Pulmonary extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue associated with granulomatous inflammation in a child with chromosome 22q11.2 deletion syndrome (DiGeorge syndrome).

Patients with immunodeficiency disorders have an increased incidence of lymphoproliferative disorders; however, only 4 such patients with DiGeorge/chromosome 22q11.2 deletion syndrome have been reported. We report a case of a pulmonary Epstein-Barr virus-negative extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in a child with this syndrome.

Lobar displacement following pediatric heart-lung transplantation: case report and review of literature.

Lobar displacement (LD) after heart-lung transplantation (HLT) has been reported in adults, but there are no reported pediatric cases. Its occurrence may cause vascular compromise of the displaced lung segment leading to necrosis, infection and bronchiectasis, as well as compression of contralateral lobes. We report two cases of LD in children following HLT, treated differently and with different outcomes. Assessment of pulmonary perfusion and weighing the risk of surgical repair may be considered for optimal patient management of this condition.

Feasibility of laparoscopic Nissen fundoplication after pediatric lung or heart-lung transplantation: should this be the standard?

BACKGROUND: Five-year graft survival in the pediatric lung transplant (LTxp) population is less than 50%, with obliterative bronchiolitis (OB) the leading cause of death at 1, 3, and 5 years post-transplant. Bronchiolitis obliterans syndrome (BOS), defined using spirometry values, is the clinical surrogate for the histological diagnosis of obliterative bronchiolitis. Surgical correction of documented gastroesophageal reflux disease (GERD) has been proposed as a means to potentially delay the onset of BOS and prolong allograft survival in adults before or after lung transplantation but only one such study exists in children. We have examined the safety and possible benefits of laparoscopic antireflux surgery in pediatric patients following lung (LTxp) and heart-lung transplantation (HLTxp). METHODS: An Institutional Review Board (IRB)-approved retrospective chart review was performed to evaluate the outcomes and complications of laparoscopic antireflux surgery in pediatric lung and heart-lung transplant patients. Spirometry data were collected for BOS staging using BOS criteria for children. RESULTS: Twenty-five lung and heart-lung transplants were performed between January 2003 and July 2009. Eleven transplant recipients, including six double-lung and five heart-lung (HLTxp), with a median age of 11.7 years (range 5.1-18.4 years), underwent a total of 12 laparoscopic Nissen fundoplications at a median of 427 days after transplant (range 51-2310 days). GERD was determined based upon clinical impression, pH probe study, gastric emptying study, and/or esophagram in all patients. Three patients already had a gastrostomy tube in place and two had one placed at the time of fundoplication. There were no conversions to open surgery, 30-day readmissions, or 30-day mortalities. Complications included one exploratory laparoscopy for free air 6 days after laparoscopic Nissen fundoplication for a gastric perforation that had spontaneously sealed. Another patient required a revision laparoscopic Nissen 822 days following the initial fundoplication for a paraesophageal hernia and recurrent GERD. The average length of hospital stay was 4.4 ± 1.7 days. Nine of the 12 fundoplications were performed in patients with baseline spirometry values prior to fundoplication and who could also complete spirometry reliably. One of these nine operations was associated with improvement in BOS stage 6 months after fundoplication; seven were associated with no change in BOS stage; and one was associated with a decline in BOS stage. CONCLUSION: It is feasible to perform laparoscopic Nissen fundoplication in pediatric lung and heart-lung transplant recipients without mortality or significant morbidity for the treatment of GERD. The real effect on pulmonary function cannot be assessed due to our small sample size and lack of reproducible spirometry in our younger patients. Additional studies are needed to elucidate the relationship between antireflux surgery and the potential for improving pulmonary allograft function and survival in children which has been previously observed in adult patients.

Standardized Treatment of Pulmonary Exacerbations (STOP) study: Observations at the initiation of intravenous antibiotics for cystic fibrosis pulmonary exacerbations.

BACKGROUND: The Standardized Treatment of Pulmonary Exacerbations (STOP) program has the intent of defining best practices in the treatment of pulmonary exacerbations (PEx) in patients with cystic fibrosis (CF). The objective of this analysis was to describe the clinical presentations of patients admitted for intravenous (IV) antibiotics and enrolled in a prospective observational PEx study as well as to understand physician treatment goals at the start of the intervention. METHODS: We enrolled adolescents and adults admitted to the hospital for a PEx treated with IV antibiotics. We recorded patient and PEx characteristics at the time of enrollment. We surveyed treating physicians on treatment goals as well as their willingness to enroll patients in various study designs. Additional demographic and clinical data were obtained from the CF Foundation Patient Registry. RESULTS: Of 220 patients enrolled, 56% were female, 19% were adolescents, and 71% were infected with P. aeruginosa. The mean (SD) FEV1 at enrollment was 51.1 (21.6)% predicted. Most patients (85%) experienced symptoms for ≥7days before admission, 43% had received IV antibiotics within the previous 6months, and 48% received oral and/or inhaled antibiotics prior to IV antibiotic initiation. Forty percent had ≥10% FEV1 decrease from their best value recorded in the previous 6months, but for 20% of patients, their enrollment FEV1 was their best FEV1 recorded within the previous 6months. Physicians reported that their primary treatment objectives were lung function recovery (53%) and improvement of symptoms (47%) of PEx. Most physicians stated they would enroll patients in studies involving 10-day (72%) or 14-day (87%), but not 7-day (29%), treatment regimens. CONCLUSIONS: Based on the results of this study, prospective studies are feasible and physician willingness for interventional studies of PEx exists. Results of this observational study will help design future PEx trials.

Site of Bronchoalveolar Lavage Via Flexible Bronchoscopy and Fluid Return in Children.

BACKGROUND: Despite its widespread use as a diagnostic tool, the procedure for bronchoalveolar lavage (BAL) via flexible bronchoscopy is not standardized in children. Our objective was to examine the dissimilarities in fluid return between the different lobes in children undergoing flexible bronchoscopies with BAL. METHODS: We conducted a review of all pediatric flexible bronchoscopies with BAL conducted at a single institution over a 2-year period. Our predictor of interest was the site of the BAL. Our outcome of interest was the percent of fluid return. We used 1-way analysis of variance with subsequent pairwise comparisons for unadjusted analyses and multivariable linear regression for adjusted analyses. RESULTS: We identified 529 procedures that met prespecified criteria. The mean (SD) percent of fluid return was 52.1 (14.4) for the right middle lobe, 50.7 (16.0) for the lingula (LIN), 50.5 (18.6) for the right or left upper lobes other than LIN (R/L-UL), and 42.2 (18.7) for the right or left lower lobes (R/L-LL). The R/L-LL had significantly lower fluid return when compared with each of the other lobes (P<0.05 for all pairwise comparisons); in contrast, there was no significant difference in fluid return between the other lobes. In our main analysis adjusting for potential confounders, performing the BAL in the right middle lobe, LIN, or R/L-UL increased the fluid return by 11.1% [95% confidence interval (CI), 6.2-16.1], 9.5% (95% CI, 3.2-15.8), and 8.7% (95% CI, 0.9-16.5%), respectively, when compared with the R/L-LL. CONCLUSION: Our results suggest that the lower lobes provide the lowest BAL fluid return in children, whereas the other lobes seem to perform similarly.

Leukotriene receptor antagonists--risks and benefits for use in paediatric asthma.

Leukotrienes (LTs) are important mediators of the pathophysiology of asthma, specifically, bronchoconstriction, airway inflammation and oedema and mucus hypersecretion. The LT receptor antagonists (LTRAs) inhibit these potent effects by selectively blocking the cysteinyl LT 1 receptor. These are the first novel therapies for asthma since the introduction of inhaled corticosteroids (ICS) in 1972. Unlike generalised inhibition of airway inflammation by ICS, the LTRAs target inhibition of specific mediators. In general, paediatric data concerning these agents remain quite limited. However, they have demonstrated efficacy against allergen- and exercise-induced bronchospasm in both adults and children. Recently, their potential role for the treatment of viral-induced wheeze in young children has been explored. In multiple, placebo-controlled trials, the LTRAs have demonstrated efficacy for the treatment of mild persistent asthma, additive benefit in the management of symptomatic moderate asthmatics on maintenance ICS and evidence of significant steroid-sparing. Findings from these clinical trials and real-world experience support the use of the LTRAs as controller agents for persistent asthma. Based on their excellent safety profiles, tolerance and ease of administration (including once daily dosing with montelukast), this drug class may offer several important features for use as controller therapy, particularly in asthmatic children as young as 1 year of age, however, this must continue to be reviewed as new paediatric data become available.

Heart-lung transplantation: pediatric indications and outcomes.

As indications for heart-lung transplant (HLT) have changed to some degree in the past 30 years, this treatment is being used less frequently in children due to more advanced care of severe heart and lung disease. This is fortunate as the outcomes for HLT are poor compared to other solid organ transplants and this is mainly due to the poorer outcome of the lung graft.

Comparison of two aspiration techniques of bronchoalveolar lavage in children.

BACKGROUND: Although bronchoalveolar lavage (BAL) via flexible bronchoscopy is an essential diagnostic tool, its technique is not standardized in children. Our objective was to compare two different aspiration techniques of BAL in children (continuous wall suction vs. handheld syringe suction) in regards to the percentage of fluid recovered and the odds of performing a technically acceptable procedure (i.e., >40% of volume return). METHODS: We conducted a review of all pediatric flexible bronchoscopies with BAL conducted at our institution over a 2-year period. To minimize the differences between groups at baseline and reduce the possibility of bias, we used one-to-one propensity score (PS) caliper matching with no replacement for statistical analyses. RESULTS: We identified 539 procedures that met pre-specified criteria. There were considerable covariate imbalances between procedures in the handheld syringe group (n = 147) and those in the continuous wall group (n = 392); however, these imbalances were substantially reduced after the PS matching. In the matched sample (n = 236), children in the handheld syringe group had ∼7% higher volume return (95% CI = 3.4-11.0, P < 0.001) from BAL and threefold higher odds (95% CI = 1.5-8.6, P = 0.002) of performing a technically acceptable procedure. CONCLUSIONS: Our results suggest that handheld syringe suction offers a higher percentage of volume return from BAL and increases the odds of performing a technically acceptable procedure in children when compared to continuous wall suction.

Malignant pleural mesothelioma in a child with ataxia-telangiectasia.

Ataxia-telangiectasia (AT) is a hereditary disorder characterized by progressive neurological dysfunction, oculocutaneous telangiectasia, immunodeficiency, cancer susceptibility, and radiation sensitivity. Pleural neoplasms are extremely rare in the pediatric population, even in patients with AT. We describe the case of a 16-year-old male with AT who developed a malignant pleural mesothelioma (MPM). Benign or infectious lung and pleural diseases are common in those with AT. Hence, delayed diagnosis of respiratory neoplasms can occur in these patients. This report highlights the need of heightened vigilance in patients with AT with recurrent or persistent pleuropulmonary disease. To our knowledge, no other cases of MPM in children with AT have been reported.

An unusual solitary endobronchial pulmonary arteriovenous malformation in a 10 year-old child.

Pulmonary arteriovenous malformations (PAVMs) are relatively rare in children. They may occur both as single isolated anomalies and, much more commonly, as one of many widely distributed arteriovenous malformations (AVMs) in other organs and anatomic locations such as the liver, brain, nose and spine. The latter phenotypically defines the condition known as Hemorrhagic Hereditary Telengiectasia (HHT). We report a case of a 10 year-old female with an isolated endobronchial right lower lobe PAVM. The patient's chest radiograph and fine-cut contrast-enhanced CT (CECT) of the neck and thorax were both uncharacteristically normal and, hence, non-diagnostic. Surgical lobectomy was the only means by which to both diagnose and treat this life-threatening condition. This unique case points out the dilemma facing the physician in such a case with only two management options neither of which is ideal: to either obtain a diagnosis with biopsy which is minimally invasive yet life-threatening due to the potential for fatal hemorrhage, or to perform a pulmonary lobectomy which entails removal of an entire lobe of the lung without a pre-operatively confirmed indication to justify the procedure. To our knowledge, this is the first reported case of a child with an endobronchial PAVM who did not carry the diagnosis of HHT, had normal chest radiography, and had a normal fine-cut CECT of the neck and thorax where such a management dilemma arose. This case points out that a high index of suspicion for PAVMs must be maintained despite normal chest radiography and CECT of the chest. In such cases, empiric lobectomy becomes the sole therapeutic and definitive diagnostic intervention. We hope that such a case published in the literature serves as a guide to physicians confronting similar circumstances as the rarity of such a constellation of variables precludes higher levels of evidence reporting such as a retrospective case series or prospective randomized controlled clinical trials.