Pediatric Lung Research Articles

Exploring the Feasibility of At-Home Lung Ultra-Portable Ultrasound: Parent-Performed Pediatric Lung Imaging.

To determine if caregivers would be able to successfully perform in home lung ultrasounds on their children without direct supervision after undergoing a basic tutorial that would allow for expert interpretation. A prospective exploratory single-center cohort study was conducted on patients (0-18 years) presenting to a pediatric emergency department with a respiratory complaint or COVID-related illness. Caregivers underwent a brief hands-on session and were instructed to scan the lungs daily for 7 days. Images were assessed using a modified POCUS IQ score. Descriptive statistics were used to describe the data and bivariate analysis was used to compare groups. Eighteen patients were enrolled; the average age of the parent scanner was 31.9 years and 78% were female. Of all participants, 77.8% scanned on day one. Parents were able to successfully perform some part of the daily scan session for an average of 3.8 out of 7 days. The average POCUS IQ score overall was 6.7 (out of 12). Our study demonstrates the feasibility and acceptability of caregiver ability to obtain adequate lung ultrasound images, at home under no guidance, using the Butterfly iQ probe. Further studies are needed to investigate the accessibility of ultra-portable ultrasound and the ability to integrate with the at-home hospital model, specifically in the pediatric population.

Evaluation of the classifications of severity in acute respiratory distress syndrome in childhood by the Berlin Consensus and the Pediatric Acute Lung Injury Consensus Conference.

To compare two methods for defining and classifying the severity of pediatric acute respiratory distress syndrome: the Berlin classification, which uses the relationship between the partial pressure of oxygen and the fraction of inspired oxygen, and the classification of the Pediatric Acute Lung Injury Consensus Conference, which uses the oxygenation index. This was a prospective study of patients aged 0 - 18 years with a diagnosis of acute respiratory distress syndrome who were invasively mechanically ventilated and provided one to three arterial blood gas samples, totaling 140 valid measurements. These measures were evaluated for correlation using the Spearman test and agreement using the kappa coefficient between the two classifications, initially using the general population of the study and then subdividing it into patients with and without bronchospasm and those with and without the use of neuromuscular blockers. The effect of these two factors (bronchospasm and neuromuscular blocking agent) separately and together on both classifications was also assessed using two-way analysis of variance. In the general population, who were 54 patients aged 0 - 18 years a strong negative correlation was found by Spearman's test (ρ -0.91; p < 0.001), and strong agreement was found by the kappa coefficient (0.62; p < 0.001) in the comparison between Berlin and Pediatric Acute Lung Injury Consensus Conference. In the populations with and without bronchospasm and who did and did not use neuromuscular blockers, the correlation coefficients were similar to those of the general population, though among patients not using neuromuscular blockers, there was greater agreement between the classifications than for patients using neuromuscular blockers (kappa 0.67 versus 0.56, p < 0.001 for both). Neuromuscular blockers had a significant effect on the relationship between the partial pressure of oxygen and the fraction of inspired oxygen (analysis of variance; F: 12.9; p < 0.001) and the oxygenation index (analysis of variance; F: 8.3; p = 0.004). There was a strong correlation and agreement between the two classifications in the general population and in the subgroups studied. Use of neuromuscular blockers had a significant effect on the severity of acute respiratory distress syndrome.

Clinical Profile and Outcome of Moderate to Severe Acute Respiratory Distress Syndrome in a Paediatric Intensive Care Unit of Eastern India: A Prospective Cohort Study

Introduction: Paediatric Acute Respiratory Distress Syndrome (PARDS) remains a major cause of mortality and morbidity in Paediatric Intensive Care Units (PICU) despite various advanced management strategies. The management and outcome of PARDS depend on the severity of the disease. Aim: To study the clinical profile in terms of aetiology and outcome of paediatric patients with moderate to severe Acute Respiratory Distress Syndrome (ARDS) in the PICU. Materials and Methods: A prospective cohort study was conducted at the PICU in Dr. BC Roy Postgraduate Institute of Paediatric Sciences, Kolkata, West Bengal, India, from July 2018 to June 2019. A total of 120 children aged between two months and 12 years who developed moderate to severe ARDS according to Paediatric Acute Lung Injury Consensus Conference (PALICC) criteria were included. Demographic details, different risk factors, morbidity patterns, and outcomes were recorded. Patients were categorised into three groups based on Positive End Expiratory Pressure (PEEP): 5-8 cm of H2 O, 9-12 cm of H2 O, and &gt;12 cm of H2 O. Based on Peak Inspiratory Pressure (PIP), patients were divided into two groups: &lt;30 cm of H2 O and 30-35 cm of H2 O. Data were statistically analysed using the Chisquare test, Fisher’s test, and t-tests where applicable. Results: Out of a total of 120 children with moderate to severe ARDS, there were 75 males and 45 females. Direct lung injury accounted for 79 cases (65.5%), while indirect lung injury occurred in 41 cases (34.5%). Pneumonia and sepsis were the most common causes of direct and indirect lung injury, respectively. Among 75 cases of pneumonia, 40 deaths were reported (53.3%), and out of 37 cases of sepsis, 31 resulted in death (83%). On the day of admission, 38.3% of cases were classified as moderate ARDS and 61.7% as severe ARDS. The mortality rate for severe ARDS was 77%, compared to 43.5% for moderate ARDS. The mortality rate was 100% in the PEEP max &gt;12 cm of H2 O group and 84.4% in the PIP 30-35 cm of H2 O group. Conclusion: The majority of ARDS cases are due to direct lung injury caused by pneumonia, and the outcome is better than in cases of indirect lung injury. Mortality is nearly twice as high in severe ARDS as in moderate ARDS.

Waiting for the Pediatric Acute Lung Injury Consensus Conference 3.

Waiting for the Pediatric Acute Lung Injury Consensus Conference 3.

Lung transplantation in children.

Lung transplantation is a well-established treatment for children facing advanced lung disease and pulmonary vascular disorders. However, organ shortage remains highest in children. For fitting the small chest of children, transplantation of downsized adult lungs, lobes, or even segments were successfully established. The worldwide median survival after pediatric lung transplantation is currently 5.7 years, while under consideration of age, underlying disease, and peri- and posttransplant center experience, median survival of more than 10 years is reported. Timing of referral for transplantation, ischemia-reperfusion injury, primary graft dysfunction, and acute and chronic rejection after transplantation remain the main challenges.

The Role of Lung Ultrasonography as a Diagnostic Tool in Different Pediatric Lung Diseases in Benha University Hospital

The Role of Lung Ultrasonography as a Diagnostic Tool in Different Pediatric Lung Diseases in Benha University Hospital

Hyperoxia-induced airflow restriction and Renin-Angiotensin System expression in a bronchopulmonary dysplasia mouse model.

Mechanisms underlying hyperoxia-induced airflow restriction in the pediatric lung disease Bronchopulmonary dysplasia (BPD) are unclear. We hypothesized a role for Renin-Angiotensin System (RAS) activity in BPD. RAS is comprised of a pro-developmental pathway consisting of angiotensin converting enzyme-2 (ACE2) and angiotensin II receptor type 2 (AT2), and a pro-fibrotic pathway mediated by angiotensin II receptor type 1 (AT1). We investigated associations between neonatal hyperoxia, airflow restriction, and RAS activity in a BPD mouse model. C57 mouse pups were randomized to normoxic (FiO2 = 0.21) or hyperoxic (FiO2 = 0.75) conditions for 15 days (P1-P15). At P15, P20, and P30, we measured airflow restriction using plethysmography and ACE2, AT1, and AT2 mRNA and protein expression via polymerase chain reaction and Western Blot. Hyperoxia increased airflow restriction P15 and P20, decreased ACE2 and AT2 mRNA, decreased AT2 protein, and increased AT1 protein expression. ACE2 mRNA and protein remained suppressed at P20. By P30, airflow restriction and RAS expression did not differ between groups. Hyperoxia caused high airflow restriction, increased pulmonary expression of the pro-fibrotic RAS pathway, and decreased expression of the pro-developmental in our BPD mouse model. These associated findings may point to a causal role for RAS in hyperoxia-induced airflow restriction.

Newer Modalities of Pediatric Lung Function Assessment

Newer Modalities of Pediatric Lung Function Assessment

Pediatric systemic juvenile idiopathic arthritis-related lung disease: Description of clinical cohort and review of management.

Since the early 2010s, there has been an increased awareness of interstitial lung disease in systemic juvenile idiopathic arthritis (sJIA-LD) in pediatric patients. Despite the increase in prevalence of sJIA-LD, little is known about this disease process and effective therapeutic management. To identify and characterize the disease process and management of interstitial lung disease related to systemic juvenile idiopathic arthritis. In this single-center, retrospective case series of 9 patients, we analyze demographic, clinical, radiographic, and laboratory data to corroborate common clinical characteristics and describe an approach for diagnosis and monitoring of sJIA-LD. All data was extracted through electronic medical records and individually reviewed by two pediatric pulmonologists and two pediatric rheumatologists. Our results were similar to other described cases of sJIA-LD as patients in our cohort were more likely to be younger, have a history of macrophage activation syndrome and prior use of biologic therapies. In contrast to prior studies, they did not present with peripheral lymphadenopathy and hepatosplenomegaly. The cohort size was small and data is reflective of one center's approach to management of a rare lung disease process. Interstitial lung disease due to sJIA is rare and management can be difficult in these complex patients.More research is necessary to understand the increased incidence and treatment of sJIA-LD in pediatric population.

Interstitial lung disease as an indication for pediatric lung transplant.

Interstitial lung disease can be an indication for lung transplant at any age, but it is a particularly common indication for lung transplant in infants. Nevertheless, not all interstitial lung diseases will lead to lung transplant in childhood. Genetic testing has aided the identification of these diseases in children. In severely affected patients, however, definitive diagnosis is not always necessary to consider referral to a transplant center. At experienced transplant centers, a multidisciplinary team educates patient families and aids in the transplant evaluation of children with interstitial lung disease. Children who have undergone transplant require lifetime immunosuppression and close surveillance, but can enjoy good quality of life for years following surgery.

Disparities in lung transplantation in children.

Lung transplantation is a recognized therapy for end-stage respiratory failure in children and young people. It is only available in selected countries and is limited by access to suitable organs. Data on disparities in access and outcomes for children undergoing lung transplantation are limited. It is clear from data from studies in adults, and from studies in other solid organ transplants in children, that systemic inequities exist in this field. While data relating specifically to pediatric lung transplantation are relatively sparse, professionals should be aware of the risk that healthcare systems may result in disparities in access and outcomes following lung transplantation in children.

Pediatric thoracoscopic lung resections: a comprehensive analysis of congenital lesion cases

Background Congenital lung lesions in pediatric patients may be managed conservatively or by video assisted thoracoscopic surgery (VATS). This study aimed to determine the complications after VATS for congenital lung lesions in children. Methods All children undergoing a lung resection between January 2009 and June 2022 were retrospectively identified. Children undergoing a primary open lobectomy or a resection other than a congenital lung lesion were excluded. Both early (<30 days) and late postoperative pulmonary complications were determined. The primary endpoint was postoperative complications within 30 days. Results In total, 56 patients were included, with a median age of 13 months (IQR 9-37). A VATS lobectomy were performed in 46 patients (82%), an extralobar sequestration in 8 patients (14%), an wedge resection in 1 patient and a segment resection in 1 patient. During the COVID pandemic, fewer resections were performed with an increase in symptomatic patients. A conversion to open occurred in 6 patients (11%), of which a preoperative lung infection was associated with an increased risk thereof(p = 0.004). The median follow-up was 22 months (IQR 7-57) and all patients were alive. A postoperative complication (Clavien Dindo ≥3) occurred in 9 patients and complications without the need of intervention in 6 patients. During follow-up a pneumonia occurred in 11 patients. Conclusion There seems to be a shift towards delayed surgery with an increase in symptomatic congenital lung malformations, which might lead to an increase in postoperative complications

Gender and authorship of publications from Pediatric Acute Lung Injury and Sepsis Investigators (PALISI).

Pediatric Acute Lung Injury and Sepsis Investigators (PALISI) is a network fostering clinical research to optimize care for critically ill children. We aim to examine the efforts of the PALISI Network to increase gender parity in research, as evidenced by authorship. The first and senior authors of all published PALISI articles from 2002 to 2021 were analyzed for gender of presentation. Funding sources, impact factors, professional roles, and location were extracted. We identified 303 articles, 61 published from 2002 to 2011, and 242 from 2012 to 2021. There were 302 first authors, representing 188 unique individuals, and 283 senior authors, representing 119 unique individuals. Over half (55.6%, n = 168) of the first authors were women. More women were first authors from 2012 to 2021 (n = 145, 60.2%) as compared to the years 2002-2011 [37.7%, n = 23, OR = 2.50 (95% CI: 1.40, 4.45, p = 0.002)]. Senior authors were 36.0% (n = 102) women, with no change over time. Women senior authors had a higher proportion of women first authors (67.7% vs. 32.4%, p = 0.017). No gender differences were noted based on article type or impact factor. The majority of authors came from institutions in the United States. Women had comparatively more NIH and CDC funding but received less funding from foundations and AHRQ. In PALISI publications, first authorship by women has increased over time, such that it now exceeds both the proportion of women pediatric intensivists and women first authors in critical care publications. Senior authorship by women has been stagnant. A multifactorial approach by individuals, institutions, networks, and journals is needed to bring senior women authors to parity.

Kids Lung Registry and Child-EU Project - Progress in Rare and Interstitial Lung Diseases in Childhood Through Collaboration

Progress in rare and interstitial lung disease in childhood can most usefully be achieved through systematic, registry-based collection. What are the practicalities and benefits of participating in the pediatric lung registry/chILD-EU project? We report our clinical experiences. Pediatricians and pediatric pulmonologists identify children with rare lung diseases. These are reported to the Kid's Lung Register after parental consent. Clinical data, imaging, and blood are sent to the registry. Genetic analysis can be arranged if desired. With completeness of the data, a peer-review process by pediatric radiology, possibly lung pathology, clinical and possibly genetic experts takes place in an interdisciplinary conference. A working diagnosis is established and communicated to the responsible physician via the registry and, if necessary, further discussed in case-related discussions. Assistance in entering the data is provided by the registry. Follow-ups are performed annually, and all registered physicians are invited to regular, web-based case discussions. Significant questions are answered in scientific projects and jointly published (>110 publications to date). Due to voluntary additional work of all participants beyond clinical routine, more than 1000 children with rare lung diseases have been included in the registry with biobank to date. A deeper understanding of the clinical courses of large cohorts of rare diseases and the initial description of new entities contributes to better care for these children.

Point-of-care ultrasound as a diagnostic tool in respiratory assessment in awake paediatric patients: a comparative study

BackgroundChest X-ray (CXR) has typically been the main investigation in children with suspected respiratory pathology. Recent advances in lung point-of-care ultrasound (POCUS) have shown the potential for it to be...

Unusual pediatric lung infections: imaging findings

Pediatric lung infections continue to be a leading cause of pediatric morbidity and mortality. Although both pediatric and general radiologists are familiar with typical lung infections and their imaging findings in children, relatively rare lung infections continue to present a diagnostic challenge. In addition, the advances in radiological imaging and emergence of several new lung infections in recent years facilitated the need for up-to-date knowledge on this topic. In this review article, we discuss the imaging findings of pediatric lung infections caused by unusual/uncommon and new pathogens. We review the epidemiological, clinical, and radiological imaging findings of viral (coronavirus disease 2019, Middle East respiratory syndrome, bird flu), bacterial (Streptococcus anginosus, Francisella tularensis, Chlamydia psittaci), and parasitic lung infections (echinococcosis, paragonimiasis, amoebiasis). Additional disorders whose clinical course and imaging findings may mimic lung infections in children (hypersensitivity pneumonitis, pulmonary hemorrhage, eosinophilic pneumonia) are also presented, to aid in differential diagnosis. As the clinical presentation of children with new and unusual lung infections is often non-specific, imaging evaluation plays an important role in initial detection, follow-up for disease progression, and assessment of potential complications.Graphical

Self-reported engagement in everyday activities following bilateral lung transplantation in paediatric cystic fibrosis: a single centre study

IntroductionFollowing lung transplantation, it is unknown how children/adolescents self-assess their performance in everyday activities, importance of these activities, and whether resumption of everyday activities influences self-reported quality of life. The...

Scenario Decision-Making About Plasma and Platelet Transfusion for Intracranial Monitor Placement: Cross-Sectional Survey of Pediatric Intensivists and Neurosurgeons.

To report pediatric intensivists' and pediatric neurosurgeons' responses to case-based scenarios about plasma and platelet transfusions before intracranial pressure (ICP) monitor placement in children with severe traumatic brain injury (TBI). Cross-sectional, electronic survey to evaluate reported plasma and platelet transfusion decisions in eight scenarios of TBI in which ICP monitor placement was indicated. Survey administered through the Pediatric Acute Lung Injury and Sepsis Investigators and the American Association of Neurologic Surgeons. Pediatric intensivists and pediatric neurosurgeons. None. A total of 184 participants responded (85 identified as pediatric intensivists and 54 as pediatric neurosurgeons). In all eight scenarios, the majority of respondents reported that they would base their decision-making about plasma transfusion on international normalized ratio (INR) alone (60-69%), or platelet transfusion on platelet count alone (83-86%). Pediatric intensivists, as opposed to pediatric neurosurgeons, more frequently reported that they would have used viscoelastic testing in their consideration of plasma transfusion (32% vs. 7%, p < 0.001), as well as to guide platelet transfusions (29 vs. 8%, p < 0.001), for the case-based scenarios. For all relevant case-based scenarios, pediatric neurosurgeons in comparison with pediatric reported that they would use a lower median (interquartile range [IQR]) INR threshold for plasma transfusion (1.5 [IQR 1.4-1.7] vs. 2.0 [IQR 1.5-2.0], p < 0.001). Overall, in all respondents, the reported median platelet count threshold for platelet transfusion in the case-based scenario was 100 (IQR 50-100) ×10 9 /L, with no difference between specialties. Despite little evidence showing efficacy, when we tested specialists' decision-making, we found that they reported using INR and platelet count in pediatric case-based scenarios of TBI undergoing ICP monitor placement. We also found that pediatric intensivists and pediatric neurosurgeons had differences in decision-making about the scenarios.

Infantile type I pleuropulmonary blastoma presenting with dyspnea due to compression by pneumothorax and an occupying tumor: a case report

BackgroundPleuropulmonary blastoma (PPB) is an extremely rare and malignant pediatric lung tumor. Purely cystic PPB has a more favorable prognosis than solid PPB, but may be difficult to distinguish from a certain type of “benign” congenital pulmonary airway malformation before and during surgery. The influence of tumor rupture on long life prognosis has not been clarified in detail.Case presentationA 5-month-old boy underwent emergency transfer from another hospital due to a left thoracic cystic lesion and left pneumothorax detected on chest radiography performed for persistent wheeze and cough. Contrast-enhanced computed tomography of the chest revealed marked deviation of the mediastinum to the right due to a giant cystic lesion and pneumothorax. Thoracotomy was performed on hospital day 2. A cystic lesion had developed from the distal alveolar region of lower lobe of the left lung and the tumor showed a tiny adhesion to the left diaphragm and a tiny rupture near the adhesion. Partial lung excision including the cyst and scraping of the adhesion were performed. Histopathological investigations revealed immature blast cell-like mesenchymal cells and differentiated striated muscle cells in a dense cambium layer were found under the epithelium of the cystic lesion. Type I PPB was diagnosed.ConclusionsSurgery should be performed with the possibility of type I PPB in mind when an extrapulmonary cystic lung lesion is found. Since issues such as the pathogenesis and long-term prognosis of ruptured cases remain unclear, continued careful follow-up of this case will be required.

Can Deep Adult Lung Segmentation Models Generalize to the Pediatric Population?

Lung segmentation in chest X-rays (CXRs) is an important prerequisite for improving the specificity of diagnoses of cardiopulmonary diseases in a clinical decision support system. Current deep learning models for lung segmentation are trained and evaluated on CXR datasets in which the radiographic projections are captured predominantly from the adult population. However, the shape of the lungs is reported to be significantly different across the developmental stages from infancy to adulthood. This might result in age-related data domain shifts that would adversely impact lung segmentation performance when the models trained on the adult population are deployed for pediatric lung segmentation. In this work, our goal is to (i) analyze the generalizability of deep adult lung segmentation models to the pediatric population and (ii) improve performance through a stage-wise, systematic approach consisting of CXR modality-specific weight initializations, stacked ensembles, and an ensemble of stacked ensembles. To evaluate segmentation performance and generalizability, novel evaluation metrics consisting of mean lung contour distance (MLCD) and average hash score (AHS) are proposed in addition to the multi-scale structural similarity index measure (MS-SSIM), the intersection of union (IoU), Dice score, 95% Hausdorff distance (HD95), and average symmetric surface distance (ASSD). Our results showed a significant improvement (p < 0.05) in cross-domain generalization through our approach. This study could serve as a paradigm to analyze the cross-domain generalizability of deep segmentation models for other medical imaging modalities and applications.