was pursued. The process included a palliative care consult, a validated decision aid and 12 key questions designed to elucidate information integral to an informed decision. Investigators reviewed a single hospital admission for each child, focusing on the 3 core elements of a medical microsystem- the patient, the providers, and information. Results 29 patients who met inclusion criteria ranged in age from 0 to 19.5 years (median 1.7) and remained in the hospital from 10 to 316 days (median 38). Patients were medically complex with multiple and varied respiratory diagnoses, multiple and varied comorbidities, and varying psychosocial environments. 14/29 children received tracheostomies. Each child encountered a mean of 6.2 medical specialties, 1.9 surgical specialties and 8.5 non-physician led services. Answers to 12 key questions were not documented systematically and often not found in the electronic medical record (EMR). Conclusion A unique SDM microsystem is formed around each child but not optimally utilized. Explicit recognition of these microsystems would enable team formation and an SDM process comprised of measurable steps and communication patterns. This article is protected by copyright. All rights reserved.

Children with chronic invasive ventilator dependence living at home are a diverse group of children with special health care needs. Medical oversight, equipment management, and community resources vary widely. There are no clinical practice guidelines available to health care professionals for the safe hospital discharge and home management of these complex children.To develop evidence-based clinical practice guidelines for the hospital discharge and home/community management of children requiring chronic invasive ventilation.The Pediatric Assembly of the American Thoracic Society assembled an interdisciplinary workgroup with expertise in the care of children requiring chronic invasive ventilation. The experts developed four questions of clinical importance and used an evidence-based strategy to identify relevant medical evidence. Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology was used to formulate and grade recommendations.Clinical practice recommendations for the management of children with chronic ventilator dependence at home are provided, and the evidence supporting each recommendation is discussed.Collaborative generalist and subspecialist comanagement is the Medical Home model most likely to be successful for the care of children requiring chronic invasive ventilation. Standardized hospital discharge criteria are suggested. An awake, trained caregiver should be present at all times, and at least two family caregivers should be trained specifically for the child's care. Standardized equipment for monitoring, emergency preparedness, and airway clearance are outlined. The recommendations presented are based on the current evidence and expert opinion and will require an update as new evidence and/or technologies become available.

The transition from a fee-for-service payment system to value-based payment system gained momentum in the US in 2010 with the passage of the Affordable Care Act and continues to progress rapidly. Market research estimates that value-based payment models will surpass fee-for-service by 2020. This change offers both great opportunity and great risk to the medical care of the heterogeneous populations of children with chronic respiratory disease. The fee-for-service model has driven the emergence of a healthcare delivery infrastructure markedly misaligned with the medical needs of children with chronic respiratory disease. A change to value-based payment models offers the opportunity to create systems better aligned with the complex and varied care needs of these children. However, rapid change without input from the relevant stakeholders could yield an infrastructure even more misaligned with the needs of children with chronic respiratory disease than the current one and threaten access to high quality medical care for these populations. Through the lens offered by three fictional case studies, this review: (1) illustrates current and evolving payment models; (2) describes limitations of these payment models; and (3) suggests a novel way to envision and evaluate value-based payment models for children with chronic respiratory disease.

Rationale: We identified a 6-year-old girl with pulmonary alveolar proteinosis (PAP), impaired granulocyte-macrophage colony–stimulating factor (GM-CSF) receptor function, and increased GM-CSF. Objectives: Increased serum GM-CSF may be useful to identify individuals with PAP caused by GM-CSF receptor dysfunction. Methods: We screened 187 patients referred to us for measurement of GM-CSF autoantibodies to diagnose autoimmune PAP. Five were children with PAP and increased serum GM-CSF but without GM-CSF autoantibodies or any disease causing secondary PAP; all were studied with family members, subsequently identified patients, and controls. Measurement and Main Results: Eight children (seven female, one male) were identified with PAP caused by recessive CSF2RA mutations. Six presented with progressive dyspnea of insidious onset at 4.8 ± 1.6 years and two were asymptomatic at ages 5 and 8 years. Radiologic and histopathologic manifestations were similar to those of autoimmune PAP. Molecular analysis demonstrated that GM-CSF signaling was absent in six and severely reduced in two patients. The GM-CSF receptor β chain was detected in all patients, whereas the α chain was absent in six and abnormal in two, paralleling the GM-CSF signaling defects. Genetic analysis revealed multiple distinct CSF2RA abnormalities, including missense, duplication, frameshift, and nonsense mutations; exon and gene deletion; and cryptic alternative splicing. All symptomatic patients responded well to whole-lung lavage therapy. Conclusions: CSF2RA mutations cause a genetic form of PAP presenting as insidious, progressive dyspnea in children that can be diagnosed by a combination of characteristic radiologic findings and blood tests and treated successfully by whole-lung lavage.

Gastrin-releasing peptide (GRP) is produced by pulmonary neuroendocrine cells (PNECs), with highest numbers of GRP-positive cells present in fetal lung. Normally GRP-positive PNECs are relatively infrequent after birth, but PNEC hyperplasia is frequently associated with chronic lung diseases. To address the hypothesis that GRP mediates chronic lung injury, we present the cumulative evidence implicating GRP in bronchopulmonary dysplasia (BPD), the chronic lung disease of premature infants who survive acute respiratory distress syndrome. The availability of well-characterized animal models of BPD was a critical tool for demonstrating that GRP plays a direct role in the early pathogenesis of this disease. Potential mechanisms by which GRP contributes to injury are analyzed, with the main focus on innate immunity. Autoreactive T cells may contribute to lung injury late in the course of disease. A working model is proposed with GRP triggering multiple cell types in both the innate and adaptive immune systems, promoting cascades culminating in chronic lung disease. These observations represent a paradigm shift in the understanding of the early pathogenesis of BPD, and suggest that GRP blockade could be a novel treatment to prevent this lung disease in premature infants.

A 2.6-kg infant was born at 38 weeks’ gestation to a 16-year-old G1P0 woman. The prenatal history included maternal Chlamydia infection. The baby was delivered via emergent cesarian section because of nonreassuring fetal heart tones. His Apgar scores were 7 and 9 at 1 and 5 minutes, respectively. He exhibited tachypnea and hypoxia at birth, with a respiratory rate of 80 to 100 breaths/min, grunting, chest retractions, and coarse breath sounds. Other than a mild systolic ejection murmur, the remainder of the physical examination findings were normal. The baby's A-a gradient (a measure of the difference in the partial pressure of oxygen in the alveolar spaces and the arterial blood) was elevated, initially 473 mm Hg on 100% Fio2. Chest radiographs showed no signs of acute air space disease for the first several postnatal weeks. Over the next several months, the infant continues to have a respiratory rate of 60 to 90 breaths/min, with increased work of breathing, gradually requiring increases in supplemental oxygen to achieve an Spo2 in the mid-90s. Chest computed tomography (CT) scan performed at 2 weeks of age showed attenuation of the pulmonary vasculature and hyperinflation of the lungs. Although motion artifact limited the evaluation, the chest CT showed no focal lung opacities and no other anatomic defects. Repeated echocardiography revealed a small patent foramen ovale (left-to-right shunt) and normal ventricular size and function. Multiple therapies are tried without obvious …

Trauma series radiographs (ie, lateral cervical spine, anteroposterior chest, and anteroposterior pelvis) are routinely recommended for victims of multiple trauma. However, the utility of the chest and pelvic radiographs has never been adequately evaluated. The purpose of this study is to determine whether clinical findings alone predict the results of these radiographs.The pediatric radiology department at the Jacobi Medical Center, Albert Einstein College of Medicine, Bronx, New York, maintains a log of all patients who have undergone a complete trauma series. From this group, we selected all patients younger than 2 years with a Glasgow Coma Scale greater than 14 points. The patients' emergency department charts were reviewed to note the presence or absence of localizing signs and symptoms referable to the chest and pelvis, including chest or pelvic pain, tenderness, ecchymoses or abrasions, shortness of breath or other respiratory symptoms, hematuria or difficulty voiding, and abdominal distention.Sixteen of the 91 subjects (18%) had localizing chest findings. Two of these patients had positive chest radiographs, whereas the 75 patients without localizing chest findings had no positive chest radiographs (P0.03). Thirty-two of 91 subjects (35%) had localizing pelvic signs. Five of these patients had positive pelvic radiographs, whereas the 56 patients without localizing pelvic signs had no positive pelvic radiographs (P0.01). The negative predictive value of localizing signs and symptoms was 100% for both chest and pelvic radiographs.These data suggest that if an adequate examination can be performed, trauma series radiographs can be ordered selectively, based on the patient's clinical findings.

Using a mouse model of neonatal respiratory distress syndrome (RDS), we demonstrate a central role for macrophage migration inhibitory factor (MIF) in lung maturation at the developmental stage when human neonates are most susceptible to RDS. We prematurely delivered mouse pups at embryonic day 18, during the early saccular stage of pulmonary development. Only 8% of the prematurely delivered pups genetically deficient in MIF survived 8 h vs 75% of wild-type controls (p < 0.001). This phenotype was corrected when pups of all genotypes were bred from dams heterozygote for MIF deficiency. Local production of MIF in the lung increased at embryonic day 18, continued until full-term at embryonic day 19.5, and decreased in adulthood, thus coinciding with this developmental window. The lungs of pups genetically deficient in MIF were less mature upon histological evaluation, and demonstrated lower levels of vascular endothelial growth factor and corticosterone – two factors that promote fetal lung maturation. In vitro studies support a role for MIF in surfactant production by pulmonary epithelial cells. In a cohort of human neonates with RDS, higher intrapulmonary MIF levels were associated with a lower likelihood of developing bronchopulmonary dysplasia, a sequelae of RDS (p < 0.03). This study demonstrates for the first time a role for MIF in lung maturation, and supports a protective role for MIF in newborn lung disease.