Swyer-James syndrome (SJS) is a rare syndrome characterized by hyperlucency of one or more lobes of the unilateral lung and decreased pulmonary vascularity which is considered to be secondary to the childhood obliterative bronchiolitis. We report anesthetic management of a girl with SJS who underwent flexible bronchoscopy. A 15-year-old girl weighing 47 kg was referred to our hospital with suspected SJS. She had a history of bronchiolitis in early childhood. On admission, chest radiograph showed a hyperlucent lung with diminished pulmonary vasculature on the left lung. Pulmonary function test revealed slightly restrictive pattern (%VC 69.3%, %FEVa1.0 77.9%). Ventilation and perfusion scanning demonstrated the markedly decreased ventilation (left : right, 33 : 67) and perfusion (left : right, 10 : 90) of the left lung. To rule out the foreign body and bronchial stenosis, flexible bronchoscopy under general anesthesia was planned. Anesthesia was induced with propofol and maintained with propofol and remifentanil. Spontaneous respiration was maintained by using laryngeal mask airway to prevent the pressure alveolar damage by positive pressure ventilation. Flexible bronchoscopy was performed safely by using topical laryngeal lidocaine administration and additional bolus of propofol. Her postoperative course was uncomplicated and she was discharged on the following day.

Interstitial lung disease in children (chILD) is a group of disorders characterized by lung inflammation and interstitial fibrosis. In the past recent years, we noted an outbreak of child in Korea, which is possibly associated with inhalation toxicity. Here, we report a series of cases involving toxic inhalational injury-associated chILD with bronchiolitis obliterans pattern in Korean children. This study included 16 pediatric patients confirmed by lung biopsy and chest computed tomography, between February 2006 and May 2011 at Asan Medical Center Children's Hospital. The most common presenting symptoms were cough and dyspnea. The median age at presentation was 26 months (range: 12-47 months), with high mortality (44%). Histopathological analysis showed bronchiolar destruction and centrilobular distribution of alveolar destruction by inflammatory and fibroproliferative process with subpleural sparing. Chest computed tomography showed ground-glass opacities and consolidation in the early phase and diffuse centrilobular nodular opacity in the late phase. Air leak with severe respiratory difficulty was associated with poor prognosis. Although respiratory chemicals such as humidifier disinfectants were strongly considered as a cause of this disease, further studies are needed to understand the etiology and pathophysiology of the disease to improve the prognosis and allow early diagnosis and treatment.

BACKGROUND:

In the management of acute bronchiolitis there is a generalised use of treatments that have not been shown to be useful or efficacious in clinical studies. The objective of this study was to determine the appropriateness in the treatment of acute bronchiolitis of different severity within different clinical care settings.

METHODS:

This is a cross-sectional, descriptive study of 5647 cases of acute bronchiolitis in 91 Spanish hospitals and primary care centres. We classified the appropriateness of the treatments according to the recommendations of a consensus conference.

RESULTS:

There was an inappropriate use of treatments in 58.3% of the cases during the acute phase and in 45.4% during the maintenance phase. There was a generalised use of inhaled beta 2 agonists, regardless of the severity of the patients (hospitalised patients 69.3%, emergency care 63.2% and ambulatory 64.1%). Adrenaline was used in 30.1% of hospitalised cases and in 80.2% of intensive care patients. Systemic corticosteroids were not only used in one-third of hospitalised patients but also in 25.8% of ambulatory cases.

CONCLUSIONS:

In acute bronchiolitis in Spain there is a wide use of treatments that are not recommended by the available clinical practice guidelines. Beta 2 agonist bronchodilators and corticosteroids are widely used and maintained, regardless of the severity of the patients.

Aberrant epithelial repair is a crucial event in the airway remodeling that characterizes obliterative bronchiolitis (OB) in transplanted lungs. Recent data from experiments using epithelial cell lines and human airway tissues from lung transplant recipients suggest that epithelial to mesenchymal transition (EMT) plays an important role in OB. The aim of this study was to clarify whether EMT is involved in airway remodeling in an animal model.We performed orthotopic tracheal transplantation from BALB/c to C57BL/6 mice with from BALC/c to BALB/c mouse grafts as controls. Five allogeneic and 3 syngeneic recipients were humanely killed at predetermined postoperative days 2-12 as well as 14 and 21. Histology was evaluated using hematoxylin-eosin (H&E) staining. We studied the expression of specific markers, including E-cadherin, an epithelial marker; α-smooth muscle actin (SMA), and S100A4, mesenchymal markers, and zinc finger E-box-binding homeobox 1 (ZEB1), an EMT-related transcription factor.Histologic assessment of serial H&E stains of allogeneic grafts showed remarkable pseudostratified respiratory epithelium with subepithelial inflammatory cell infiltration, as well as denuded and flattened epithelium and subepithelial fibrosis. The dynamic epithelial changes occurred earlier than the subepithelial fibrosis. Immunohistochemical evaluation indicated the emergence of α-SMA- positive epithelial cells that were most prominent on day 7. The expression of E-cadherin was attenuated in α-SMA-positive epithelial cells. S100A4 was also expressed in epithelial cells. A few days before the intraepithelial expression of α-SMA, ZEB1 emerged in the nuclei of epithelial cells.We observed expression of an EMT-related transcription factor and mesenchymal markers along with the attenuation of epithelial marker expression in epithelial cells, several days before prominent subepithelial fibrosis formation, results that suggest epithelial cells to play an important fibrosis role in airway remodeling during epithelial to mesenchymal transition.

Viral illness with wheezing during infancy is associated with the inception of childhood asthma, and small airway dysfunction is a component of childhood asthma, but little is known about how viral illness at an early age may affect the structure and function of small airways. We employed a well-characterized rat model of post-bronchiolitis chronic airway dysfunction to address how post-infectious small airway lesions affect airway physiological function, and if the structure/function correlates persist into maturity. BN rats were sham- or virus-inoculated at 3-4 weeks of age, and allowed to recover from the acute illness. At 3-14 months of age, physiology (respiratory system resistance, Newtonian resistance, tissue damping, and static lung volumes) was assessed in anesthetized, intubated rats. Serial lung sections revealed lesions in the terminal bronchioles that reduced luminal area and interrupted further branching, affecting 26% (range 13-39%) of the small airways at 3 months of age and 22% (range 6-40%) at 12-14 months. At 3 months (n=29 virus, 7 sham), small airway lesions correlated with tissue damping (rs=0.69), but not with Newtonian resistance (rs=0.23), and Newtonian resistance was not elevated compared with controls, indicating that distal airways were primarily responsible for the airflow obstruction. Older rats (n=7 virus, 6 sham) had persistent small airway dysfunction, as well as significantly increased Newtonian resistance in the post-bronchiolitis group. We conclude that viral airway injury at an early age may induce small airway lesions that are associated quantitatively with small airway physiological dysfunction early on, and that these defects persist into maturity.

AIMS AND OBJECTIVES:

To measure the benefit of a short-family therapeutic conversation (STC) intervention in an acute paediatric unit.

BACKGROUND:

Studies of children with bronchiolitis caused by respiratory syncytial virus (RSV) have shown that this virus may have an impact on their respiratory system in the form of a wheezing disorder, asthma and even allergy during their childhood. Studies of the parents of these children indicate that they experience distress, vulnerability and anxiety through the illness period and therefore need support from healthcare professionals. However, little is known about what intervention is of most benefit for these parents.

DESIGN:

Quasi-experimental.

METHOD:

Data were collected from a convenience sample from February throughout April 2009 at an acute unit at a children's hospital in Iceland. Parents of infants diagnosed with bronchiolitis caused by RSV were invited to attend. In total, there are 41 participants: 21 in the intervention group (n = 21) and 20 in the control group (n = 20). Parents in both groups answered questionnaires about perceived support and family expressive functioning both before the intervention and on an average of 11 days after the intervention.

RESULTS:

The main findings showed that mothers in the intervention group perceive significantly higher support after the intervention compared with the control group. The findings also showed a significant difference between the genders (mothers and fathers) in the intervention group. The mothers perceived higher cognitive support than the fathers.

CONCLUSIONS:

Despite the often chaotic environment in an acute care setting, the research findings give paediatric nurses reason to conclude that a STC intervention benefits mothers of infants with bronchiolitis caused by RSV. RELEVANCE TO CLINICAL PRACTICE: A STC intervention offered by a nurse within an acute paediatric unit can support families in handling the illness experience.

Ricin toxin, a type 2 ribosome-inactivating protein and a category B bioterrorism agent, is produced from the seeds of castor oil plant (Ricinus communis). Chronic pathological changes in survivors of aerosolized ricin exposure have not been reported in primates. Here we compare and contrast the pathological changes manifested between rhesus macaques (RM) that succumbed to lethal dose of ricin (group I) and survivor RM exposed to low dose of ricin (group II). All animals in group I exhibited severe diffuse, necrotizing bronchiolitis and alveolitis with fibrinopurulent bronchointerstitial pneumonia, massive alveolar, perivascular and peribronchial/bronchiolar edema with hemorrhage, and necropurulent and hemorrhagic tracheobronchial lymphadenitis. All animals from group II had multifocal, fibrosing interstitial pneumonia with prominent alveolar histiocytosis and type II pneumocyte hyperplasia. Subacute changes like infiltration by lymphocytes and plasma cells and persistence of edematous fluid were occasionally present in lung and tracheobronchial lymph nodes. The changes appear to be a continuum wherein the inflammatory response shifts from an acute to subacute/chronic reparative process if the animals can survive the initial insult.

Acute bronchiolitis in infants frequently results in hospitalization, but there is no established consensus on inhalation therapy--either the type of medication or the frequency of administration--that may be of value. We aimed to assess the effectiveness of inhaled racemic adrenaline as compared with inhaled saline and the strategy for frequency of inhalation (on demand vs. fixed schedule) in infants hospitalized with acute bronchiolitis.In this eight-center, randomized, double-blind trial with a 2-by-2 factorial design, we compared inhaled racemic adrenaline with inhaled saline and on-demand inhalation with fixed-schedule inhalation (up to every 2 hours) in infants (<12 months of age) with moderate-to-severe acute bronchiolitis. An overall clinical score of 4 or higher (on a scale of 0 to 10, with higher scores indicating more severe illness) was required for study inclusion. Any use of oxygen therapy, nasogastric-tube feeding, or ventilatory support was recorded. The primary outcome was the length of the hospital stay, with analyses conducted according to the intention-to-treat principle.The mean age of the 404 infants included in the study was 4.2 months, and 59.4% were boys. Length of stay, use of oxygen supplementation, nasogastric-tube feeding, ventilatory support, and relative improvement in the clinical score from baseline (preinhalation) were similar in the infants treated with inhaled racemic adrenaline and those treated with inhaled saline (P>0.1 for all comparisons). On-demand inhalation, as compared with fixed-schedule inhalation, was associated with a significantly shorter estimated mean length of stay--47.6 hours (95% confidence interval [CI], 30.6 to 64.6) versus 61.3 hours (95% CI, 45.4 to 77.2; P=0.01) - as well as less use of oxygen supplementation (in 38.3% of infants vs. 48.7%, P=0.04), less use of ventilatory support (in 4.0% vs. 10.8%, P=0.01), and fewer inhalation treatments (12.0 vs. 17.0, P<0.001).In the treatment of acute bronchiolitis in infants, inhaled racemic adrenaline is not more effective than inhaled saline. However, the strategy of inhalation on demand appears to be superior to that of inhalation on a fixed schedule. (Funded by Medicines for Children; ClinicalTrials.gov number, NCT00817466; EudraCT number, 2009-012667-34.).

To understand the infections and molecular biological characteristics of different human rhinovirus (HRV) genotypes -A, B, C, especially C in children with acute respiratory tract infections (ARI) in Beijing. Seven hundreds and three respiratory tract specimens were collected from children with ARI during Jan. 2011 to Dec. 2011. Semi-nested PCR was developed for detecting HRVs. Gene fragment of VP4/VP2 capsid protein amplified from HRV positive specimens was sequenced and analyzed by software DNAStar, the phylogenetic tree was then constructed by MEGA 5. 05. Among these 703 specimens tested, 54 (7.7%, 54/703) were HRV positive, including 25 (46.3%, 25/54) positive for HRV-A, 8 (14. 8%, 8/54) for HRV-B, 21 (38. 9%, 21/54) for HRV-C determined by sequence analysis. Most of these children (94. 4%00, 51/54) infected with HRVs were younger than 5 years old, and the highest positive rate was shown in group younger than 1 year (11. 4%). These patients positive for HRVs were diagnosed as bronchiolitis (23.1%), asthma (20.0%), pneumonia (1.0%), bronchitis (4.4%) and upper respiratory tract infections (4. 1%). Sequence analysis of VP4/VP2 gene fragment revealed that 70. 0% to 100. 0% nucleotide identity was shown among the sequences within the same HRV genotype, and 55. 5% to 65. 8% nucleotide identity among the sequences from different HRV genotypes. In conclusion, HRVs, especially HRV-C, are important pathogens for children with ARI in Beijing. The prevalence of HRV-C is similar to that of HRV-A, higher than that of HRV-B. High sequence variation among different HRV genotypes was indicated in this study.