Preliminary indicate that sclerotherapy of microcystic LMs using bleomycin is effective and safe.

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

To characterize epidemiology and risk factors for respiratory viral infections (RVI) in pediatric lung transplant recipients within the first post-transplant year, a retrospective multicenter study of pediatric lung transplant recipients from 1988 to 2005 was conducted at 14 centers in the United States and Europe. Data were recorded for 1 year post transplant. Associations between RVI and continuous and categorical risk factors were assessed using Wilcoxon's rank-sum and chi(2) tests, respectively. Associations between time to RVI and risk factors or survival were assessed by multivariable Cox proportional hazards models. Of 576 subjects, 79 subjects (14%) had 101 RVI in the first year post transplant. Subjects with RVI were younger than those without RVI (median ages 9.7, 13; P<0.01). Viruses detected included adenovirus (n=25), influenza (n=9), respiratory syncytial virus (n=21), parainfluenza virus (n=19), enterovirus (n=4), and rhinovirus (n=22). In a multivariable model for time to first RVI, etiology other than cystic fibrosis (CF), younger age, and no induction therapy were independently associated with risk of RVI. Cytomegalovirus serostatus and acute rejection were not associated with RVI. RVI was independently associated with decreased 12-month survival (hazard ratio 2.6, 95% confidence interval 1.6-4.4). RVI commonly occurs after pediatric lung transplantation with risk factors including younger age and non-CF diagnosis. RVI is associated with decreased 1-year survival.

Two fully human mAbs specific for epitopes dependent on intact carboxylate groups on the C6 carbon of the mannuronic acid components of Pseudomonas aeruginosa alginate were found to promote phagocytic killing of both mucoid and nonmucoid strains as well as protection against both types of strains in a mouse model of acute pneumonia. The specificity of the mAbs for alginate was determined by ELISA and killing assays. Some strains of P. aeruginosa did not make detectable alginate in vitro, but in vivo protection against lethal pneumonia was obtained and shown to be due to rapid induction of expression of alginate in the murine lung. No protection against strains genetically unable to make alginate was achieved. These mAbs have potential to be passive therapeutic reagents for all strains of P. aeruginosa and the results document that alginate is a target for the proper type of protective Ab even when expressed at low levels on phenotypically nonmucoid strains.

Background A retrospective review of pediatric lung transplant recipients at 14 centers in North America and Europe was conducted to characterize the epidemiology and risk factors for cytomegalovirus (CMV) and to explore the impact of preventative antiviral therapy. Methods Data were recorded for one year post-transplant. Associations between CMV and continuous and categorical risk factors were assessed using Wilcoxon rank-sum and Chi-square tests, respectively. Associations between time to CMV and risk factors or survival were assessed by multivariable Cox proportional hazards models. Results Within 12 months posttransplant, 172 of 577 subjects (29.8%) developed 218 CMV episodes (90 asymptomatic infection, 25 syndrome, 103 disease). Forty-one subjects developed more than one episode of CMV. Donor or recipient CMV seropositivity was associated with increased risk of CMV episodes. Except for decreased prophylaxis in CMV D-/R- subjects, duration of prophylaxis did not vary by D/R serostatus. For CMV D+ subjects, not being on prophylaxis at the time of CMV episode increased the risk of CMV (D+/R+ HR 3.5: 95% CI 1.4, 8.4; D+/R- 1.9: 1.02, 3.7). CMV was associated with increased mortality within the first posttransplant year among those with donor or recipient CMV seropositivity (HR 2.0: 95% CI 1.1, 3.6; p=0.024). Conclusions CMV remains a serious complication after pediatric lung transplant, and the impact of prophylaxis is complex.

Background Immune cytopenias are a recognized life-threatening complication following pediatric solid organ transplants (SOT), but treatment responses and overall outcome are not well described. The aim of this study was to evaluate the demographic characteristics, response to treatments, and outcomes of a cohort of patients who developed immune cytopenias following SOT. Methods In this single center retrospective review, patients with immune cytopenias after SOT were identified by electronic medical record (EMR) search and transplant databases from 1995-2012. Results Of 764 SOT patients, 19 (2.4%) developed immune cytopenias. Incidence varied widely by transplant type from 1.2% (renal) to 23.5% (multivisceral). Autoimmune hemolytic anemia (AIHA) was the most common immune cytopenia. Overall median time from transplant to immune cytopenia was 8 m and varied by transplant type from 3 m (liver) to 74 m (heart). Standard therapies for immune cytopenias were often used and ineffective. The most effective therapy for the immune cytopenia was changing immunosuppression from tacrolimus to another agent. Three of 19 patients died; none directly attributed to the immune cytopenia. Conclusions Immune cytopenias are not rare after SOT, and patients usually do not respond well to traditional first line therapies. Provided that the risk of organ rejection is otherwise manageable, temporary cessation of tacrolimus could be more widely explored in this challenging clinical context.

Churg-Strauss syndrome is a vasculitis accompanied by asthma and eosinophilia. It is generally considered a disease of adults; occurrence in children has been reported infrequently. Here we report 2 pediatric patients with Churg-Strauss syndrome manifesting with prominent pulmonary involvement. One, a 16-year-old with a previous history of asthma, presented with pleuritic chest pain and a peripheral pulmonary nodule complicated by an eosinophilic pleural effusion. The other patient presented at age 6 with cough, weight loss, and radiographic infiltrates. Lung biopsies revealed elements characteristic of Churg-Strauss syndrome, including eosinophilic microabscesses and vasculitis. Three- and 5-year follow-up showed continued symptoms in both patients despite medical therapy. Both patients illustrate many of the typical features of Churg-Strauss syndrome. We report these cases to expand the scant knowledge about Churg-Strauss syndrome in pediatric patients and to heighten awareness that this serious disease may affect the pediatric population. The relevant literature on Churg-Strauss syndrome, with specific reference to childhood cases, is reviewed.

Pediatric lung transplantation is a lifesaving option for patients with end stage lung disease, although the scarcity of suitable donor organs results in long wait times and increased waitlist mortality. Many pediatric centers consider mechanical ventilatory support, such as long-term invasive ventilation and ECMO, a contraindication to lung transplantation. We hypothesized that current survival rates and outcomes for patients on mechanical ventilatory support in the pre-transplant period were not remarkably different. In our retrospective analysis we included patients between the ages of 0-21 years listed for lung transplantation from deceased donors between 2007 and 2014 at our institution. One-year survival outcomes were compared between three groups of patients: (i) patients bridged to transplant on ECMO (n = 6, 1-year survival = 67%); (ii) patients needing mechanical ventilation (either through endotracheal intubation or tracheostomy) but not ECMO (n = 12, 1-year survival = 75%); and (iii) patients who did not need endotracheal ventilation, tracheostomy, or ECMO (n = 25, 1-year survival = 88%). Comparison of outcomes of transplanted patients between these three groups were not statistically different in terms of successful hospital discharge and 1-year survival rates (P > 0.05). We believe that "bridging" the end-stage lung disease patient with long-term mechanical ventilation and/or ECMO support is a reasonable option in selected patients until suitable donors become available. Pediatr Pulmonol. 2017;52:360-366. © 2016 Wiley Periodicals, Inc.