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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 state- ment indicates level of evidence, benefit-harm relationship, and level of recommendation. Key action statements are as follows: Pediatrics 2014;134:e1474-e1502

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.

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.

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. In this paper, the authors present the ESCF study design in detail. Further, enrollment data collected at 194 study sites in 18,411 subjects enrolled from December 1, 1993 to December 31, 1995 are presented in summary form. This comprehensive study is unique in the detail of clinical data collected regarding patient monitoring and therapeutic practices in CF care. Two companion articles present data regarding practice patterns in cystic fibrosis care, including data on resource utilization and prescribing practices.

Objectives: Using the large database from the Epidemiologic Study of Cystic Fibrosis (ESCF), the objectives of this study were to (1) estimate the reported prevalence of allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis (CF); (2) compare reported prevalence rates across geographic regions; (3) compare reported prevalence rates between patient subgroups based on demographic and disease characteristics; and (4) describe the ABPA group with regard to their sex, age, and disease severity. Study design: All patients > 5 years of age enrolled in ESCF between December 1993 and May 1996 were eligible. Criteria for the diagnosis of ABPA were defined by the ESCF guidelines. Prevalence rates for ABPA were calculated, and potential risk factors for the diagnosis of ABPA were analyzed, including sex, age, pulmonary function, diagnosis of asthma, presence of wheeze, and positive respiratory culture for Pseudomonas. Results: There were 14,210 eligible patients enrolled in ESCF during this period, and ABPA was diagnosed in 281 patients (2%). Regional prevalence varied from 0.9% in the Southwest to 4.0% in the West. Increased prevalence rates occurred in female patients, the adolescent age group, and subjects with lower lung function, wheeze, asthma, and positive Pseudomonas cultures. Although most ABPA patients had evidence of airway obstruction, 10% had an FEV1 of > 100% of predicted. The rates of wheeze (17%) and asthma (30%) were lower than expected in the ABPA group. Conclusions: This observational study found a reported prevalence rate of ABPA of 2% of CF patients in a large database. This rate was lower than the 5 to 15% rate reported in smaller studies, suggesting that ABPA is underdiagnosed in the CF population. There was wide regional variation in reported prevalence rates, which is unexplained at this time. The characteristics of the patients with ABPA and the epidemiologic risk factors for diagnosis of ABPA were described. Simplified diagnostic criteria were adapted for ESCF with the intent of increasing awareness of ABPA among the participants in this study. (CHEST 1999; 116:639 ‐ 646)

Investigators participating in the Epidemiologic Study of Cystic Fibrosis project began to collect microbiological, pulmonary, and nutritional data on cystic fibrosis (CF) patients at 180 North American sites in 1994. Part of this study was a survey undertaken in August 1995 to determine microbiology laboratory practices with regard to pulmonary specimens from CF patients. The survey included a section on test ordering, completed by a site clinician, and a section on test performance and reporting, completed by each site’s clinical microbiology laboratory staff. Seventy-nine percent of the surveys were returned. There was intersite consistency of microbiology laboratory practices in most cases. The majority of sites follow most of the CF Foundation consensus conference recommendations. There were differences in the frequency at which specimens for culture were obtained, in the use of selective media for Staphylococcus aureus and Haemophilus influenzae , and in the use of a prolonged incubation for Burkholderia cepacia . These variations in practice contribute to prevalence differences among sites and may result in differences in clinical care.

This study evaluated a home-based, parent-managed, behavioral program to increase routine physical activity of ten 7-to 14-year-old children with cystic fibrosis. In each of 3 replications of a multiple baseline design, physical activity increased only after the intervention was initiated. Eight children increased total activity 42.5% to 321 %, and 2 children exercised more consistently. Study II recorded further activity increases at 6-week follow-up. Study III validated reported activity with increases of 7% to 27% in VO2max, 8% and 31.6% in VEmax, and 14.2% and 20% in Wmax. Results suggest that a home-based contingency management program can increase physical activity among chronically ill children with cystic fibrosis. Future studies are needed to assess maintenance and possible health benefits.

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.

The importance of RSV is well recognized, especially in the first few months of life. It is the most prevalent cause of lower respiratory tract infection during infancy causing both bronchiolitis and pneumonia. The spectrum of RSV illness varies from a mild upper respiratory infection that is clinically similar to a cold, all the way to respiratory failure and death from a LRTI. RSV has been demonstrated to cause dual infection both with another virus as well as not uncommonly being associated with a bacterial infection.

The authors sent questionnaires to 127 apnea monitoring programs asking whether they had treated patients whose apnea appeared to have been induced by a parent (Munchausen syndrome by proxy-apnea, or MBPA). Fifty-one programs (40%) reported 54 cases of this kind from among their 20,090 monitored patients (0.27%). The authors obtained further information on 32 of these patients, 83% of whom presented with infantile apnea before the third month of life.Although medical problems were documented, including apnea, the clinical condition of these infants was inconsistent with the multiple life-threatening episodes typically reported by parents. Twenty-one of the infants reportedly received cardiopulmonary resuscitation at home, 15 had ambulance calls to the home, and 24 were rehospitalized. Child Protective Service agencies were consulted for 12 patients, 5 of whom were placed in foster homes. Three index infants and five siblings are known to be dead, and one additional infant is severely brain damaged from abuse.The typical presentation of MBPA is multiple, reported, serious episodes of apnea occurring in the presence of only one person. When neither episodes nor cause can be documented satisfactorily, the diagnosis of MBPA should be considered and steps taken to confirm or refute it.

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.

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.

We investigated the hypothesis that children with cystic fibrosis (CF) and their parents would show more maladaptive behaviors during dinner than children without CF and their parents.Children with CF (n = 32) and their parents were compared with 29 children without CF and their parents on the rate and frequency of parent-child behaviors during a typical dinner in the families' homes by using multivariate analyses of variance.When the rate of behavior, controlling for meal length, was examined, no differences were found between groups. However, parents of children with CF were found to differ from parents of control subjects in the frequency of direct and indirect commands (P.05), coaxes (P.01), physical prompts (P.01), and feeding their child (P.05). Children with CF were found to engage in more talk, spend more time away from the table, refuse food, and exhibit more noncompliance toward commands to eat than control children (P.05 for all child variables). When behaviors were examined as a function of meal phase, parents of children with and without CF both showed an increase in commands (P.01), coaxes (P.05), feeds (P.01), and physical prompts (P.01) in the second half of the meal as compared with the first. Children with CF and the control children showed an increase in behaviors incompatible with eating during the second half of the meal compared with the first (P.01). When faster eaters were compared with slower eaters, faster eaters consumed a higher percentage of the recommended daily allowance of energy (P.01) than slower eaters and showed a trend to be at higher weight percentiles for age and sex (P =.08) regardless of group (CF or control).Children with CF and their parents do not differ from children without CF and their parents in the rate of behaviors exhibited or types of strategies used to encourage eating. However, children with CF and their parents engage in these behaviors more frequently. Our data do not support typical parenting behaviors as effective in meeting the CF dietary requirements. Additional support in the form of child behavior management training may be needed to assist parents in meeting their child's caloric requirements.

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. J Am Diet Assoc. 1997;97(suppl 2):S186-S191 .

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 group's 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.

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.

Sleep monitoring at 3 months post term of clinically normal term infants, pre-term infants with no demonstrable pathologic clinical signs, and pre-term and term infants with a history of metabolic disorders show that these infants have EEG sleep stages resembling those seen in adults, as well as the adult pattern of sleep stage organization. The presence of NREM stage organization and stage sequencing suggest that sleep regulatory mechanisms are approaching a level of functional maturity in the human infant at 3 months post term. There is a significant relationship between sleep staging at 3 months post term and mental and motor performance at 12 months post term. In light of this, it is hypothesized that early bioelectric maturation may reflect the development of neural mechanisms which are also the substrate for later cognitive and behavioral functioning. Sleep stage organization at 3 months post term may be utilized as a benchmark of CNS development and for research on the pathophysiology of sleep disorders.

Twenty-four preterm infants with respiratory failure from severe hyaline membrane disease (HMD) received mechanical ventilation at high respiratory frequencies. The average birthweight of the infants was 1244 +/- 301 g, and 7 babies weighed less than 1000 g. The average gestational age was 30 +/- 2 weeks, and 6 infants were born at 28 weeks or less. The method of ventilation included (1) respiratory frequencies of 60--110/min, sometimes with brief manual ventilation at more rapid rates, (2) peak inflation pressures (PIP) of less than 35 cm H2O, (3) inspiratory durations of 0.15--0.25 sec, (4) positive end-expiratory pressure (PEEP) of 4--9 cm H2O, and (5) weaning from mechanical ventilation by reducing tidal volume until peak inflation pressure (PIP) reached 20--25 cm H2O, whereupon respiratory frequency was decreased. PaCO2 was kept at 30--40 torr and PaO2 at 60--80 torr. Of the infants, 22 survived (92%) with few major complications.

HIGH-FREQUENCY MECHANICAL VENTILATION OF LOW-BIRTH-WEIGHT INFANTS WITH RESPIRATORY FAILURE FROM HYALINE MEMBRANE DISEASE: 92% SURVIVAL

Research has demonstrated that Sudden Infant Death Syndrome (SIDS) is emotionally devastating to families. An episode of apnea occurring at home requiring resuscitation is commonly referred to as near-miss SIDS. The relationship between nearmiss SIDS and SIDS is unclear, and the effect upon the family has been less well defined. 40 families who had experienced a near-miss SIDS episode responded to a questionnaire, 25 in writing and 15 by telephone. The questionnaire was completed 1 to 38 months after the episode. 37 of the respondents were present when the apnea occurred. 33% of the caretakers checked the baby as part of their routine activity, and 18% had a premonition that something was wrong and went to check the baby. 63% described it as “one of the hardest things in my life.” 10% described the experience as similar to a death in the family. Only 5% believed that their baby would have survived without resuscitation and 63% believed that their baby would die without intervention. As a result of this episode 20% decided not to have any more children, but half of these later reconsidered this decision. The average duration of time before things returned to normal was 3.7 (S.D. ± 3.8) months. These findings suggest that there is a significant psychological impact as a result of a near-miss SIDS episode, consistent with post-traumatic stress disorder, and that support services should be available to the family.