Pulmonary hemosiderosis in children with bronchopulmonary dysplasia.

We describe a possible association between pulmonary hemosiderosis (PH) and a history of bronchopulmonary dysplasia (BPD). Both patients were born at 28-week gestation and presented with PH at ages 22 months and 6 years, respectively. Both initially presented with cough and tachypnea, and bronchoalveolar lavage showed evidence of hemosiderin-laden macrophages. Initial hemoglobin levels were < 4 g/dL and chest radiographs showed diffuse infiltrates that cleared dramatically within days after initiation of intravenous corticosteroids. In the first case, frank pulmonary blood was observed upon initial intubation, prompting the need for high frequency ventilation, immediate corticosteroids, and antibiotics. The mechanical ventilation wean was made possible by the addition of mycophenolate mofetil (MMF) and hydroxychloroquine. Slow tapering off of medications was accomplished over 6 years. These cases represent a possible correlation between prematurity-associated BPD and PH. We present a review of the literature regarding this possible association. In addition, MMF proved to be life-saving in one of the PH cases, as it has been in pulmonary hemorrhage related to systemic lupus erythematosus. Further studies are warranted to investigate the possible association between PH and prematurity-related BPD, as well as the use of MMF in the treatment of PH.

Linking patient-centered medical home and asthma measures reduces hospital readmission rates.

OBJECTIVE: We sought to achieve 100% compliance with all 3 Children's Asthma Care (CAC; CAC-1, CAC-2, CAC-3) measures and track attendance at follow-up appointments with the patient-centered medical home. The impact of these measures on readmission and emergency department utilization rates was evaluated. METHODS: This quality improvement study evaluated compliance with CAC measures in pediatric patients aged 2 to 18 years old hospitalized with a primary diagnosis of asthma from January 1, 2008, through June 30, 2012. A multidisciplinary Asthma Task Force was assembled to develop interventions. Attendance at the follow-up appointment was tracked monthly from January 1, 2009. Readmission and emergency department utilization rates were compared between the preimplementation period (January 1, 2006, through December 31, 2007) and the postimplementation period (January 1, 2008, through June 30, 2012). RESULTS: The preimplementation period included 231 subjects and the postimplementation period included 532 subjects. Compliance with CAC-3 was 95% from October 1, 2009, through June 30, 2012. Compliance with the postdischarge follow-up appointment was 69% from January 1, 2009 through September 30, 2009, increasing significantly to 90% from October 1, 2009, through June 30, 2012 (P < .001). Postimplementation readmission rates significantly decreased in the 91- to 180-day postdischarge interval (odds ratio: 0.29; 95% confidence interval: 0.11-0.78). CONCLUSIONS: In children hospitalized with asthma, compliance with the asthma core measures and the postdischarge follow-up appointment with the primary care provider was associated with reduced readmission rates at 91 to 180 days after discharge. We attribute our results to a comprehensive set of interventions designed by our multidisciplinary Asthma Task Force.

Disaster preparedness for technology and electricity-dependent children and youth with special health care needs.

BACKGROUND: Children and youth with special health care needs (CYSHCN) are complex and often dependent on electrical devices (technoelectric dependent) for life support/maintenance. Because they are reliant on electricity and electricity failure is common, the purpose of this study was to survey their preparedness for electricity failure. METHODS: Parents and caregivers of technoelectric CYSHCN were asked to complete a preparedness questionnaire. We collected a convenience sample of 50 patients. RESULTS: These 50 patients utilized a total of 166 electrical devices. A home ventilator, oxygen concentrator, and a feeding pump were identified as the most important device for the children in 35 of the 50 patients, yet only 19 of the 35 patients could confirm that this device had a battery backup. Also, 22 of the 50 patients had a prolonged power failure preparedness plan. CONCLUSION: Technoelectric-dependent CYSHCN are poorly prepared for electrical power failure.

In vivo evidence of Pseudomonas aeruginosa nutrient acquisition and pathogenesis in the lungs of cystic fibrosis patients.

One of the hallmarks of Pseudomonas aeruginosa infection in cystic fibrosis (CF) patients is very-high-cell-density (HCD) replication in the lung, allowing this bacterium to induce virulence controlled by the quorum-sensing systems. However, the nutrient sources sustaining HCD replication in this chronic infection are largely unknown. Here, we performed microarray studies of P. aeruginosa directly isolated from the lungs of CF patients to demonstrate its metabolic capability and virulence in vivo. In vivo microarray data, confirmed by real-time reverse transcription-PCR, indicated that the P. aeruginosa population expressed several genes for virulence, drug resistance, and utilization of multiple nutrient sources (lung surfactant lipids and amino acids) contributing to HCD replication. The most abundant lung surfactant lipid molecule, phosphatidylcholine (PC), induces key genes of P. aeruginosa pertinent to PC degradation in vitro as well as in vivo within the lungs of CF patients. The results support recent research indicating that P. aeruginosa exists in the lungs of CF patients as a diverse population with full virulence potential. The data also indicate that there is deregulation of several pathways, suggesting that there is in vivo evolution by deregulation of a large portion of the transcriptome during chronic infection in CF patients. To our knowledge, this is the first in vivo transcriptome analysis of P. aeruginosa in a natural infection in CF patients, and the results indicate several important aspects of P. aeruginosa pathogenesis, drug resistance, nutrient utilization, and general metabolism within the lungs of CF patients.