Michael Light

Diagnosis and Management of Bronchiolitis

Bronchiolitis is a disorder most commonly caused in infants by viral lower respiratory tract infection. It is the most common lower respiratory infection in this age group. It is characterized by acute inflammation, edema, and necrosis of epithelial cells lining small airways, increased mucus production, and bronchospasm. The American Academy of Pediatrics convened a committee composed of primary care physicians and specialists in the fields of pulmonology, infectious disease, emergency medicine, epidemiology, and medical informatics. The committee partnered with the Agency for Healthcare Research and Quality and the RTI International-University of North Carolina Evidence-Based Practice Center to develop a comprehensive review of the evidence-based literature related to the diagnosis, management, and prevention of bronchiolitis. The resulting evidence report and other sources of data were used to formulate clinical practice guideline recommendations. This guideline addresses the diagnosis of bronchiolitis as well as various therapeutic interventions including bronchodilators, corticosteroids, antiviral and antibacterial agents, hydration, chest physiotherapy, and oxygen. Recommendations are made for prevention of respiratory syncytial virus infection with palivizumab and the control of nosocomial spread of infection. Decisions were made on the basis of a systematic grading of the quality of evidence and strength of recommendation. The clinical practice guideline underwent comprehensive peer review before it was approved by the American Academy of Pediatrics. This clinical practice guideline is not intended as a sole source of guidance in the management of children with bronchiolitis. Rather, it is intended to assist clinicians in decision-making. It is not intended to replace clinical judgment or establish a protocol for the care of all children with this condition. These recommendations may not provide the only appropriate approach to the management of children with bronchiolitis.

Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis—State of the Art: Cystic Fibrosis Foundation Consensus Conference

Because of the difficulties of recognizing allergic bronchopulmonary aspergillosis (ABPA) in the context of cystic fibrosis (because of overlapping clinical, radiographic, microbiologic, and immunologic features), advances in our understanding of the pathogenesis of allergic aspergillosis, new possibilities in therapy, and the need for agreed-upon definitions, an international consensus conference was convened. Areas addressed included fungal biology, immunopathogenesis, insights from animal models, diagnostic criteria, epidemiology, the use of new immunologic and genetic techniques in diagnosis, imaging modalities, pharmacology, and treatment approaches. Evidence from the existing literature was graded, and the consensus views were synthesized into this document and recirculated for affirmation. Virulence factors in Aspergillus that could aggravate these diseases, and particularly immunogenetic factors that could predispose persons to ABPA, were identified. New information has come from transgenic animals and recombinant fungal and host molecules. Diagnostic criteria that could provide a framework for monitoring were adopted, and helpful imaging features were identified. New possibilities in therapy produced plans for managing diverse clinical presentations.

Clinical Practice Guideline: The Diagnosis, Management, and Prevention of Bronchiolitis

This guideline is a revision of the clinical practice guideline, “Diagnosis and Management of Bronchiolitis,” published by the American Academy of Pediatrics in 2006. The guideline applies to children from 1 through 23 months of age. Other exclusions are noted. Each key action statement indicates level of evidence, benefit-harm relationship, and level of recommendation. Key action statements are as follows:

Updated Guidance for Palivizumab Prophylaxis Among Infants and Young Children at Increased Risk of Hospitalization for Respiratory Syncytial Virus Infection

Palivizumab was licensed in June 1998 by the Food and Drug Administration for the reduction of serious lower respiratory tract infection caused by respiratory syncytial virus (RSV) in children at increased risk of severe disease. Since that time, the American Academy of Pediatrics has updated its guidance for the use of palivizumab 4 times as additional data became available to provide a better understanding of infants and young children at greatest risk of hospitalization attributable to RSV infection. The updated recommendations in this policy statement reflect new information regarding the seasonality of RSV circulation, palivizumab pharmacokinetics, the changing incidence of bronchiolitis hospitalizations, the effect of gestational age and other risk factors on RSV hospitalization rates, the mortality of children hospitalized with RSV infection, the effect of prophylaxis on wheezing, and palivizumab-resistant RSV isolates. This policy statement updates and replaces the recommendations found in the 2012 Red Book.

Epidemiologic study of cystic fibrosis: Design and implementation of a prospective, multicenter, observational study of patients with cystic fibrosis in the U.S. and Canada

Cystic fibrosis (CF) is a complex illness characterized by chronic lung infection leading to deterioration in function and respiratory failure in over 85% of patients. An understanding of the risk factors for that progression and the interaction of these factors with current therapeutic strategies should materially improve the prevention of this progressive lung disease. The Epidemiologic Study of Cystic Fibrosis (ESCF) was therefore designed as a multicenter, longitudinal, observational study to prospectively collect detailed clinical, therapeutic, microbiologic, and lung function data from a large number of CF treatment sites in the U.S. and Canada. The ESCF also serves an important role as a phase‐IV study of dornase alfa. To be eligible for enrollment, subjects must have the diagnosis of CF and receive the majority of their care at an ESCF site.

Management of chronic sinusitis in cystic fibrosis.

Chronic rhinosinusitis is extremely common in patients with cystic fibrosis. It causes numerous problems in these patients and can put them at risk for life‐threatening illness. Potential problems include nasal obstruction, congestion, sinus pain and pressure, infection (usually with Pseudomonas organisms), hyposmia or anosmia, and the seeding of bacteria into the lower respiratory tract. Cystic fibrosis patients with chronically infected sinuses are at increased risk for pneumonia following lung transplantation. A prophylactic protocol has been developed for the management of chronic sinusitis in patients with cystic fibrosis. These patients are fully evaluated at the Nasal Dysfunction Clinic of the University of California, San Diego (UCSD), Medical Center. Based on the results of the evaluation, they are treated with endoscopic sinus surgery, partial middle turbinectomy, septoplasty, and a large middle meatal maxillary antrostomy. Surgery is followed by a rigorous regimen of pulsatile hypotonic saline nasal irrigation to wash away tenacious cystic secretions. Tobramycin (Nebcin®) is given once daily in the nasal irrigant to inhibit the growth of Pseudomonas organisms. At the USCD Nasal Dysfunction Clinic, this prepulmonary transplantation protocol is now used in all cystic fibrosis patients with chronic sinusitis.

Behavioral intervention to improve calorie intake of children with cystic fibrosis : Treatment versus wait list control

Changes in calorie intake and weight gain were evaluated in five children with cystic fibrosis (CF) who received behavioral intervention and four children with CF who served as wait list controls. The behavioral intervention was a 6-week group treatment that provided nutritional education plus management strategies aimed at mealtime behaviors that parents find most problematic. The control group was identified prospectively and was evaluated on all dependent measures at the same points in time pre- and posttreatment as the intervention group. Difference scores on calorie intake and weight gain from pre- to posttreatment were compared between groups using t tests for independent samples. The behavioral intervention group increased their calorie intake by 1,032 calories per day, while the control groups intake increased only 244 calories per day from pre- to posttreatment [t(6) = 2.826, p = 0.03]. The intervention group also gained significantly more weight (1.7 kg) than the control group (0 kg) over the 6 weeks of treatment [t(7) = 2.588, p = 0.03] and demonstrated catchup growth for weight, as indicated by improved weight Z scores (-1.18 to -0.738). The control group showed a decline in weight Z scores over this same time period (-1.715 to -1.76). One month posttreatment, the intervention was replicated with two of the four children from the control group. Improved calorie intake and weight gain pre- to posttreatment were again found in these children. At 3- and 6-month follow-up study of children receiving intervention, maintenance of calorie intake and weight gain was confirmed. No changes were found on pulmonary functioning, resting energy expenditure, or activity level pre- to posttreatment. This form of early intervention appears to be promising in improving nutritional status and needs to be investigated over a longer period of time to evaluate the effects of treatment gains on the disease process.

Immunogenicity of a new purified fusion protein vaccine to respiratory syncytial virus: A multi-center trial in children with cystic fibrosis

A third generation, purified fusion protein (PFP-3) vaccine was developed to prevent severe respiratory syncytial virus (RSV) disease in high-risk groups. A phase II, multi-center, adjuvant-controlled trial was performed in RSV seropositive children with cystic fibrosis (CF); 151 received the adjuvant-control and 143 received the vaccine. Details of the vaccine-induced immune response are presented. At enrollment, RSV-specific, serum antibodies were comparable between both groups. A highly sensitive and specific serum antibody vaccine profile was established for the PFP-3 vaccine. At post-vaccination and end-of-study, RSV-specific, neutralizing antibody (Nt Ab) and binding antibody (Bd Ab) to the fusion (F) protein were significantly higher in PFP-3 vaccinees. After 28 days post-vaccination, Nt Ab and Bd Ab to F protein titers declined slowly at rates of 0.23 and 0.37 log2 per month, respectively. The PFP-3 vaccine-induced a robust immune response that lasted throughout the RSV season.

Cystic Fibrosis, Nutrition, and the Health Care Team

Because of the multiple systems involved in cystic fibrosis, the variability and chronicity of the disease, and the increased survival of this population, a specialty team of experts for care has evolved. A multidisciplinary approach is essential to assist patients and their families in adjusting to the disease and to optimize treatment interventions. The dietitian is responsible for assessment of nutritional status, including the determination of energy requirements and eating habits, interpretation of anthropometric data, and evaluation of nutritional adequacy. The nutrition care plan forms an integral part of the overall treatment objectives and is reported to other team members as it is devised, implemented, and monitored. A consensus report issued in April 1990 by the Cystic Fibrosis Foundation includes both general nutrition guidelines and detailed recommended treatment standards aimed at providing optimal nutrition care.

Sinus Disease in Cystic Fibrosis

The current understanding of pathophysiology, diagnosis, and management of sinus disease has resulted in a more aggressive approach that appears to benefit children and adults with cystic fibrosis (CF). Chronic sinusitis, with and without nasal polyps, is problematic in the majority of CF patients, and previous treatment strategies have been associated with disappointing results. The new approaches for management of chronic sinus disease are clearly associated with improved outcome. The otolaryngologist has become an integral member of the team caring for CF patients.