Pediatric Cystic Fibrosis Patients Research Articles

P110 A soft, flexible, wearable device for cough detection in paediatric cystic fibrosis patients

Objectives: New or increased cough is an important sign of pulmonary exacerbation. There is unmet need to objectively quantify and analyse cough trends in early life. Advances in flexible electronics and materials allow development of soft, skin-like, accelerometer-based wearable devices that seamlessly interface with the human body in unique locations, enabling the simultaneous capture of low-frequency body, chest, and throat motion along with high-frequency vocal, throat, and lung sound signals associated with various body processes including coughs and vital signs. Methods: A small, flexible, fully wireless, accelerometer-based mechano-acoustic sensor (MAS) was applied using gentle adhesives to the suprasternal notch of 10 paediatric CF subjects during regular clinic visits. A 15-minute protocol consisting of eliciting cough, throat clearing, speech, and laughs in various head/body orientations, ambient environments, and physical activity intensities. The MAS recorded at a high bandwidth 1.6kHz rate and automatically uploaded to a cloud server upon replacement on a wireless charging platform. Results: The captured sensor data was analysed using an existing machine learning algorithm developed for COVID-19 symptom tracking. Preliminary results show the device differentiates cough from other vocal, respiratory noises and motion artifacts, both at rest and during activity. A majority of children found the device to be acceptable, though device size and adhesive removal caused minor discomfort for some. Conclusion: The extraction of well-classified cough events is feasible, facilitating further analysis of cough episode duration, force, and clinically relevant features associated with early decompensation, response to treatment, and daily symptom tracking. Objective measurement of cough may be useful as a clinical study outcome measure and for clinical monitoring. Future work will include evaluation for longer time periods during stability and pulmonary exacerbations.

EPS2.07 How did the COVID-19 lockdown influence the nutritional status and body composition of cystic fibrosis patients in Poland?

Background: The global COVID-19 pandemic has changed daily life, due to restrictions affecting day-to-day functioning. The strict lockdown changed habits of cystic fibrosis (CF) patients as well. The aim of our study was to evaluate the impact of the one-month strict spring lockdown in Poland on the nutritional status and body composition of CF paediatric patients. Methods: All CF patients between 8 and 18 years old who had both in- and outpatient visits with completed data at the Cystic Fibrosis Centre in Warsaw were included in the study. BMI z-scores (CDC calculator), as well as the fat free mass index (FFMI) and the fat mass index (FMI) were calculated according to the following formulas: FFMI = FFM/height2, FMI = FM/height2. The strict lockdown (SL) in Poland was between 16.03.20 and 20.04.20. The measures were evaluated on average: 3 months before SL (M1);3 months after SL (M2), and 3 months after M2 (M3). Results: 101 CF subjects (51% female), with a mean age of 11.53 ± 3.29 years were assessed. 48% of them were heterozygotes F508del and 80% had pancreatic insufficiency. The highest significant increase in values of BMI z-score and FMI was observed after SL (M2) with decrease in M3 (F = 7.18;p = 0.001;F = 4.71;p = 0.01, respectively). FFMI increased significantly and continually from M1 to M3 (F = 14.17;p < 0.001). The mean values for BMI z-score, FMI and FFMI were, respectively, as follows: M1: [–0.20 ± 0.86;4.00 ± 1.51;13.81 ± 1.86];M2: [–0.05 ± 0.82;4.19 ± 1.54;14.05 ± 1.91];M3: [–0.14 ± 0.84;4.07 ± 1.46;14.17 ± 1.91]. The mean 3 months differences between measures [M2 vs M1] and [M3 vs M2] were negative for BMI z-score [–0.17 ± 0.49;p = 0.005];FMI [–0.21 ± 0.77;p = 0.006];FFMI [–0.07 ± 1.15;p = 0.56]. Conclusion: CF paediatric patients improved nutritional status after strict lockdown with increase both FMI and FFMI, nevertheless a decline with time was observed in BMI z-scores and FMI, but not for FFMI.

Decreased Wait Time and Increased Satisfaction With Bedside Pancreatic Enzyme Dosing for the Inpatient Adolescent With Cystic Fibrosis: A Quality Improvement Project Comparing Enzyme Self-Administration to Nurse Administration.

For children with cystic fibrosis (CF), enzymes are essential with meals to absorb nutrients and ensure adequate growth. When hospitalized, CF patients typically rely on nurse-administered medications. Recently, a pediatric hospital unit began allowing adolescents with CF enzymes at the bedside. Postimplementation, a satisfaction questionnaire was administered to participating patients and nurses measuring patient and nurse satisfaction with access to bedside enzymes versus nurse administration and overall time for enzyme delivery. The survey utilized a 5-point Likert scale. The wait time for pancreatic enzymes decreased for self-administered enzymes when compared to those that were nurse administered. All (11/11) patients and 86% (12/14) of nurses preferred the self-administration of enzymes. Hospitalized pediatric CF patients and nurses had higher levels of satisfaction with enzyme self-administration. Immediate access to enzymes in room safes impact patient autonomy, reflecting home self-care practices. Decreases in wait times optimize nutritional growth and healing while hospitalized. As a result, a new limited scope policy allowing patient-administered enzymes is now in place in the pediatric inpatient CF unit.

Trajectories of oral glucose tolerance testing in cystic fibrosis.

Annual oral glucose tolerance testing (OGTT) is the recommended screening modality for cystic fibrosis-related diabetes (CFRD) in patients with cystic fibrosis (CF). This study aimed to determine if there were patterns of progression of worsening glucose homeostasis in pediatric CF patients and to explore any relationship to lung function. We conducted a retrospective cohort study of CF patients, ages 10-18 years, without CFRD and with ≥3 OGTT from 2013 to 2016. Latent class mixture models were used to determine unique trajectories of 2-hOGTT glucose values (2hrGlu) over time. Multivariable linear models were used to adjust for clinical covariates. For 63 subjects, three unique 2hrGlu trajectories were identified: high (impaired glucose tolerance) to higher (n = 8), low (normal glucose tolerance [NGT]) and increasing (n = 47), and low (NGT) and flat (n = 8). There was high variability of 2hrGlu, but most patients belonged to a trajectory that increased over time. After controlling for age, pancreatic insufficiency, modulator use, and mutation type, there was a significant difference in the study baseline forced expiratory volume in 1 s percent predicted (ppFEV1) in the high to higher group compared to the low and increasing and low and flat groups (p < .005). Among pediatric CF patients without diabetes, three 2hrGlu trajectories were identified with 87% of patients exhibiting a trajectory where glucose homeostasis worsened over time. Starting ppFEV1 was lower in those with a high to higher trajectory, supporting that lower lung function is present early in the development of CFRD.

Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis

BackgroundTwo functional measurements (multiple breath washout [MBW] and hyperpolarized 129Xe ventilation magnetic resonance imaging [129Xe MRI]) have been shown to be more sensitive to cystic fibrosis (CF) lung obstruction than traditional spirometry. However, functional techniques may be sensitive to different underlying structural abnormalities. The purpose of this study was to determine relationships between these functional markers, their pathophysiology, and 1-year clinical outcomes. MethodsSpirometry, MBW, 129Xe MRI, and ultrashort echo-time (UTE) MRI were obtained in a same-day assessment of 27 pediatric CF patients (ages 11.5±5.0) who had not begun CFTR modulator therapies. UTE MRI was scored for structural abnormalities and functional metrics obtained via spirometry, MBW and 129Xe MRI. 1-year outcomes (ΔFEV1 and pulmonary exacerbations), during which ≈50% initiated modulator therapy, were obtained from the electronic medical record. ResultsMBW, 129Xe MRI, and UTE MRI detected clinically significant disease in more subjects (>78%) compared to spirometry (<30%). UTE MRI suggests increased odds of bronchial changes when mucus plugging is present in the same lobe. MBW and 129Xe MRI correlated best with mucus plugging, while spirometry correlated best with consolidations. Bronchial abnormalities were associated with future pulmonary exacerbations. ConclusionsMBW, 129Xe MRI, and UTE MRI are more sensitive for detection of pediatric CF lung disease when compared to spirometry. MBW and 129Xe MRI correlated with structural abnormalities which occur in early CF disease, suggesting MBW and 129Xe MRI are valuable tools in mild CF lung disease that can guide clinical decision making.

Acute hyperglycaemia in cystic fibrosis pulmonary exacerbations.

BackgroundHyperglycaemia may contribute to failure to recover from pulmonary exacerbations in cystic fibrosis (CF). We aimed to evaluate the prevalence and mechanism of hyperglycaemia during and post‐exacerbations.MethodsNine paediatric CF patients, not on insulin, hospitalized for intravenous antibiotics, underwent an oral glucose tolerance test (OGTT) and continuous glucose monitoring (CGM) upon admission (visit 1) and an OGTT 2 weeks (visit 2) and 6 weeks to 12 months later when at stable baseline (visit 3). Insulin and glucose levels were measured before, 30, 60 and 120 min after glucose ingestion during OGTT. Hyperglycaemia on OGTT was defined according to the American Diabetes Association criteria as abnormal OGTT or consistent with diabetes. Hyperglycaemia on CGM was defined as CGM time above 140 mg/dL > 4.5%.ResultsAt visit 1, 8/9 patients had hyperglycaemia on both CGM and OGTT (2 diabetes and 6 abnormal OGTT). At visit 2, 5/8 had hyperglycaemia (all abnormal OGTT). At visit 3, (median (IQR) time since visit 1, 4.9 (3.8‐6.3) months), 5/7 had hyperglycaemia (2 diabetes and 3 abnormal OGTT). At visits 1, 2 and 3, respectively, mean (SD) 2‐hour OGTT glucose was 175.8 (42.3), 146.3 (31.9) and 176.9 (51.7) mg/dL. CGM time above 140 mg/dL at visit 1 was 25.3% (16.9). Insulin AUC decreased from visit 2 (median (IQR) 5449 (3321‐8123) mcIU‐min/mL) to visit 3 (3234 (2913‐3680) mcIU‐min/mL).ConclusionHyperglycaemia is prevalent during paediatric CF exacerbations; it appears to improve with exacerbation treatment but to worsen later in association with decreased insulin secretion.

Local and Systemic Alterations of the L-Arginine/Nitric Oxide Pathway in Sputum, Blood, and Urine of Pediatric Cystic Fibrosis Patients and Effects of Antibiotic Treatment.

Alterations in the L-arginine (Arg)/nitric oxide (NO) pathway have been reported in cystic fibrosis (CF; OMIM 219700) as the result of various factors including systemic and local inflammatory activity in the airways. The aim of the present study was to evaluate the Arg/NO metabolism in pediatric CF patients with special emphasis on lung impairment and antibiotic treatment. Seventy CF patients and 78 healthy controls were included in the study. CF patients (43% male, median age 11.8 years) showed moderately impaired lung functions (FEV1 90.5 ± 19.1% (mean ± SD); 21 (30%) had a chronic Pseudomonas aeruginosa (PSA) infection, and 24 (33%) had an acute exacerbation). Plasma, urinary, and sputum concentrations of the main Arg/NO metabolites, nitrate, nitrite, Arg, homoarginine (hArg), and asymmetric dimethylarginine (ADMA) were determined in pediatric CF patients and in healthy age-matched controls. Clinical parameters in CF patients included lung function and infection with PSA. Additionally, the Arg/NO pathway in sputum samples of five CF patients was analyzed before and after routine antibiotic therapy. CF patients with low fractionally exhaled NO (FENO) showed lower plasma Arg and nitrate concentrations. During acute exacerbation, sputum Arg and hArg levels were high and dropped after antibiotic treatment: Arg: pre-antibiotics: 4.14 nmol/25 mg sputum vs. post-antibiotics: 2.33 nmol/25 mg sputum, p = 0.008; hArg: pre-antibiotics: 0.042 nmol/25 mg sputum vs. post-antibiotics: 0.029 nmol/25 mg sputum, p = 0.035. The activated Arg/NO metabolism in stable CF patients may be a result of chronic inflammation. PSA infection did not play a major role regarding these differences. Exacerbation increased and antibiotic therapy decreased sputum Arg concentrations.

Gastroesophageal Reflux Disease and Need for Antireflux Surgery in Children with Cystic Fibrosis: A Systematic Review on Incidence, Surgical Complications, and Postoperative Outcomes.

Introduction Gastroesophageal reflux disease (GERD) is associated with accelerated decline in lung health in children with cystic fibrosis (CF). Thus, antireflux surgery (ARS) is offered to a selected CF cohort with refractory GERD, but outcomes remain poorly investigated. This study aimed to determine the incidence of GERD in children with CF and to evaluate complications and outcomes of ARS. Materials and Methods A systematic literature-based search was conducted using various online databases according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The number of GERD cases in pediatric CF cohorts who underwent diagnostic investigation(s) was recorded. Data on postoperative complications and outcomes (including symptoms, lung function, and nutritional status) following ARS were analyzed. Results Ten articles ( n = 289 patients) met the defined inclusion criteria (51% male; age range, 0.5 month–36 years). The overall incidence of GERD was 46% (range, 19–81%), derived from seven studies ( n = 212 patients). Four publications ( n = 82 patients) reported on ARS due to uncontrolled GERD. All ARSs were Nissen fundoplication (majority with gastrostomy placement). Major postoperative complications occurred in 15 (18%) patients, two required redo-ARS. Median follow-up time was 2 years (range, 3 months–6 years); 59% showed symptom improvement, and pulmonary exacerbations and decline in lung function were reduced. Nutritional status mainly improved in milder CF cases. There were no deaths related to ARS. Conclusion Approximately half of pediatric CF patients have GERD. Published data for children with CF are limited and heterogeneous in terms of GERD diagnosis and outcomes following ARS. However, ARS has shown to slow the deterioration of lung function in CF.

Incidence of nephrotoxicity with prolonged aminoglycoside exposure in patients with cystic fibrosis

Cystic fibrosis (CF) patients, with Pseudomonas aeruginosa infection, often require repeated aminoglycoside courses for the management of acute pulmonary exacerbations (APEs). Acute kidney injury (AKI) due to aminoglycosides has been reported; little data exist regarding long-term nephrotoxicity with repeated exposure. The objective of this study was to describe the incidence of acute and chronic nephrotoxicity due to cumulative intravenous (IV) aminoglycoside exposure. This is a retrospective, observational study of pediatric and adult CF patients admitted to an academic medical center between January 1, 2006 and October 1, 2018 for APE management. Patients were eligible for inclusion if they received at least five courses of an IV aminoglycoside for at least 7 days each. Cumulative weight-based aminoglycoside dose was reported in milligrams per kilogram. For each admission, baseline and highest serum creatinine were collected to assess the incidence of AKI. The baseline and final estimated glomerular filtration rate (eGFR) were calculated to assess long-term effects on renal function. Sixty-six patients, representing greater than 700 courses, were included in the final analysis. The median cumulative weight-based aminoglycoside dose was 1183 mg/kg of tobramycin or tobramycin equivalent. Twenty percent of courses resulted in AKI; 86% were Stage 1. A repeated measure multivariate model showed colistin, piperacillin/tazobactam, vancomycin, and age were significant AKI risk factors. There was no correlation between cumulative aminoglycoside dose and change in eGFR. AKI from IV aminoglycoside exposure occurred in 20% of courses. Cumulative exposure to IV aminoglycosides in APE management was not correlated with long-term renal dysfunction.

Utility of Mid‐Upper Arm Circumference in Diagnosing Malnutrition in Children With Cystic Fibrosis

Standardized diagnostic indicators for malnutrition using growth percentile z-scores (weight for length or body mass index [BMI]) and mid-upper arm circumference (MUAC) z-scores are being used in clinical practice; however, their application to the pediatric cystic fibrosis (CF) population is not well described. In this study, we aim to compare growth percentile z-scores and MUAC z-scores in diagnosing and classifying malnutrition in children with CF and assess the relationship between their degree of malnourishment and corresponding pulmonary function tests (PFTs). In this retrospective observational outpatient study of 49 pediatric CF patients, data were collected on baseline characteristics, anthropometrics, and PFTs over 12 months. Agreement in malnutrition diagnoses was quantified by Cohen κ statistics. Pearson test assessed the correlation between MUAC and BMI z-scores as well as PFTs and anthropometrics. Serial anthropometrics and PFTs were obtained and compared for a subset of patients (n = 28). Growth percentile and MUAC z-scores were positively correlated in diagnosing malnutrition (Pearson correlation r = 0.87), but MUAC z-scores identified more patients as malnourished compared with growth percentile z-scores (49% vs 12%, Cohen κ of 0.22 [95% CI, 0.04-0.40]). There was no significant relationship between anthropometrics and PFTs. MUAC z-scores increased significantly over time, but BMI z-scores did not show this trend. Our small-scale data suggest a promising role for MUAC z-scores in classifying malnutrition and in measuring changes in nutrition status over time in pediatric CF.

Adherence to Dietary Recommendations, Nutrient Intake Adequacy and Diet Quality among Pediatric Cystic Fibrosis Patients: Results from the GreeCF Study.

Nutrition is an important component of cystic fibrosis (CF) therapy, with a high-fat diet being the cornerstone of treatment. However, adherence to the dietary recommendations for CF appears suboptimal and burdensome for most children and adolescents with CF, leading to malnutrition, inadequate growth, compromised lung function and increased risk for respiratory infections. A cross-sectional approach was deployed to examine the degree of adherence to the nutrition recommendations and diet quality among children with CF. A total of 76 children were recruited from Aghia Sophia’s Children Hospital, in Athens, Greece. In their majority, participants attained their ideal body weight, met the recommendations for energy and fat intake, exceeding the goal for saturated fatty acids consumption. Carbohydrate and fiber intake were suboptimal and most participants exhibited low or mediocre adherence to the Mediterranean diet prototype. It appears that despite the optimal adherence to the energy and fat recommendations, there is still room for improvement concerning diet quality and fiber intake.

Factors influencing clinical trial participation for adult and pediatric patients with cystic fibrosis

There remains a limited understanding of the factors influencing clinical trial participation for individuals with Cystic Fibrosis (CF). A comprehensive survey was developed to examine the interests, preferences, and barriers/facilitators to research and clinical trial participation for CF patients. A consecutive sample of 198 CF adults attending the St. Paul's Hospital CF Clinic and parents of children with CF attending the BC Children's Hospital CF Clinic from Vancouver, Canada were surveyed. Parents of pediatric patients were less comfortable with blood collection, required more safety data prior to participating, and were more concerned about potential side effects. Very few respondents (<10%) appeared able/willing to fulfill the typical requirements to participate in a phase 1 clinical trial. Overall, there were more similarities than differences between the responses of adult and parents of pediatric CF patients. The patient-centered information can be used to inform the design of future clinical trials to enhance feasibility.

Ceftaroline vs vancomycin for the treatment of acute pulmonary exacerbations in pediatric patients with cystic fibrosis.

Respiratory infection with methicillin-resistant Staphylococcus aureus (MRSA) is an increasing complication in cystic fibrosis (CF) that results in accelerated lung function decline and mortality. Vancomycin is considered a first-line intravenous treatment agent for MRSA associated acute pulmonary exacerbations (APEs); however, rates of vancomycin intolerance and resistance have been observed. These factors have led to the exploration of additional treatment options for treating MRSA associated APEs. This is a retrospective chart review conducted at a CF center including patients 0 to 21 years of age with CF admitted for an APE and treated with either vancomycin or ceftaroline between January 2016 and August 2018. The primary endpoint was to determine ceftaroline efficacy compared to vancomycin in the treatment of MRSA associated APEs. There were 180 patients included in the study with 90 patients in each antibiotic group. Admission to discharge forced expiratory volume in 1 second (FEV1 ) improved in the ceftaroline (66.5% vs 81.1%; P < .001) and vancomycin (65.5% vs 77.3%; P < .001) treatment groups. No difference existed in mean change in FEV1 (14.1% vs 13.5%; P = .25) or readmissions (15% vs 22; P = .27) between ceftaroline and vancomycin groups, respectively. In this retrospective study, no difference existed between ceftaroline and vancomycin with regard to observed improvement in lung function from admission to discharge. Additionally, no difference was observed in mean FEV1 or readmission rate between the two groups. Ceftaroline may represent an effective and safe intravenous antimicrobial option for targeting MRSA in pediatric CF patients with APEs.

Choline in cystic fibrosis: relations to pancreas insufficiency, enterohepatic cycle, PEMT and intestinal microbiota.

Cystic Fibrosis (CF) is an autosomal recessive disorder with life-threatening organ manifestations. 87% of CF patients develop exocrine pancreas insufficiency, frequently starting in utero and requiring lifelong pancreatic enzyme substitution. 99% develop progressive lung disease, and 20-60% CF-related liver disease, from mild steatosis to cirrhosis. Characteristically, pancreas, liver and lung are linked by choline metabolism, a critical nutrient in CF. Choline is a tightly regulated tissue component in the form of phosphatidylcholine (Ptd'Cho) and sphingomyelin (SPH) in all membranes and many secretions, particularly of liver (bile, lipoproteins) and lung (surfactant, lipoproteins). Via its downstream metabolites, betaine, dimethylglycine and sarcosine, choline is the major one-carbon donor for methionine regeneration from homocysteine. Methionine is primarily used for essential methylation processes via S-adenosyl-methionine. CF patients with exocrine pancreas insufficiency frequently develop choline deficiency, due to loss of bile Ptd'Cho via feces. ~ 50% (11-12g) of hepatic Ptd'Cho is daily secreted into the duodenum. Its re-uptake requires cleavage to lyso-Ptd'Cho by pancreatic and small intestinal phospholipases requiring alkaline environment. Impaired CFTR-dependent bicarbonate secretion, however, results in low duodenal pH, impaired phospholipase activity, fecal Ptd'Cho loss and choline deficiency. Low plasma choline causes decreased availability for parenchymal Ptd'Cho metabolism, impacting on organ functions. Choline deficiency results in hepatic choline/Ptd'Cho accretion from lung tissue via high density lipoproteins, explaining the link between choline deficiency and lung function. Hepatic Ptd'Cho synthesis from phosphatidylethanolamine by phosphatidylethanolamine-N-methyltransferase (PEMT) partly compensates for choline deficiency, but frequent single nucleotide polymorphisms enhance choline requirement. Additionally, small intestinal bacterial overgrowth (SIBO) frequently causes intraluminal choline degradation in CF patients prior to its absorption. As adequate choline supplementation was clinically effective and adult as well as pediatric CF patients suffer from choline deficiency, choline supplementation in CF patients of all ages should be evaluated.

Extended Screening for Cystic Fibrosis-related Liver Disease Including Elastography in Children and Adolescents.

Advances in treatment of cystic fibrosis (CF) have increased survival and thereby prevalence of patients with liver disease, making chronic liver disease one of the major complications of CF. We describe the prevalence of liver fibrosis, portal hypertension, and liver decompensation by extended screening for cystic fibrosis-related liver disease (CFLD) including ultrasound, elastography, and an extended panel of biochemical markers. A cross sectional study of CFLD in all pediatric CF patients (1-18 years) from the Copenhagen CF Center. Screening for liver disease included physical examination, biochemical analysis, Vibration-Controlled Transient Elastography (FibroScan), conventional ultrasound, and Real-Time Shear Wave elastography (SWE). Patients were scored according to Williams ultrasound scoring scale (WUSS) within 6 months. A total of 84 consecutive patients (male sex 46.4%, median age 10.4 years) were included. Eight patients (9.5%) had both ≥2 abnormal results of sonographic methods and ≥2 abnormal biochemical results and were in this study categorized as having manifest CFLD. Manifest CFLD patients were significantly older and had a higher mean value of APRI, but no differences in gender, z-height, z-weight, z-BMI, FEV1%, or mean value of bilirubin or albumin were found. In total, 8 patients (9.5%) in this pediatric CF population were categorized as having CFLD according to both biochemical and sonographic tests. Consistency was found among the results of FibroScan and SWE. We suggest WUSS and either FibroScan or SWE, combined with GGT as diagnostic markers for CFLD.

Utility of Second-Look Endoscopy with Debridement After Pediatric Functional Endoscopic Sinus Surgery in Patients with Cystic Fibrosis.

To determine the utility of Second-look endoscopy with debridement (SLED) after functional endoscopic sinus surgery (ESS) in pediatric cystic fibrosis (CF) patients. To compare outcomes in pediatric CF patients undergoing sinus surgery for chronic sinusitis with or without SLED. To describe findings present at the time of SLED. Retrospective chart review of 61 ESS procedures performed at a tertiary care pediatric center from 2013 to 2016. Data collected included demographics, SLED findings, and 6-month pre-/postoperative disease specific outcomes including incidence of sinonasal and pulmonary exacerbations and revisions. Sixty-one cases were reviewed. SLED was performed in 38 cases on average 22.4 days postoperatively. Average preoperative Lund-Mackay score was 14.9 and 14.8 among patients undergoing ESS with and without SLED, respectively. Pre-/postoperative intranasal steroid use and extent of surgery performed was similar among all patients. At the time of SLED, rates of synechiae, polyps and maxillary antrostomy obstruction were 26.3%, 23.7%, and 7.9%, respectively. The incidence and number of days to onset of postoperative sinonasal exacerbations requiring antibiotic therapy within 6 months of ESS were 1.0 (SD 1.0) and 85 days (SD 45.7); and 1.3 (SD 1.0) and 80.4 days (SD 40.5) for patients undergoing ESS with and without SLED, respectively (P value .33). The number of days to first pulmonary exacerbation was 113.9 (SD 45.5) and 47.4 (SD 34.1) among SLED and non-SLED patients, respectively (P value .01). No significant difference was observed in revision rates and time to revision ESS (30% and overall average 1.4 years, respectively). The utility of SLED among pediatric CF patients remains unclear. While debridement did not have a significant impact on sinonasal exacerbations or revision rates, pulmonary exacerbations for patients undergoing SLED were delayed. Further studies are needed to clarify the impact of SLED.

Evaluation of a New Culture Protocol for Enhancing Fungal Detection Rates in Respiratory Samples of Cystic Fibrosis Patients.

Cystic fibrosis (CF) can be complicated by fungal infection of the respiratory tract. Fungal detection rates in CF sputa are highly dependent on the culture protocol and incubation conditions and thus may lead to an underestimation of the true prevalence of fungal colonization. We conducted a prospective study to evaluate the additional value of mucolytic pre-treatment, increased inoculum (100 μL), additional fungal culture media (Sabouraud agar; SAB, Medium B+, Scedosporium selective agar; SceSel+ and Dichloran-Glycerol agar; DG18) and longer incubation time (3 weeks) compared with our current protocol. Using the new protocol, we prospectively analyzed 216 expectorated sputum samples from adult and pediatric CF patients (n = 77) and compared the culture yield to a three year retrospective cohort that used direct 10 μL loop inoculation on SAB with 5 days incubation (867 sputum samples/103 patients). Detection rates for molds increased from 42% to 76% (p < 0.0001). Twenty-six percent of cultures were polymicrobial in the prospective cohort as opposed to 4.7% in the retrospective cohort (p < 0.0001). Colonization rate with A. fumigatus increased from 36% to 57%. SAB and DG18 showed the highest detection rates for all molds (SAB 58.6%; DG18 56.9%) and DG18 had the best performance for molds other than A. fumigatus. The larger sample volume and longer incubation also contributed to the increased recovery of molds. The introduction of a modified fungal culture protocol leads to a major increase in detection rate and the diversity of molds, which influences fungal epidemiology and may have implications for treatment decisions.

WS10.5 Dietary patterns and body composition in paediatric cystic fibrosis patients: preliminary study

WS10.5 Dietary patterns and body composition in paediatric cystic fibrosis patients: preliminary study

P100 Mycobacterium abscessus in paediatric cystic fibrosis patients in a reference centre in Argentina

P100 Mycobacterium abscessus in paediatric cystic fibrosis patients in a reference centre in Argentina

Tolerance of 7% Hypertonic Saline in Pediatric Cystic Fibrosis Patients.

Background: Clinical trials of 7% hypertonic saline (HTS) in cystic fibrosis (CF) show short- and long-term benefits, including improved pulmonary function and reduced exacerbation risk. Adverse effects of HTS include bronchospasm, and guidelines recommend tolerance be tested in a clinical environment before prescribing. We evaluated the rate of, and risk factors for, intolerance of HTS at a single pediatric CF program. Methods: Single-center retrospective study in patients with CF, aged 6-20 years, who received an HTS test dose between 2006 and 2017. HTS intolerance was defined as either a ≥10% decline in forced expiratory volume in 1 s (FEV1) percent predicted or wheezing/severe cough. Results: Fifty-one patients met inclusion criteria, and 13 (25%) showed intolerance of 7% HTS. There were trends toward higher rates of comorbidities in the patients intolerant versus tolerant of HTS, including allergies and/or rhinitis 85% versus 66% (P = 0.30) and sinus disease 85% versus 58% (P = 0.10). A trend toward more methicillin-sensitive Staphylococcus aureus (58% vs. 31%, P = 0.09) and asthma (42% vs. 24%, P = 0.09) was seen in patients tolerant of HTS. Demographics, pulmonary function, nutritional parameters, laboratory tests, respiratory cultures, chronic therapies, and antibiotics for exacerbations did not differ. Conclusions: In our program, 1:4 children with CF exhibited objective findings of HTS intolerance. There were trends suggesting higher frequency of allergies and rhinosinusitis in intolerant children. Assessing tolerance of HTS before prescribing chronic daily therapy is important. Larger studies are needed to more accurately define the incidence of intolerance and risk factors.