Purpose To compare and evaluate the usefulness of MRI with CT as radiation free alternative To compare the reproducibility of CT and MRI scores To compare MRI and CT scores with pulmonary function tests (PFT) To evaluate the role of DWI in bronchiectasis. Methods In this prospective study, 25 patients between 7-21 years of age with a clinical/radiological diagnosis of bronchiectasis underwent MDCT (with HRCT reconstruction) and MRI chest. MRI and CT scoring was performed using modified Bhalla score -Helbich’s score by two independent radiologists for all parameters. A final consensus score was recorded. both in CT and MRI. The overall image quality of different MRI sequences to identify the pathologies was also assessed. Appropriate statistical tests were used for inter-observer agreements, and correlation amongst CT and MRI; as well as CT, MRI and PFT. Results Strong agreement (ICC 0.80-0.95) between CT and MRI was seen for extent and severity of bronchiectasis, number of bullae, sacculation/abscess, emphysema, collapse/ consolidation, mucus plugging, and mosaic perfusion. Overall CT and MRI scores had perfect concordance (ICC 0.978). Statistically significant (p-value <0.01) intraobserver and interobserver agreement for all CT and MRI score parameters was seen. A strong negative correlation was seen between total CT and MRI severity scores and FEV1, FVC, FEF 25-75%. DWI MR, with an ADC cut-off of 1.62 x 10 -3 mm 3/sec had a sensitivity of 70% and specificity of 75% in detecting true mucus plugs. Conclusion MRI with DWI can be considered in the diagnostic algorithm for assessment of lung changes in bronchiectasis as a radiation-free non-invasive method of imaging in children, especially in follow-up.

Objective The objectives of this study were to determine the prevalence of insulin resistance (IR), dyslipidemia and metabolic syndrome (MS) in children with asthma, aged 10 to 15 years and to determine if these metabolic abnormalities showed an association with asthma symptom control and lung function. Methods We conducted a cross-sectional study at a tertiary centre in north India. Consecutive children with physician diagnosed asthma were enrolled. Asthma symptom control over previous four weeks was assessed as per GINA recommendations. Fasting plasma glucose, serum insulin and lipid levels were estimated. HOMA-IR was used as a marker of IR. Spirometry was performed for assessing lung function. Results Eighty-three children were enrolled. Median (IQR) age was 12.0 (11.0, 13.5) years and mean (SD) BMI z score was -0.42 (1.0). Median (IQR) Homeostasis Model Assessment- Insulin Resistance (HOMA-IR) was 1.65 (1.06, 2.39). Prevalence of IR was 42.3% (95% CI: 31.7-52.9%). Number of children with elevated triglycerides, total cholesterol, and LDL-cholesterol was 4 (4.8%), 4 (4.8%) and 5 (6%), respectively. 67 (80.7%) children had low HDL-cholesterol. Only one subject was found to have MS. Presence of IR and elevation in serum insulin and triglycerides were associated with poorer asthma control, independent of BMI. None of the metabolic parameters were associated with lung function, after adjusting for height. Conclusions A high proportion of children with asthma aged 10-15 years had IR but not MS currently. Increasing serum insulin, triglycerides, and presence of IR were associated with poorer asthma control, after adjusting for BMI.

Introduction: For the diagnosis of airway anomalies, bronchoscopy is the gold standard. Infant pulmonary function testing is an emerging modality to assess airways and its utility in diagnosis and monitoring is unexplored in clinical studies. Objectives: To evaluate infant Pulmonary Function Test [Tidal Breathing Flow Volume Loop (TBFVL)] in children with airway anomalies and to correlate with bronchoscopy findings. Methods: We performed a prospective cohort study from July 2018 to April 2020 in children from 0-2 years with physician suspected airway anomalies. We performed TBFVL (graphic pattern and parameters) and bronchoscopy in these children and correlated the results. The primary outcome measure was a graphic pattern of TBFVL in children with laryngomalacia. Secondary outcome measures were bronchoscopy diagnosis of various airway anomalies, graphic pattern in children with airway anomalies other than laryngomalacia, measurement of TBFVL parameters and measurement of change in TBFVL graphic patterns and parameters at six months follow up. We compared TBFVL parameters with controls without airway anomalies. Results: We enrolled 53 children with both Infant Pulmonary Function Tests and bronchoscopy data. Isolated laryngomalacia (28, 52.8%) was the most common airway anomaly, followed by laryngo-tracheomalacia (7, 13.2%), laryngo-tracheo-bronchomalacia (6, 11.3%), and laryngomalacia with subglottic stenosis (4, 7.5%). Among isolated laryngomalacia, pattern 3 (fluttering of inspiratory limb) was most common in TBFVL, followed by pattern 4 (fluttering of inspiratory limb and flattening of expiratory limb) in 13 (46.4) and 8 (28.6%) cases, respectively. There was no strikingly predominant pattern in other groups of bronchoscopy diagnoses. Among TBFVL parameters, the ratio of Ti/Te was significantly high in children with isolated laryngomalacia compared to controls. Compared to controls, the tPTEF/tE was significantly higher in laryngomalacia plus sub-glottic stenosis. At six months of follow-up, clinical symptoms improved significantly, TBFVL pattern 1 (normal) became the most common pattern, and expiratory time increased significantly among TBFVL parameters. Conclusion: A particular type of airway anomaly may have a characteristic graphic pattern in TBFVL. Further, the TBFVL pattern may indicate improvement in the follow-up either spontaneously or after an intervention.

Background: Pediatric bronchoscopy is an important tool in pediatric pulmonology. However, the practices involved in the procedure are variable. Objective: To evaluate prevalent practice and variations in pediatric flexible bronchoscopy in India. Methods: An online survey conducted between September 2018 to March 2019 via Google forms. The survey was sent to members of the Respiratory Chapter of IAP, personal contacts, and members of Indian Chest Society. Physicians performing flexible bronchoscopy in children were asked to respond. Survey had 95 questions in seven domains including patient preparation, sedation, procedural aspects, monitoring, bronchoscope cleaning, and complications. Results: The survey received 24 (14 in private sector) complete responses from 14 cities. Pediatric bronchoscopy was mainly done for diagnostic purposes. Conscious sedation was used by most (19, 79%). Midazolam plus fentanyl 9 (37.5%) was the preferred sedation regimen. Routine atropine was used by 4 (16%). For topical anaesthesia- nebulized only, both nebulized and spray as go, and spray as go lignocaine only was used by 1 (4.2%), 6 (25%), and 17 (71%) centres, respectively. The methods of providing oxygen during bronchoscopy were free flow (9,37.5%), nasal prongs (8,33.3%), mask (6,25%), and LMA (1,4.2%). Therapeutic procedures included removal of mucus plugs (17, 71%), bronchoscopic intubation (11, 45%) and foreign body removal (10, 41%). The suction for BAL included wall mounted suction in maximum (15, 62.5%). The number of aliquots for BAL varied from 2-6 and volume for each aliquot also varied (1-2 ml/kg or 5-10 ml). The complications rate of less than 5 % was reported by almost all. Conclusion: There is large variation in pediatric flexible bronchoscopy practices across the country highlighting the need to develop a uniform guideline.

Background: Childhood cancer survivors are 8.8 times more likely to die of pulmonary causes when compared to general population: an aspect of concern. Pulmonary dysfunction is the third leading cause of non recurrence related cause of death among Hodgkin lymphoma survivors. Methods: A cross section study on Hodgkin lymphoma survivors in complete remission, who completed treatment within last 5 years was done. All children were subjected to detail history including drugs, past history of respiratory illnesses, physical and respiratory system examination followed by spirometry and three minute step test under supervision. Pulmonary dysfunction was determined as presence of obstructive, restrictive or mixed pattern on spirometry or abnormality in three minute step test. Subclinical pulmonary dysfunction was determined as patients who were clinically asymptomatic but had pulmonary dysfunction Results: A total of 60 children were enrolled (Mean age of 11.3 years and 53 were boys) Abnormal pulmonary function tests were documented in 11 (18.3%) of HL survivors at a median time of 2 years (IQR 1,3) from treatment completion. Restrictive pattern was documented in 10 (16.67%) and obstructive pattern in only one patient (1.67%), mostly mild in severity. Older age at start of chemotherapy and radiotherapy and past history of respiratory illness were found to be significantly associated with pulmonary dysfunction. Conclusion: Majority of Hodgkin lymphoma survivors had subclinical pulmonary dysfunction at median follow up of 2 years from treatment completion. Hodgkin lymphoma survivors require long term follow up for timely detection of pulmonary dysfunction and improve quality of life.

Background: There is a lack of studies on outcomes in cystic fibrosis (CF) in children from developing countries like India. Identifying risk factors for mortality may help identify the high-risk group and plan policy management of such patients. Objective: To determine the factors associated with outcomes among Indian children with CF. Design: Retrospective analysis of data collected from January 2010 to Dec 2020. Setting: Tertiary care hospital in Northern India. Participants: Children diagnosed with CF during the study period. Methods: We extracted data related to demography, clinical features, laboratory data and outcome from children’s medical records with CF. Bivariate and multivariate analysis was performed to identify variables associated with mortality. Results: We enrolled 178 children, and there were 32 (18.0%) deaths. Significant factors associated with mortality included history of neonatal complications; hazard ratio (HR): 8.5 (95% CI, 3.0 - 23.9, p < 0.001), low Z-scores for body mass index (BMI) at the time of diagnosis; HR: 7.1 (95% CI 2.3 - 22.0, p < 0.001), FEV1/FVC at the time of diagnosis; HR: 5.1 (95% CI, 1.65 - 15.4, p-value < 0.004), and FEV1 25-75; HR: 3.6 (95% CI, 1.1- 11.8, p-value = 0.03). Conclusions: Factors associated with increased risk of mortality included presence of neonatal complications, low BMI and lower pulmonary function test results. Low BMI and low PFT indices are modifiable and possibly can be improved by early diagnosis. A new-born screening test may help in early diagnosis and identification of the neonatal problem of CF.

STAT5B protein is an important component of signalling pathway for growth hormone and IL-2 mediated responses. Mutation in STAT5B gene has been reported to be associated with growth hormone insensitivity, immunodeficiency and autoimmunity. Chronic pulmonary involvement is also one component of the disorder. We describe a case an adolescent girl who suffered with poor growth and loose stools since 1 year of age and was subsequently diagnosed with celiac disease and hypothyroidism. She developed features of interstitial lung disease (ILD) which were progressively worsening. Common causes of ILD were ruled out on investigations. She underwent whole exome sequencing and found to be positive for a novel mutation in STAT5B gene on exon 16. On further evaluation, she had growth hormone insensitivity and CD8 cell deficiency. This case highlights that in a child with chronic pulmonary involvement with features suggestive of growth hormone insensitivity, immunodeficiency and autoimmunity, evaluation for STAT5B mutation should be thought of.

Introduction: Data are scarce on hs-CRP as a biomarker for airway inflammation in pediatric asthma. We aimed to examine correlation between hs-CRP and asthma control levels. Methods: Children with physician-diagnosed asthma, ages 6 to 15 years, were enrolled. Asthma control criteria of GINA-2016 were used to assess the level of asthma control. The relationships between serum hs-CRP and each of asthma control measures (Asthma control criteria, spirometry, impulse oscillometry, eosinophil counts and Fractional exhaled nitric oxide (FeNO) were assessed. Results: Out of total 150 asthmatic children; 52 (35%) had well controlled asthma, 76 (51%), and 22 (14%) children had partly controlled and uncontrolled asthma, respectively. Median (IQR) values of hs-CRP were 0.47 (0.1, 1.67) mg/L in well controlled, 0.30 (0.1, 1.83) mg/L in partly controlled, and 2.74 (0.55, 3.74) mg/L in uncontrolled asthma (p=0.029). Correlation between hs-CRP and FEV1% was significant (p=0.019). Using Receiver Operator Characteristic (ROC) analysis, area under curve for hs-CRP mg/L to discriminate between uncontrolled and (controlled + partly controlled) asthma was 0.67 (95% CI 0.55, 0.80) and a cutoff 1.1 mg/L of serum hs-CRP level had a sensitivity of 68.1% with specificity of 67.97%. In two groups of hs-CRP (< 3 mg/L) and hs-CRP (≥ 3 mg/L), high hs-CRP group had higher proportion of uncontrolled asthmatic children (p=0.03). Conclusion: Our study demonstrated higher serum hs-CRP values in uncontrolled asthma that supports the use of hs-CRP as surrogate marker of small airway inflammation. hs-CRP may be considered as a tool to predict asthma status.

A document by Aruna Herath. Click on the document to view its contents.

Background: Endobronchial Ultrasound-guided Transbronchial Needle Aspiration (EBUS-TBNA) and Transesophageal Bronchoscopic Ultrasound-guided fine-needle aspiration (EUS-B-FNA) are established modalities for evaluation of mediastinal/hilar lymphadenopathy in adults. Limited literature is available on the utility of these modalities in the pediatric population. Herein, we perform a systematic review and meta-analysis on the yield and safety of EBUS-TBNA and EUS-B-FNA in children. Methods: We performed a systematic search of the PubMed and EMBASE databases to extract the studies reporting the utilization of EBUS-TBNA/EUS-B-FNA in children (<18 years of age). Pooled diagnostic yield and sampling adequacy (proportions with 95% confidence intervals) were calculated using the random-effects model. Details of any procedure-related complications were noted. Results: The search yielded 12 relevant studies (five case series and seven case reports on EBUS-TBNA/EUS-B-FNA, 173 patients). Data from five case series (164 patients) were summarized for the calculation of sampling adequacy and diagnostic yield. Safety outcomes were extracted from all publications. The pooled sampling adequacy and combined diagnostic yield of EBUS TBNA/EUS-B-FNA were 98% (95% CI, 92-100%) and 61% (95% CI, 43-77%) respectively. A procedure-related major complication was reported in 1 patient (1/173, Major complication rate 0.6%), and minor complications occurred in 6 patients (6/173, Minor complication rate 3.5%). Conclusions: EBUS-TBNA and EUS-B-FNA are safe modalities for evaluation of mediastinal lymphadenopathy in the pediatric population. EBUS-TBNA/EUS-B-FNA may be considered as the first-line diagnostic modalities for this indication as they have a good diagnostic yield and can avoid the need for invasive diagnostic procedures.