Comparison of International Growth Standards for Assessing Nutritional Status in Cystic Fibrosis: The GreeCF Study.

OBJECTIVE: The aim of the study was to compare 3 international growth references and explore their differences in assessing growth in Greek school-aged patients with cystic fibrosis (CF). METHODS: Sample included 114 patients (50 boys, age 11.5 ±â€Š3.9 years), provided care at Aghia Sofia Children's Hospital, Greece. Anthropometrics and predicted forced expiratory volume in 1 second (FEV1%) were measured. Body mass index (BMI) and height z scores were computed according to the Centers for Disease Control and Prevention (CDC), World Health Organization (WHO), and International Obesity Task Force (IOTF) references. Agreement between methods was analyzed with kappa statistics, repeated-measures analysis of variance, and Bland-Altman analysis. The relationship between FEV1% and BMI was explored with linear regression. RESULTS: Mean CDC BMI z score was the lowest (0.06 ±â€Š1.08), followed by WHO (0.17 ±â€Š1.14) and IOTF (0.35 ±â€Š1.05) (P ≤ 0.001 for all). The CDC and WHO growth references highly agreed for most weight status strata and stunting; all other comparisons produced lower agreements. Except for CDC and IOTF BMI z scores, all other comparisons produced wide levels of agreement and proportional bias. CDC reference classified more children as attaining low or normal weight, against WHO or IOTF (P ≤ 0.001 for all). Lowest prevalence of ideal and excess weight was recorded by CDC, compared to all other standards (P ≤ 0.001 for all). All BMI z scores provided moderate associations with FEV1%. CONCLUSION: Large variations across weight status classification were present when employing 3 growth standards in school-aged patients . Given than BMI z-scores from all references provided comparable associations with pulmonary function, our data indicate that no studied reference is better than others in assessing growth in CF.

Isolation of Candida auris from cystic fibrosis patient, Greece, April 2019.

We report the first isolation of Candida auris in Greece from a sputum culture of a cystic fibrosis patient in their 20s under posaconazole treatment. The pathogen was identified as C. duobushaemulonii by VITEK2YST, but as C. auris by MALDI-TOF MS. This case underscores the need for species-level identification of all non-albicans Candida (NAC) isolates from cystic fibrosis patients and patients with predisposing factors to fungal infection.

Body composition and lung function in children with cystic fibrosis and meconium ileus.

The aim of this study was to explore whether history of meconium ileus (MI) at birth in children and adolescents with cystic fibrosis (CF) adversely affects body composition and lung function in later life. Data of children and adolescents with CF who underwent spirometry and DXA as part of their routine care were analyzed. Associations between MI (explanatory variable) and areal bone mineral density (total body less head-TBLH aBMD), lean tissue mass (LTM), and fat mass (FM) (outcomes) were assessed using general linear models. Potential relationships of TBLH aBMD, LTM, and FM with FEV1 (additional outcome) were also explored. One hundred and one subjects with CF (mean age 14 ± 3 years) were included, 19 (18.8%) of whom had history of MI. Negative associations were demonstrated between history of MI and FEV1 (P = 0.04), TBLH aBMD (P = 0.03), and FM (P < 0.01) but not between history of MI and LTM (P = 0.07) after adjustment for other variables. Lung function was positively associated with TBLH aBMD (P < 0.01) and LTM (P = 0.02) but not with FM (P = 0.20). CONCLUSION: Among children and adolescents with CF, those with history of MI have lower bone mineral density, FM, and lung function. What is Known: • Among children and adolescents with cystic fibrosis, those with history of meconium ileus in the neonatal period are at risk of having lower body mass index percentile and FEV 1 percent predicted. What is New: • Children and adolescents with cystic fibrosis and history of meconium ileus have decreased bone mineral density and fat mass compared to patients without such history. • Lower lung function in children with MI coexists with suboptimal bone mineral density.

Dietary intake, weight status, pulmonary function, and metabolic profile in children with cystic fibrosis with or without pancreatic sufficiency.

OBJECTIVE: Nutritional status and growth is well associated with disease outcomes and lung function in patients with cystic fibrosis (CF). Current dietary guidelines for the management of CF suggest a high-calorie, high-fat diet. Pancreatic insufficiency (PI) is present in most patients and contributes to malabsorption and malnutrition, but a considerable number of patients have pancreatic sufficiency (PS). The aim of this study was to compare weight status, clinical characteristics, and dietary intake of children with CF, with PS or PI. METHODS: Patients with a diagnosis of CF (sweat test ≥60 mmol/L) and/or two known mutations for CF, ages 1 to 19 y were included in the study. Weight status, pulmonary characteristics, and blood lipid concentrations were evaluated. Dietary intake was evaluated through four 24-h recalls and energy, macronutrient intake, and intake in terms of food groups were assessed. RESULTS: Included in the present analyses were 134 patients with CF (30 with PS and 104 with PI). The percentage of overweight/obesity (47%) was higher in children with PS than in those with PI (22%). Overall, children with PS had higher body mass index, blood lipid levels, and pulmonary function levels than those with PI (all P < 0.05). Total energy intake was lower in children with PS than in those with PI (P < 0.001), even after adjustment for age and sex (Padj < 0.001). CONCLUSIONS: Weight status, dietary intake, pulmonary function, and lipid profile differed significantly in children with CF by pancreatic status. Nevertheless, the percentage of overweight and obesity was higher in children with PS than in those with PI. To avoid obesity, dietary recommendations for a high-calorie, high-fat diet should be reconsidered in patients with CF regarding their pancreatic status.

Inflammation and Infection in Cystic Fibrosis: Update for the Clinician.

Inflammation and infection play an important role in the pathophysiology of cystic fibrosis, and they are significant causes of morbidity and mortality in CF. The presence of thick mucus in the CF airways predisposes to local hypoxia and promotes infection and inflammation. A vicious cycle of airway obstruction, inflammation, and infection is of critical importance for the progression of the disease, and new data elucidate the different factors that influence it. Recent research has been focused on improving infection and inflammation in addition to correcting the basic gene defect. This review aims to summarize important advances in infection and inflammation as well as the effect of new treatments modulating the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein. New approaches to target infection and inflammation are being studied, including gallium, nitric oxide, and phage therapy for infection, along with retinoids and neutrophil elastase inhibitors for inflammation.

Impact of COVID-19 Lockdown on Pulmonary and Nutritional Status in Children and Young Adults With Cystic Fibrosis, in Greece.

During the first wave of the coronavirus pandemic in 2020, Greece adopted strict lockdown measures. We aimed to investigate the effects of lockdown and the resultant changes in the standard of care, on the lung function and somatic growth of cystic fibrosis (CF) patients. We analyzed data on body mass index and lung function of 103 CF patients 5.0- to 23.0-years-old before and after the lockdown period. Body mass index did not change significantly, but there was a significant improvement in lung function after the end of the lockdown period.

Pancreatitis in A Patient with Cystic Fibrosis Taking Ivacaftor.

Pancreatitis is rare in pancreatic insufficient cystic fibrosis patients. While pancreatic insufficiency has been considered irreversible until now, in the current era of new therapies with modulators of the Cystic Fibrosis Transmembrane Regulator CFTR channel, there are reports of improvement of pancreatic exocrine function. We describe the case of an adolescent with cystic fibrosis who developed pancreatitis after the partial recovery of pancreatic function while taking ivacaftor. This case adds to the limited body of evidence that CFTR modulators lead to the improvement of pancreatic exocrine function in cystic fibrosis.

Nebulizer Care and Inhalation Technique in Children with Cystic Fibrosis.

Nebulizers are used by the great majority of cystic fibrosis patients for delivery of cornerstone treatments. Inhalation technique and adequate disinfection and maintenance are important for optimizing medication delivery. In this study, inhalation technique and nebulizer disinfection/maintenance were assessed in cystic fibrosis patients by direct observation in clinic and completion of a scoring sheet. A total of 108 patients were recruited. The maximum inhalation technique score was attained by 30.5% and adequate inhalation technique score by 74.08% of patients. The inhalation technique score was best with the vibrating mesh nebulizer (p = 0.038), while patient age and number of nebulized medications did not affect ITS significantly (p > 0.05). Nebulizer disinfection/maintenance score was excellent in only 31.48%. Most families kept the nebulizer clean and used appropriate disinfection method, but only half of them replaced the nebulizer and nebulizer cup at the recommended time intervals. Nebulizer disinfection/maintenance score was positively affected by a number of nebulized medications and negatively by years of equipment use (p = 0.009 and p = 0.001, respectively). Even though inhalation technique and disinfection/maintenance practices were found to be adequate in a large proportion of cases, there is still a need for regular review and education. The type of nebulizer was associated with improved inhalation technique, but more data are required before making specific recommendations.

Triage for Malnutrition Risk among Pediatric and Adolescent Outpatients with Cystic Fibrosis, Using a Disease-Specific Tool.

Malnutrition prevails in considerable proportions of children with Cystic Fibrosis (CF), and is often associated with adverse outcomes. For this, routine screening for malnutrition is pivotal. In the present cross-sectional study, we aimed to assess the risk for malnutrition in pediatric outpatients with CF. A total of 76 outpatients (44 girls, 11.9 ± 3.9 years old, 39.5% adolescents) were recruited and anthropometric, clinical, dietary and respiratory measures were collected. All outpatients were screened for malnutrition risk with a validated disease-specific instrument. Most children exhibited a low risk for malnutrition (78.9%), whereas none of the participants were characterized as having a high malnutrition risk. In the total sample, malnutrition risk was positively associated with age (r = 0.369, p = 0.001), and inversely related to the body mass index (r = -0.684, p < 0.001), height z-score (r = -0.264, p = 0.021), and forced expiratory volume (FEV1%, r = -0.616, p < 0.001). Those classified as having a low malnutrition risk were younger (p = 0.004), heavier (p < 0.001) and taller (p = 0.009) than their counterparts with a moderate risk. On the other hand, patients in the moderate risk group were more likely pubertal (p = 0.034), with a reduced mid-upper arm fat area (p = 0.011), and worse pulmonary function (p < 0.001). Interestingly, none of the children attaining ideal body weight were classified as having a moderate malnutrition. risk, whereas 37.5% of the patients allocated at the moderate risk group exhibited physiological lung function. In this cohort of outpatients with CF that were predominantly well-nourished and attained physiological lung function, malnutrition risk was identified only in small proportions of the sample. Our data support that patients that are older, pubertal, or have diminished fat mass are at greater risk for malnutrition.

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.

Early-Life Pseudomonas aeruginosa Infection in Cystic Fibrosis and Lung Disease Progression.

Lung disease in cystic fibrosis (CF) starts early, with studies identifying abnormalities on chest computed tomography (CT) scan even in infancy. In this retrospective study, abnormal chest CT was the main outcome; body mass index (BMI) z score and forced expiratory volume percent predicted (FEV1%) predicted at age 6 to 7 years were secondary outcomes. Pseudomonas aeruginosa infection prior to 12 months of age was the main explanatory variable. There was no association between early P aeruginosa infection and abnormal CT after adjustment for CFTR (cystic fibrosis transmembrane conductance regulator) functional mutation class, gender, and other pathogens (odds ratio = 0.30; 95% confidence interval = 0.07-1.35; P = .11). No significant associations were demonstrated for BMI z score and FEV1% predicted. Children with class I-III CFTR mutations had increased risk of abnormal CT findings (odds ratio = 11.67; 95% confidence interval = 1.11-115.06; P = .035) and lower FEV1% predicted (P = .04). In the current era, early-life P aeruginosa infection in CF might not influence the severity of lung disease in school age as much as previously. Larger studies are needed to confirm this finding.

Viral croup: diagnosis and a treatment algorithm.

Viral croup is a frequent disease in early childhood. Although it is usually self-limited, it may occasionally become life-threatening. Mild croup is characterized by the presence of stridor without intercostal retractions, whereas moderate-to-severe croup is accompanied by increased work of breathing. A single dose of orally administered dexamethasone (0.15-0.6 mg/kg) is the mainstay of treatment with addition of nebulized epinephrine only in cases of moderate-to-severe croup. Nebulized budesonide (2 mg) can be given alternatively to children who do not tolerate oral dexamethasone. Exposure to cold air or administration of cool mist are treatment interventions for viral croup that are not supported by published evidence, but breathing heliox can potentially reduce the work of breathing related to upper airway obstruction. In summary, corticosteroids may decrease the intensity of viral croup symptoms irrespective to their severity on presentation to the emergency department.

Wilson-Mikity syndrome: updated diagnostic criteria based on nine cases and a review of the literature.

BACKGROUND: Nearly 50 years ago, Wilson and Mikity described a syndrome (WMS) of chronic lung disease (CLD) in premature infants, characterized by early development of cystic interstitial emphysema (PIE), despite minimal ventilatory support. The validity of the diagnosis is currently unclear; now considered either an anachronism, part of BPD spectrum or included within various poorly defined diagnoses such as chronic pulmonary insufficiency of prematurity (CPIP). OBJECTIVES: To define clinically useful diagnostic criteria for WMS so its position in the spectrum of CLD of infancy can be established. METHODS: We studied nine patients who fulfilled WMS criteria, combining this data with a detailed review of the available literature. RESULTS: Despite minimal respiratory support at birth, all developed generalized or lobar cystic PIE by 3 weeks of age, followed by slow inflammatory progression over a further 2-3 months. Final outcome was variable but most were left with some degree of CLD. CONCLUSIONS: WMS is a rare but clearly identifiable syndrome with significant morbidity, predominantly affecting infants below 1,500 g birth weight. The earliest pathology appears to be alveolar air leak. Inflammatory activation induced by cystic interstitial air may cause the subsequent progressive respiratory disease. Management is supportive but should include investigation for pulmonary hypertension.

Evolution of Pseudomonas aeruginosa type III secretion in cystic fibrosis: a paradigm of chronic infection.

Pseudomonas aeruginosa (PA) from acute and chronic (eg, cystic fibrosis (CF)) infections differ in several respects, but they can worsen prognosis in each context. Factors that facilitate conversion from an acute to chronic phenotype are poorly understood. T3 secretion proteins are virulence factors associated with poorer outcomes in acute infections, but little is known about their role in CF. We wished to characterize T3 secretion in CF PA isolates and to examine its role in clinical outcomes. A total of 114 CF subjects were divided into 3 cohorts: 1st infected individuals, CI children, and adults. Serial respiratory cultures were analyzed for T3 secretion. Serial spirometry and exacerbation data were collected prospectively. In 1st infection, 45.2% +/- 9.1% of PA isolates secreted T3 proteins compared with 29.1% +/- 4.2% and 11.5% +/- 3.0% in CI children and CI adults, respectively (P < 0.001). An inverse correlation was observed between duration of PA infection and percent T3 positive isolates (r = -0.32, P < 0.001). Overall, no association was observed between T3 secretion and pulmonary outcomes, but in the subgroup of subjects who had at least 1 T3 positive organism, T3 secretion was inversely correlated with the forced expiratory volume in 1 s (FEV(1)) decline (r = -0.35, P = 0.02). In 1st infection, 82% of cultures grew either all or no T3-positive organisms. In these patients, T3 secretion was associated with a greater risk of subsequent PA isolation (P < 0.001). In CF, PA T3 secretion decreases with residence time in lung, may predict FEV(1) decline in patients who have detectable T3 organisms, and may facilitate persistence after 1st infection.