Pediatric unilobar resection in primary ciliary dyskinesia.

Primary ciliary dyskinesia (PCD) causes chronic infections and progressive bronchiectasis that can lead to severe lung disease. Because there are no cures or regenerative therapy options for PCD, treatment of severe lung disease in PCD is focused on managing symptoms, including aggressive administration of antibiotics and diligent airway clearance. The Genetic Disorders of Mucociliary Clearance Consortium (GDMCC) does not recommend routine lobectomy, reserving its use for "rare cases of PCD with severe, localized bronchiectasis" and warns that a lobectomy should be treated with caution.

Opportunities on the road to value-based payment for children with chronic respiratory disease.

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.

Standardizing Care and Parental Training to Improve Training Duration, Referral Frequency, and Length of Stay: Our Quality Improvement Project Experience.

Objectives: At our institution, there is a six bed Pediatric Respiratory Care Unit for technology dependent infants and children with a tracheostomy tube. A lack of consistency in patient care and parent/guardian education prompted our group to critically evaluate the services we provided by revisiting our teaching protocol and instituting a new model of care in the Unit. The aims of this quality improvement (QI) project were to standardize care and skills proficiency training to parents of infants with a tracheostomy tube in preparation for discharge to home.

Risk factors for tracheostomy requirement in extremely low birth weight infants.

Aim: To identify variables that affect the risk of tracheostomy in a population of extremely low birth weight (ELBW) infants.

Methods: A retrospective matched case-control study was conducted. ELBW infants with a tracheostomy were compared with controls without tracheostomy.

Official American Thoracic Society Clinical Practice Guidelines: Diagnostic Evaluation of Infants with Recurrent or Persistent Wheezing.

Background: Infantile wheezing is a common problem, but there are no guidelines for the evaluation of infants with recurrent or persistent wheezing that is not relieved or prevented by standard therapies.

Methods: An American Thoracic Society-sanctioned guideline development committee selected clinical questions related to uncertainties or controversies in the diagnostic evaluation of wheezing infants. Members of the committee conducted pragmatic evidence syntheses, which followed the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach.

Biochemical Screening for Pulmonary Hypertension in Preterm Infants with Bronchopulmonary Dysplasia.

Background: Pulmonary hypertension (PH) in infants with bronchopulmonary dysplasia (BPD) is associated with increased morbidity and mortality. Elevated levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) and decreased levels of amino acid precursors of nitric oxide (NO) have been associated with PH, but have not been studied in infants with PH secondary to BPD.

Objective: The aim of this study was to identify a biochemical marker for PH in infants with BPD.

Association between right ventricular dysfunction and restrictive lung disease in childhood cancer survivors as measured by quantitative echocardiography.

Background: Restrictive lung disease is a complication in childhood cancer survivors who received lung-toxic chemotherapy and/or thoracic radiation. Left ventricular dysfunction is documented in these survivors, but less is known about right ventricular (RV) function. Quantitative echocardiography may help detect subclinical RV dysfunction.

Effect of vitamin D-binding protein genotype on the development of asthma in children.

Background: Potential vitamin D-related influences on inflammatory diseases such as asthma are controversial, including the suggestion that vitamin D insufficiency is associated with increased asthma morbidity. Vitamin D-binding protein transports vitamin D metabolites in the circulation. Single nucleotide polymorphisms in the GC gene encoding vitamin D-binding protein are associated with circulating vitamin D metabolite levels in healthy infants and toddlers.

Heparin binding VEGF isoforms attenuate hyperoxic embryonic lung growth retardation via a FLK1-neuropilin-1-PKC dependent pathway.

Background: Previous work in our laboratory demonstrated that hyperoxia suppressed the expression of vascular endothelial growth factor (VEGF) by the embryonic lung, leading to increased epithelial cell apoptosis and failure of explant airway growth and branching that was rescued by the addition of Vegf165. The aims of this study were to determine protective pathways by which VEGF isoforms attenuate hyperoxic lung growth retardation and to identify the target cell for VEGF action.

Methods: Timed pregnant CD-1 or fetal liver kinase (FLK1)-eGFP lung explants cultured in 3% or 50% oxygen were treated ± Vegf121, VEGF164/Vegf165 or VEGF188 in the presence or absence of anti-rat neuropilin-1 (NRP1) antibody or GO6983 (protein kinase C (PKC) pan-inhibitor) and lung growth and branching quantified.

DOR activation inhibits anoxic/ischemic Na+ influx through Na+ channels via PKC mechanisms in the cortex.

Activation of delta-opioid receptors (DOR) is neuroprotective against hypoxic/ischemic injury in the cortex, which is at least partially related to its action against hypoxic/ischemic disruption of ionic homeostasis that triggers neuronal injury. Na(+) influx through TTX-sensitive voltage-gated Na(+) channels may be a main mechanism for hypoxia-induced disruption of K(+) homeostasis, with DOR activation attenuating the disruption of ionic homeostasis by targeting voltage-gated Na(+) channels. In the present study we examined the role of DOR in the regulation of Na(+) influx in anoxia and simulated ischemia (oxygen-glucose deprivation) as well as the effect of DOR activation on the Na(+) influx induced by a Na(+) channel opener without anoxic/ischemic stress and explored a potential PKC mechanism underlying the DOR action.

Unusual pulmonary findings in mucolipidosis II.

We report undescribed pulmonary findings in a child with mucolipidosis II (ML-II). Children with ML-II bear significant pulmonary morbidity that may include extensive pulmonary fibrosis, persistent hemosiderosis as well as pulmonary airway excrescences as they reach preschool age.

Accuracy of a computerized clinical decision-support system for asthma assessment and management.

Objective: To evaluate the accuracy of a computerized clinical decision-support system (CDSS) designed to support assessment and management of pediatric asthma in a subspecialty clinic.

Design: Cohort study of all asthma visits to pediatric pulmonology from January to December, 2009.

Measurements: CDSS and physician assessments of asthma severity, control, and treatment step.

Secure web messaging in a pediatric chronic care clinic: a slow takeoff of "kids' airmail".

Background: Although e-mail may be an efficient clinician-patient communication tool, standard e-mail is not adequately secure to meet Health Insurance Portability and Accountability Act (HIPAA) guidelines. For this reason, firewall-secured electronic messaging systems have been developed for use in health care. Impact and usability of these secure systems have not been broadly assessed.

Utilization of a second caregiver in the care of a child with a tracheostomy in the homecare setting.

Objective: To safely discharge a child with a tracheostomy tube to home, we require two legal guardians/parents to complete a special training program. However, there are times when two parents/guardians are unavailable or unwilling to be trained. Therefore, the purpose of this study was to evaluate the role of the second caregiver of a child with a tracheostomy tube in the home setting.

Pulmonary squamous cell carcinoma associated with repaired congenital tracheoesophageal fistula and esophageal atresia.

We report a 19-year-old man with pulmonary squamous cell carcinoma (SCC) who had a history of vertebral, anal, cardiac, tracheal, esophageal, renal, and radial limb defects (VACTERL) association and tracheoesophageal fistula (TEF) + esophageal atresia (EA) repair as an infant. Children that undergo TEF + EA repair may have an increased risk for developing cancer as they reach adulthood.

Predictors of asthma-related pediatric emergency department visits and hospitalizations.

Objective: Asthma is a leading cause of emergency department visits and hospitalizations for children in the United States. As part of a larger study, the purpose of this analysis was to determine which variables were most effective at predicting subsequent pediatric asthma-related emergency department visits and hospitalizations.

Methods: A retrospective, descriptive study was conducted.

A novel presentation of diffuse lung disease caused by congenital hypothyroidism.

A term infant was born with respiratory distress, and subsequent imaging, histopathologic, and hormonal studies confirmed congenital hypothyroidism. This report is intended to alert pediatricians to the possibility of congenital hypothyroidism as a cause of respiratory symptoms of unknown cause in neonates with respiratory distress.

Longitudinal follow up of elevated pulmonary artery pressures in children with sickle cell disease.

Elevated pulmonary artery pressures (PAP) occur in approximately 30% of children with sickle cell disease. In adults, pulmonary hypertension is significantly associated with mortality. There are no data on the long term significance in children.

Electronic medical records in a sub-specialty practice: one asthma center's experience.

There are numerous known benefits associated with the use of an electronic medical record (EMR). In October of 2004, a pediatric respiratory medicine practice at a major academic institution began the process of implementing an EMR system. Through this process, another benefit was realized, improved coordination between out-patient and in-patient care in relation to asthma education.

Sleep-disordered breathing in children with metabolic syndrome: the role of leptin and sympathetic nervous system activity and the effect of continuous positive airway pressure.

Objective: The purpose of this work was to determine whether, in children with metabolic syndrome and sleep-disordered breathing, metabolic markers separate them from children with metabolic syndrome without sleep-disordered breathing and whether treatment of sleep-disordered breathing with continuous positive airway pressure is associated with an improvement in metabolic derangement.

Patients And Methods: Subjects aged 7 to 19 years old with metabolic syndrome and a positive validated sleep questionnaire were recruited. Subjects underwent overnight polysomnography, during which sympathetic nervous system activity was assessed via 8-hourly measurements of norepinephrine and epinephrine, together with leptin.

Prevalence and risk factors of elevated pulmonary artery pressures in children with sickle cell disease.

Objectives: The objectives of this study were (1) to determine the prevalence and risk factors of elevated pulmonary artery pressures in children with homozygous SS or Sbeta(0) thalassemia using Doppler echocardiography and (2) to determine a correlation between abnormal transcranial Doppler examinations and elevated pulmonary artery pressures.

Methods: Screening echocardiograms were prospectively performed during an annual comprehensive clinic visit on children who were older than 6 years and had homozygous SS or Sbeta(0) thalassemia. Detailed history, examination, and laboratory tests were done, and transcranial Doppler examinations were obtained in children 2 to 14 years of age.

Activation of DOR attenuates anoxic K+ derangement via inhibition of Na+ entry in mouse cortex.

We have recently found that in the mouse cortex, activation of delta-opioid receptor (DOR) attenuates the disruption of K(+) homeostasis induced by hypoxia or oxygen-glucose deprivation. This novel observation suggests that DOR may protect neurons from hypoxic/ischemic insults via the regulation of K(+) homeostasis because the disruption of K(+) homeostasis plays a critical role in neuronal injury under hypoxic/ischemic stress. The present study was performed to explore the ionic mechanism underlying the DOR-induced neuroprotection.

A role for macrophage migration inhibitory factor in the neonatal respiratory distress syndrome.

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.

VEGF attenuates hyperoxic injury through decreased apoptosis in explanted rat embryonic lung.

Ambient oxygen concentration and vascular endothelial growth factor (VEGF)-A are vital in lung development. Since hypoxia stimulates VEGF-A production and hyperoxia reduces it, we hypothesized that VEGF-A down-regulation by exposure of airways to hyperoxia may result in abnormal lung development. An established model of in vitro rat lung development was used to examine the effects of hyperoxia on embryonic lung morphogenesis and VEGF-A expression.