Acute respiratory distress syndrome (ARDS) is a disabling and potentially lethal syndrome requiring prompt recognition and urgent interventions to prevent morbidity and mortality. Although constipation is not generally recognized as a cause for ARDS or usually listed within the differential diagnosis, there have been case reports describing such an association[2,3]. We present the case of a patient with history of intermittent constipation presenting with progressive abdominal pain and an acute abdomen that required emergent surgical fecal decompaction. This was followed by hypoxemic respiratory distress leading to respiratory failure in the setting of severe constipation and aspirated feculent material. To our knowledge, this is the first published case report describing aspirated feculent material in a child with respiratory failure due to ARDS.
Clinical care in cystic fibrosis (CF) has continued to advance over the last several years, particularly with the widespread eligibility and use of highly effective modulator therapy. Awareness of the multisystem concerns that face persons with CF (PwCF) has also continued to be recognized as an important aspect of the burden of the disease. This review will cover a broad array of topics, from diagnosis to multisystem effects related to mental health, endocrine, palliative care, reproductive health, otolaryngology, and cardiac issues. Additionally, an understanding of worldwide care delivery will be reviewed, demonstrating variation in outcomes based on resources and populations served, ranging from the advances in care in the United States (US) to the challenges of disease recognition and diagnosis in low-and-middle-income countries (LMIC). This review is the third in a three-part CF Year in Review 2020 series, focusing on the multi-system effects of CF. Part one focused on the literature related to CFTR (cystic fibrosis transmembrane conductance regulator protein) modulators, while part two focused on pulmonary outcomes, radiographic and physiologic assessments, as well as infection and inflammation. Part three has been split into two individual parts, Part 3A presented here, and Part 3B related to CF specific nutrition and gastrointestinal publications will also be published this year. This review focuses on articles from Pediatric Pulmonology but also includes articles published in 2020 from other journals that are of particular interest to clinicians.
The multisystemic manifestations of cystic fibrosis (CF) involve all parts of the gastrointestinal (GI) system, including the pancreas, intestine and liver. As providers who care for people with CF (PwCF), knowledge of the manifestations, treatment and research related to nutrition and GI disease is important. This review is last installment of the CF Year in Review 2020 series, focusing on the multisystem effects of CF. Part one focused on the literature related to CFTR (cystic fibrosis transmembrane conductance regulator protein) modulators, while part two focused on pulmonary outcomes, radiographic and physiologic assessments, as well as infection and inflammation. Part three was split into Part 3A, focusing on the multisystem impact of CF, and this review, Part 3B, focusing on nutritional, gastrointestinal and hepatobiliary articles. Articles were chosen from Pediatric Pulmonology but also include articles published in 2020 from other journals that are of particular interest to clinicians.
Objective: To analyze the proportion of backup ventilation during neurally adjusted ventilatory assist (NAVA) in preterm infants at different gestational ages and to analyze the trends in backup ventilation in relation to clinical deteriorations. Methods: A prospective observational study was conducted in 18 preterm infants born at a median (range) 27+4 (23+4–34+4) weeks of gestation with a median (range) birth weight of 1,100 (460–2,820) g, who received respiratory support with either invasive or noninvasive NAVA. Data on ventilator settings and respiratory variables were collected daily; the mean values of each 24-hour recording were computed for each respiratory variable. For clinical deterioration, ventilator data were reviewed at 6-hour intervals for 30 hours prior to the event. Results: A total of 354 ventilator days were included: 269 and 85 days during invasive and noninvasive NAVA, respectively. The time on backup ventilation (%/min) significantly decreased, and the neural respiratory rate increased with increasing postmenstrual age during both invasive and noninvasive NAVA. The median time on backup ventilation was less than 15%/min, and the median neural respiratory rate was more than 45 breaths/min for infants above 26+0 weeks of gestation during invasive NAVA. The relative backup ventilation significantly increased prior to the episode of clinical deterioration. Conclusion: The proportion of backup ventilation during NAVA showed how the control of breathing matured with increasing gestational age. Even the most immature infants triggered most of their breaths by their own respiratory effort. An acute increase in the proportion of backup ventilation anticipated clinical deterioration.
Actinomycosis is a rare, indolent and invasive infection caused by Actinomyces species. Pulmonary actinomycosis is very rarely seen in the paediatric population. The classic radiological presentation of thoracic involvement of actinomycosis includes lower lobe consolidation, empyema and periostitis of the ribs. We report a case of endobronchial pulmonary actinomycosis in a child diagnosed on endobronchial biopsy and broncho-alveolar lavage taken during bronchoscopy. Bronchoscopy can be dangerous when performed on these cases, as there is a risk of severe bleeding and large airway obstruction, as was the case with this patient.
Reintubation in the pediatric intensive care unit (PICU) increases morbidity, mortality, and the overall cost of care. Post-extubation airway obstruction (PEAO) is a potentially predictable cause of extubation failure and may be prevented by the use of corticosteroids. Defining which patients are most at risk for the development of POAE as well as the optimal dose and timing of corticosteroids for prevention is critical. We review the current literature regarding the use of corticosteroids surrounding extubation in the PICU and discuss the implications that a clear algorithm for identification and treatment of these patients would have in the care of critically ill children.
Objectives: Discharging a child home on long term ventilation (LTV) via tracheostomy is complex and involves multiple healthcare providers across healthcare sectors. To date, patient and family feedback of a newly developed LTV discharge pathway has been anecdotal. Our objective was to explore the perceptions of family caregivers (FCs) that have completed the LTV pathway to home with respect to their: (1) experience with transitions across the pathway (2) perceptions of competency attainment and, (3) viewed opportunities for improvement. Methods: We conducted 11 semi structured interviews with FCs. Interviews focused on FCs experience with the training process, perception of competency from a knowledge and skill perspective and opportunities for improvement. Interviews were audiotaped, transcribed verbatim, coded and analyzed using an inductive thematic analysis approach. Results: Eight mothers and 3 fathers of 10 children participated. Six primary themes were identified: 1) making an informed decision, 2) transitioning to rehabilitation, 3) building capacity for self-care, 4) coordinating case management, 5) readying for discharge home and, 6) experiencing home care. Conclusion: Overall, FCs felt that the preparation and transition support obtained through the application of a standardized LTV discharge pathway allowed successful attainment of new knowledge and skills necessary to care for their child with LTV at home.
Diagnosing asthma in preschool children remains an unsolved challenge, at a time when early identification would allow for better education and treatment to prevent morbidity and lung function deterioration. Objective: To evaluate if the Asthma Predictive Index (API) can be used as surrogate for asthma diagnosis in preschoolers. Methods: Birth cohort of 339 pregnant women enrolled at delivery and their offspring, who were followed for atopy, wheezing, and other respiratory illnesses through 30 months of age. The API was determined at 30 months of age by the researchers; and examined its association with physician-diagnosed asthma during the first 30 months, made independently by the primary care physician not involved in the study. Results: Among 307 offspring with complete follow-up, 44 (14.3%) were API+. Maternal body mass index, maternal education, past oral contraceptive use, birthweight, placenta weight, age of daycare at 12m, gastroesophageal reflux disease at 12m, acute otitis media at 18m, bronchiolitis, croup and pneumonia, cord blood adiponectin were all associated with API+. In the multivariable analysis, API+ was associated with almost 6-fold odds of asthma diagnosis (adjusted OR= 5.7, 95% CI [2.6-12.3]), after adjusting for the relevant covariates above including respiratory infections like bronchiolitis and pneumonia. The API sensitivity was 48%, specificity 92%, 61% PPV, 88% NPV, 6.4 LR+, 0.56 LR-, 0.84 diagnosis accuracy. The adjusted odds for asthma was 11.4. Conclusions: This longitudinal birth cohort suggests, for first time, that API could be used as a diagnostic tool, not only as a prognostic tool, in toddlers and preschoolers.
Background and Objective: The knowledge about the impact of the nonpharmacological measures to control the COVID-19 pandemic can give insight to ways in which they can also be applied for other respiratory diseases. To assess the impact of containment measures of the COVID-19 pandemic on pneumonia hospitalizations in children from 0 to 14 years of age in Brazil. Methods: Data from hospital admissions for pneumonia were obtained from the Department of Informatics of Brazilian Public Health System database in the period of 2015–2020 and analyzed by macro-regions and age groups. To evaluate the effect of containment measures, used in the pandemic, on the incidence of pneumonia, the absolute reduction and relative reduction were calculated by analyzing the subsets 2015-2019 vs 2020. Results: Comparing the subsets of April-August 2015-2019 vs April-August 2020, there was an expressive reduction in the average incidence of hospitalizations, with numbers ranging from -87% [IRR 0.12 (0.10 to 0.14)] for < 4 years, -79% [IRR 0.21 (0.07 to 0.57)] for 5-9 years, -73% [IRR 0.26 (0.05 to 1.21)] for 10-14 and -86% [IRR 0.14 (0.06 to 0.29)] for <14 years. Conclusion: We found a significant decrease in cases of pneumonia during the COVID-19 pandemic. Nonpharmacological public health interventions can contribute to the decline of other respiratory infectious diseases.
Objective: Investigate the cardiorespiratory effects of non-invasive neurally adjusted ventilatory assist (NIV-NAVA), non-synchronized nasal intermittent positive pressure ventilation (NIPPV), and nasal continuous positive airway pressure (NCPAP) during the critical period shortly after extubation. Hypothesis: Levels of non-invasive pressure support provided and/or presence of synchronization can affect cardiorespiratory parameters. Study design: Randomized crossover trial. Patient-subject selection: Infants with birth weight (BW) ≤ 1250g undergoing their first planned extubation were randomly assigned to all 3 modes following extubation. Methodology: Electrocardiogram and electrical activity of the diaphragm (Edi) were recorded during 30min on each mode. Analysis of heart rate variability (HRV), diaphragmatic activity (Edi area, breath area, amplitude, inspiratory and expiratory times) and respiratory variability (RV) were compared between modes. Results: 23 enrolled infants had full data recordings and analysis: median [IQR] gestational age = 25.9 weeks [25.2-26.4], BW = 760g [595-900], and post-natal age 7 [4-19] days. There were no differences in HRV parameters between modes. During NIV-NAVA and NIPPV, diaphragmatic activity was significantly lower and RV higher than NCPAP. Delivered peak inflation pressures (PIPs) were lower during NIV-NAVA than NIPPV (14 cmH2O [13-16] vs cmH2O 16 [16-17]; p<0.001). However, due to a significantly higher proportion of assisted breaths (99% [92-103] vs. 51% [38-82]; p<0.001) NIV-NAVA provided a higher mean airway pressure (MAP)(9.4 cmH2O [8.2-10.0] vs. 8.2 cmH2O [7.6-9.3]; p=0.002). Conclusions: NIV-NAVA and NIPPV applied shortly after extubation were associated with positive cardiorespiratory effects. This effect was more evident during NIV-NAVA where patient-ventilator synchronization provided a higher MAP with lower PIPs.
Silent RSV in infants with SARS‑CoV‑2 infection: a case seriesAntonietta Giannattasio, MD, PhD1, Marco Maglione, MD, PhD1, Carolina D’Anna, MD1, Stefania Muzzica, MD1,Francesca Angrisani, MD1, Sabrina Acierno, MD1, Alessandro Perrella2, MD, PhD2, Vincenzo Tipo, MD11Santobono-Pausilipon Children’s Hospital, Pediatric Emergency and Short Stay Unit, Naples, Italy2Cardarelli Hospital, Infectious Disease-Health Policy Direction, Naples, Italy
Objectives: Pneumothorax (PTX) in newborns is a life-threatening condition associated with high morbidity and mortality especially in premature infants. The frequency of PTX in neonates at different gestational ages (GA) and its impact on neonatal mortality have not been quantified. We aimed to determine: 1) the prevalence of PTX in neonates at different GA from ≤24 weeks to ≥37 weeks, 2) the impact of PTX on mortality per GA, and 3) the impact of PTX on the length of stay (LOS) per GA. Methods: The national Kids’ Inpatient Database (KID) for the years of 2006 to 2012 were used. We included all infants admitted to the hospital with a documented GA and ICD9 code of pneumothorax. Bivariate and multivariate analyses were conducted and odds ratios (OR) were calculated. Results: A total of 10 625 036 infants were included; of them 3665 infants (0.034 %) had a diagnosis of PTX, with highest prevalence at ≤24 weeks GA (0.67%), and lowest at term (0.02%). The overall mortality rate of patients with PTX was 8.8%, and greater in preterm (16.3%) vs. term infants (2.7%). The association of mortality with PTX was greatest at GA of 29−32 weeks (OR = 8.55 (95% CI: 6.56−11.13). Infants who survived until discharge had a median of 2–12 days longer length of stay depending on GA category. Conclusions: The prevalence of PTX peaks in infants <24 weeks, however its impact on mortality is greatest at 29-32 weeks. PTX is associated with longer length of stay in survivors.
Background: A series of repeated questionnaire surveys among 8- and 9-year-old schoolchildren in the city of Patras, Greece, demonstrated a continuous rise in the prevalence of wheeze/asthma from 1978 to 2003, with a plateau between 2003 and 2008. We further investigated wheeze/asthma trends within the same environment during the last decade. Methods: Two further identical cross-sectional surveys were conducted in 2013 (N=2554) and 2018 (N=2648). Physician-diagnosed wheeze and asthma were analysed separately and in relation to their occurrence (recent-onset: solely within the last two years; non-current: prior to the last two years; persistent: both before and within the last two years). In addition, in 2018, spirometry was performed in participants reporting relevant symptoms and in a random sample of healthy controls. Results: The prevalence of current wheeze/asthma declined from 6.9% in 2008 to 5.2% in 2013 and 4.3% in 2018. The persistent and non-current wheeze/asthma groups (both including children with symptoms at preschool age) followed this overall trend, while the prevalence of recent-onset wheeze/asthma remained unchanged. Persistent and non-current wheezers were also more frequently diagnosed with asthma, in contrast to those with recent-onset wheeze. Children with recent-onset wheeze/asthma and a considerable fraction of those with persistent symptoms had lower lung function. Conclusions: The prevalence of childhood wheeze/asthma has declined significantly during the last decade in Greece. Our analysis suggests that the reversing trend is most likely attributed to changing asthma perceptions among physicians and/or parents, especially in the case of preschool children with troublesome respiratory symptoms.
CFTR (cystic fibrosis transmembrane conductance regulator) modulators are small molecules that directly change the CFTR protein, improving function of the CFTR chloride channel. Beginning in 2012 with the FDA approval of the first CFTR modulator, ivacaftor, this class of medication has had largely positive effects on many outcomes in people with cystic fibrosis (pwCF), including lung function, quality of life, and growth. There have been continued exciting developments in the current research on CFTR modulators, expanding beyond original studies. This first part of a three-part Cystic Fibrosis (CF) Year in Review 2020 will focus on research on CFTR modulators. Subsequent parts of the CF year in review will cover pulmonary and infectious inflammatory aspects, and the multisystem effects of CF in the 2020 literature. The review focuses on articles from Pediatric Pulmonology, but it includes articles from other journals that are of particular interest to clinicians. New developments in CF research continue to be brought forth to the CF community, deepening the understanding of this disease and improving clinical care.
Bullying-induced dyspnoea in children: a case seriesIan P. Sinha 1,2Claire Hepworth 1Sujata De 1Sunil D. Sharma 1Ian Street 1Philip J. Lawrence 1Thomas Hampton* 1,21 Alder Hey Children’s Hospital, Liverpool, UK2 Division of Child Health, University of Liverpool, UK*Corresponding author (Thomas.firstname.lastname@example.org)Dear EditorWe conduct a multidisciplinary complex breathlessness clinic for children1. We conduct spirometry before and after a treadmill exercise test (until the child is breathless), continuous nasal laryngoscopy, pulse oximetry, and calculation of maximal oxygen consumption (VO2max). Here we describe a series of children who presented with troublesome breathlessness that appeared to be caused, or exacerbated by, being bullied.Case 1 (14 year old white female): She was a highly competitive sportsperson, but was recently unable to train or compete. She described her breathlessness as ‘air getting stuck in her throat’, and had a non-specific cough. She had frequent admissions to hospital, treated as presumed asthma attacks. The referring clinician felt her asthma was of insufficient severity to cause her problems. She had no documented obstruction on spirometry in clinic, nor and Fractional Exhaled Nitric Oxide (FeNO) was normal. In clinic she managed a few minutes of running, before suddenly stopping. There was no evidence of exercise-induced bronchoconstriction or exercise-induced laryngeal obstruction (EILO), but she had features of dysfunctional breathing (DB). On questioning she described feeling bullied by parents of other children in her sports team, whom she described as overcritical and disparaging. She was taught breathing exercise and referred to psychological services. Her symptoms and asthma attacks improved, and she recommenced competitive sports.Case 2 (10 year old white female): She had a chronic wet cough since the age of six months. She underwent flexible bronchoscopy which identified mild tracheobronchomalacia. She recently described breathlessness on mild exertion that was disproportionate to the degree of tracheobronchomalacia. Physiological testing never demonstrated airways obstruction, or raised FeNO. In clinic she started running but stopped within minutes, with no physiological evidence of increased work of breathing or bronchoconstriction. Laryngoscopy was normal. On questioning she described that she was bullied at school. Specifically, she described that children would not sit near her because of her cough. She was followed up in respiratory physiotherapy clinic, and her symptoms and exercise tolerance improved.Case 3 (13 year old white female): She had no medical diagnoses at the time of testing, but previously had tonsillectomy because of recurrent tonsillitis. She described breathlessness on exertion. She stopped running very suddenly, as soon as she felt breathless on the treadmill. The breathlessness started as discomfort underneath her ribs and in her throat. Spirometry and laryngoscopy were normal before and after exercise. She had apical breathing and hyperventilation at rest, suggestive of dysfunctional breathing. She described being bullied at school. She did not attend follow up sessions with physiotherapy and was discharged from the service.Case 4 (9 year old black male): He was treated for mild asthma which had, until recently, been well controlled. He had become withdrawn, and was not enjoying playing sports despite previously being very athletic. On questioning he described suffering significant and long-standing racial bullying at school. He discussed this with the teachers but his symptoms seemed to develop after he felt like his complaints were not taken seriously. His breathlessness and exercise tolerance improved temporarily after enrolling in a community sports program for children with asthma 2, and he was much better after moving school.Across these cases, we identified common themes:The character and severity of the breathlessness was out of keeping with their underlying diagnoses, and was intensely unpleasant. All children described non-specific and variable symptoms of pain in their abdomen, joints, or chest.They appeared withdrawn, unhappy, and lacking in self-confidence. They had slouched posture, and spoke quietly.They had very sudden and surprising termination of exercise after starting to feel breathless, with no significant physiological evidence of increased work of breathing – we noticed a stark difference compared with other children who saw breathlessness as a challenge and would continue to run long after showing signs of tachypnoea and tachycardia.In all cases, it was the healthcare professionals who raised the subject of bullying. The children had felt that they had raised concerns about being bullied but felt these were dismissed.When children engaged with physiotherapy, we noticed improvements. The children were relieved when we did not find significant anatomical or physiological diagnoses, and all agreed their breathlessness was caused by bullying.We feel that these patterns relate to a phenotype of childhood dyspnoea specifically related to bullying. Anxiety is associated with tachypnoea, but in our experience the pattern of breathing in these children was different: their shallow, rapid breathing at rest was felt to be related to a slouched posture causing restriction, and all had a sudden cessation to exercise after very minimal exertion. We suggest these are manifestations, specifically, of low self-esteem. It was notable that they described the breathlessness as intensely uncomfortable. The neural pathways involved in driving respiration, and sensing breathlessness, are complex, and we postulate that they are affected by low self-esteem.This, to our knowledge, is the first description of bullying-induced dyspnoea in children, as a separate phenomenon to anxiety-related hyperventilation. There may be crossover with other psychological, physiological and anatomical problems, and further research is warranted. Having asthma is a risk factor for being bullied3, and bullying is associated with worse asthma control4. A recent review has also identified possible associations between bullying and the development of childhood asthma5. It is important ask about bullying when taking a history from a child with unexplained or disproportionate breathlessness.
Bronchopulmonary dysplasia is a relatively common and severe complication of prematurity, and its pathogenesis remains ambiguous. Revolutionary advances in microbiological analysis techniques, together with the growing sophistication of the gut-lung axis hypothesis, have resulted in more studies linking gut microbiota dysbiosis to the occurrence and development of bronchopulmonary dysplasia. The present article builds on current findings to examine the intrinsic associations between gut microbiota and bronchopulmonary dysplasia. The gut microbiota affects bronchopulmonary dysplasia via several potential mechanisms including alteration of the gut-lung axis, promotion of inflammation and the ensuing growth effects, therefore these are also investigated. By evaluating the potential mechanisms, new therapeutic targets and potential therapeutic modalities for BPD can be identified from a microecological perspective.
OBJECTIVE: Sustained pharyngeal inflation (SPI) with pharyngeal oxygen and nose-close (PhO2-NC) can create positive peak inflation pressure (PIP) inside the pharyngolaryngeal space (PLS). This study measured and compared effects of four different SPI durations in the PLS. METHOD: In this prospective observational study, 20 consecutive infants aged less than 3 years, scheduled for elective flexible-bronchoscopy were enrolled. SPI was performed twice in four different durations (0, 1, 3 and 5 seconds) sequentially in each infant. PIP was measured for each SPI in the pharynx, and simultaneously took images at two locations of oropharynx and supra-larynx. Infants’ demographic details and PIP levels, lumen expansion scores and images of PLS were measured and analyzed. RESULTS: Twenty infants with 40 measurements were collected. The mean (SD) age and weight were 11.6 (9.1) months and 6.8 (2.4) kg, respectively. The measured mean (SD) pharyngeal PIPs were 4.1 (3.3), 21.9 (7.0), 42.2 (12.3) and 65.5 (18.5) cm H2O at SPI duration of 0, 1, 3 and 5 seconds, respectively; which showed significant positive association (p<0.001). At assigned locations, the corresponding PLS images also show significant increase in lumen expansion scores and number of detected lesions with increase in SPI duration (p<0.001). The mean (SD) study time was 5.7 (1.2) minutes. No study related complication was noted. CONCLUSIONS: SPI with PhO2-NC up to 5 seconds is a simple, safe and feasible clinical ventilation modality. It may provide enough PIP to expand the PLS and facilitate flexible-bronchoscopy performance in infants.