Cystic Fibrosis Children Research Articles

SERS detection of the biomarker hydrogen cyanide from Pseudomonas aeruginosa cultures isolated from cystic fibrosis patients

Pseudomonas aeruginosa is the primary cause of chronic airway infections in cystic fibrosis (CF) patients. Persistent infections are seen from the first P. aeruginosa culture in about 75% of young CF patients, and it is important to discover new ways to detect P. aeruginosa at an earlier stage. The P. aeruginosa biomarker hydrogen cyanide (HCN) contains a triple bond, which is utilized in this study because of the resulting characteristic C≡N peak at 2135 cm−1 in a Raman spectrum. The Raman signal was enhanced by surface-enhanced Raman spectroscopy (SERS) on a Au-coated SERS substrate. After long-term infection, a mutation in the patho-adaptive lasR gene can alter the expression of HCN, which is why it is sometimes not possible to detect HCN in the breath of chronically infected patients. Four P. aeruginosa reference strains and 12 clinical P. aeruginosa strains isolated from CF children were evaluated, and HCN was clearly detected from overnight cultures of all wild type-like isolates and half of the later isolates from the same patients. The clinical impact could be that P. aeruginosa infections could be detected at an earlier stage, because daily breath sampling with an immediate output could be possible with a point-of-care SERS device.

Growth assessment in Egyptian children with cystic fibrosis: A single center study

BackgroundPoor clinical outcomes in cystic fibrosis are often associated with undernutrition. Nutritional status is an important prognostic marker, regardless of the level of respiratory function in the patient. Careful and repeated nutritional assessments allow for early detection of nutritional deterioration. ObjectivesTo assess the growth of cystic fibrosis children using different parameters as body mass index percentiles (BMIP) and standard anthropometric indexes, including height-for-age percentile (HAP), weight-for-age percentile (WAP) and compare between them as measures for nutritional failure in children with cystic fibrosis (CF). MethodologyThis was a cross sectional study including fifty children of both sexes with CF below 12years of age, recruited from the cystic fibrosis clinic, Specialized Children’s Hospital, Cairo University. Patients were subjected to full history taking including age at diagnosis, frequency of hospital admissions (per year), current medications and presence of complications. Thorough clinical examination was performed with special emphasis on anthropometric measurements including body weight, length/height, BMI calculation. ResultsThis study included 30 boys and 20 girls between 2 and 11years old with a mean of 4.25±2.30yrs. Pancreatic insufficiency was found in 36% of patients, 28% had failure to thrive as the main complaint while 72% had recurrent chest infection as the main complaint. Growth assessment revealed that 21 patients (42%) were below −2 SDS for weight. As for BMI, 14 patients (28%) were below the 10th percentile. A highly significant negative correlation was reported between the weight SDS and the frequency of hospital admission (r=−0.412, p=0.003). Furthermore, there was a highly statistical significant difference between the malnourished patients and the patients with average weight regarding the frequency of hospital admission (p<0.01). As for BMI, there was a statistically significant negative correlation between the BMI and the frequency of hospital admission (r=−0.350, p=0.013), with the different categories of BMI (malnourished, those who need nutritional reassessment, normal and obese) having highly statistical significant difference as regards the frequency of hospital admission (p<0.01). ConclusionMalnourished CF patients had the highest frequency of hospital admission. BMIP predicts nutritional failure more sensitively and accurately than conventional anthropometric indexes (WAP and HAP) in children with CF.

Характер изменений гемодинамики брюшной полости при муковисцидозе у детей

Характер изменений гемодинамики брюшной полости при муковисцидозе у детей

Not All Children with Cystic Fibrosis Have Abnormal Esophageal Neutralization during Chemical Clearance of Acid Reflux.

PurposeAcid neutralization during chemical clearance is significantly prolonged in children with cystic fibrosis, compared to symptomatic children without cystic fibrosis. The absence of available reference values impeded identification of abnormal findings within individual patients with and without cystic fibrosis. The present study aimed to test the hypothesis that significantly more children with cystic fibrosis have acid neutralization durations during chemical clearance that fall outside the physiological range.MethodsPublished reference value for acid neutralization duration during chemical clearance (determined using combined impedance/pH monitoring) was used to assess esophageal acid neutralization efficiency during chemical clearance in 16 children with cystic fibrosis (3 to <18 years) and 16 age-matched children without cystic fibrosis.ResultsDuration of acid neutralization during chemical clearance exceeded the upper end of the physiological range in 9 of 16 (56.3%) children with and in 3 of 16 (18.8%) children without cystic fibrosis (p=0.0412). The likelihood ratio for duration indicated that children with cystic fibrosis are 2.1-times more likely to have abnormal acid neutralization during chemical clearance, and children with abnormal acid neutralization during chemical clearance are 1.5-times more likely to have cystic fibrosis.ConclusionSignificantly more (but not all) children with cystic fibrosis have abnormally prolonged esophageal clearance of acid. Children with cystic fibrosis are more likely to have abnormal acid neutralization during chemical clearance. Additional studies involving larger sample sizes are needed to address the importance of genotype, esophageal motility, composition and volume of saliva, and gastric acidity on acid neutralization efficiency in cystic fibrosis children.

Aspergillus pulmonary infection in cystic fibrosis patients – more than just allergic bronchopulmonary aspergillosis A 5-year retrospective study in a pediatric CF Population

AbstractPurpose of review: Among pediatric patients with cystic fibrosis (CF), the Aspergillus is commonly found in sputum causing various pulmonary infections. However, the prevalence and clinical impact is still unclear. The aim was to show prevalence of three Aspergillus pulmonary infections: Aspergillus bronchitis, allergic bronchopulmonary aspergillosis (ABPA) and invasive pulmonary aspergillosis (IPA) in the period 2007-2011 in a pediatric CF population (<18 years), and relate them to gender, age and lung function.Recent findings: Aspergillus pulmonary infections are a considerable problem among children with CF. Almost 40% of the CF children suffered from an Aspergillus pulmonary infection within the 5-year study period. Aspergillus bronchitis was the most common manifestation, seen from an early age and throughout childhood, whereas ABPA and IPA were rarely seen in children below 7 years of age. We found no difference in gender distribution when comparing the three diagnoses. In the majority of CF patients, lung function declined during an Aspergillus pulmonary infection.Summary: Aspergillus pulmonary infections in CF patients under the age of 18 years are frequent and probably underestimated. The majority of patients with Aspergillus pulmonary infections experienced a decline in lung function.

How Well Do Children with Cystic Fibrosis Sleep? An Actigraphic and Questionnaire-Based Study

To measure sleep patterns and quality, objectively and subjectively, in clinically stable children with cystic fibrosis (CF) and healthy control children, and to examine the relationship between sleep quality and disease severity. Clinically stable children with CF and healthy control children (7-18 years of age) were recruited. Sleep patterns and quality were measured at home with actigraphy (14 days). Overnight peripheral capillary oxygen saturation was measured via the use of pulse oximetry. Daytime sleepiness was evaluated by the Pediatric Daytime Sleepiness Scale (PDSS) and subjective sleep quality by the Sleep Disturbance Scale for Children and Obstructive Sleep Apnea-18. A total of 87 children with CF and 55 control children were recruited with no differences in age or sex. Children with CF had significantly lower total sleep time and sleep efficiency than control children due to frequent awakenings and more wake after sleep onset. In children with CF, forced expiratory volume in 1 second and overnight peripheral capillary oxygen saturation nadir correlated positively with total sleep time and sleep efficiency and negatively with frequency of awakenings and wake after sleep onset. Patients with CF had significantly greater Sleep Disturbance Scale for Children (45 vs 35; P < .001), Obstructive Sleep Apnea-18 (35 vs 24; P < .001), and PDSS scores (14 vs 11; P < .001). There was a negative correlation between PDSS and forced expiratory volume in 1 second (r = -0.23; P < .05). Even in periods of clinical stability, children with CF get less sleep than their peers due to more time in wakefulness during the night rather than less time spent in bed. Objective measures of sleep disturbance and subjective daytime sleepiness were related to disease severity. In contrast, parents of children with CF report high levels of sleep disturbance unrelated to disease severity.

Два випадки захворювання дітей на муковісцидоз у сім'ї

Два випадки захворювання дітей на муковісцидоз у сім'ї

The cost-effectiveness of palivizumab in infants with cystic fibrosis in the Canadian setting: A decision analysis model

ABSTRACTBackground: Children with cystic fibrosis (CF) are at higher risk of severe respiratory syncytial virus (RSV) infection, which can lead to a decline in lung function. A monoclonal antibody, palivizumab (PMB), effectively prevents RSV hospitalizations; however, the high cost of PMB, approximately C$10,000 per patient per RSV season, limits its widespread use. We assess the cost-effectiveness of PMB prophylaxis in CF children less than 2 y of age from the Canadian healthcare payer's perspective. Methods: In 2014, a Markov cohort model of CF disease and infant RSV infections in the Canadian setting was developed based on literature data. Infants were treated with monthly PMB injections over the 5-month RSV season. Lifetime health outcomes, quality-adjusted life years (QALYs) and 2013 $CAD costs, discounted at 5%, were estimated. Findings are summarized as incremental cost-effectiveness ratios (ICERs) and budget impact. Deterministic sensitivity analysis was conducted to assess parameter uncertainty. Results: Implementation of a hypothetical Canadian RSV prophylaxis program resulted in ICERs of C$652,560 (all CF infants) and C$157,332 (high-risk CF infants) per QALY gained and an annual budget impact of C$1,400,000 (all CF infants) and C$285,000 (high-risk CF infants). The analysis was highly sensitive to the probability of severe RSV, the degree of lung deterioration following infection, and the cost of PMB. Conclusions: Our results suggest PMB is not cost-effective in Canada by commonly used thresholds. However, given the rarity of CF and relatively small budget impact, consideration may be given for the selective use of PMB for immunoprophylaxis of RSV in high-risk CF infants on a case-by-case scenario basis.

Hospitalization of Children with Cystic Fibrosis Adversely Affects Mothers’ Physical Activity, Sleep Quality, and Psychological Status

Having a child with a chronic disease can cause anxiety and depression, which may impair the sleep quality of mothers. The aim of this study was to compare physical activity level, sleep quality, anxiety and depression in mothers of hospitalized cystic fibrosis patients, cystic fibrosis outpatients and healthy controls. Twenty-three of the patients were hospitalized and 38 were outpatients and 37 age-matched healthy children served as controls. The mothers’ fatigue levels, sleep disturbances, stress levels and perceptions of their child’s disease severity were assessed with a Visual Analog Scale. The International Physical Activity Questionnaire, Pittsburgh Sleep Quality Index and Hospital Anxiety and Depression Scale were administered to the mothers of children. Mothers of hospitalized and outpatient cystic fibrosis children had significantly higher fatigue severity, sleep disturbance, and stress level according to Visual Analog Scale than mothers of healthy controls. All of International Physical Activity Questionnaire scores were significantly lower in cystic fibrosis patients’ mothers than healthy controls’. Hospital Anxiety and Depression Scale depression score, Pittsburgh Sleep Quality Index subdomains and total scores of hospitalized patients’ mothers were higher than those of the other two groups (p < 0.05). Chronic illness in children with cystic fibrosis may impair the physical activity level, sleep quality and psychological state of their mothers, especially during hospitalization period.

Innovative assessment of inpatient and pulmonary drug costs for children with cystic fibrosis.

Previous estimates of the cost of care for pediatric Cystic fibrosis (CF) showed wide variation, without specific summary of pulmonary drug costs. Enrolled CF children from the Wisconsin newborn screening trial were evaluated quarterly per protocol. Assessments systematically included all treatments, hospitalizations, and nutritional and pulmonary outcomes. Direct medical costs from hospital billing and medical records from 1989 to 2010 were used to describe costs by age-ranges and subgroups throughout follow-up. Outpatient drugs were separated by category (pulmonary/otherwise). Inpatient and drug costs were examined by clinical risk factors (presence of meconium ileus, pancreatic insufficiency, and expected severity of genetic mutations). Seventy-three children were followed for an average of 12.9 years with an average annual total cost of care of $24,768. Outpatient drug costs (53%) and hospitalizations (32%) represented the majority of costs. Drug costs were 48% for pulmonary indications and 52% for non-pulmonary. Pulmonary drug costs for children taking dornase were 54% of their drug costs while pulmonary drug costs were only 31% for children not taking dornase. Significant differences in frequency of inpatient stays existed for children with pancreatic insufficiency. Substantial differences in treatment costs exist as children age and by clinical risk factor. This study provides more accurate longitudinal estimates of CF care costs throughout childhood and shows that increasing age, pancreatic insufficiency, use of dornase, and hospitalizations are key determinants of cost. These estimates can be included in evaluations of the cost-effectiveness of new, highly expensive treatments being introduced for any CF population. Pediatr Pulmonol. 2016;51:1295-1303. © 2016 Wiley Periodicals, Inc.

The Impact of Secondhand Smoke Exposure on Children with Cystic Fibrosis: A Review

Secondhand smoke exposure (SHSe) has multiple adverse effects on lung function and growth, nutrition, and immune function in children; it is increasingly being recognized as an important modifier of disease severity for children with chronic diseases such as cystic fibrosis (CF). This review examines what is known regarding the prevalence of SHSe in CF, with the majority of reviewed studies utilizing parental-reporting of SHSe without an objective biomarker of exposure. A wide range of SHSe is reported in children with CF, but under-reporting is common in studies involving both reported and measured SHSe. Additionally, the impact of SHSe on respiratory and nutritional health is discussed, with potential decreases in long-term lung function, linear growth, and weight gain noted in CF children with SHSe. Immunologic function in children with CF and SHSe remains unknown. The impact of SHSe on cystic fibrosis transmembrane conductance regulator (CFTR) function is also examined, as reduced CFTR function may be a pathophysiologic consequence of SHSe in CF and could modulate therapeutic interventions. Finally, potential interventions for ongoing SHSe are delineated along with recommended future areas of study.

Novel methodology to perform sulfur hexafluoride (SF6)-based multiple-breath wash-in and washout in infants using current commercially available equipment.

Multiple-breath inert gas washout (MBW) is ideally suited for early detection and monitoring of serious lung disease, such as cystic fibrosis, in infants and young children. Validated commercial options for the MBW technique are limited, and suitability of nitrogen (N2)-based MBW is of concern given the detrimental effect of exposure to pure O2 on infant breathing pattern. We propose novel methodology using commercially available N2 MBW equipment to facilitate 4% sulfur hexafluoride (SF6) multiple-breath inert gas wash-in and washout suitable for the infant age range. CO2, O2, and sidestream molar mass sensor signals were used to accurately calculate SF6 concentrations. An improved dynamic method for synchronization of gas and respiratory flow was developed to take into account variations in sidestream sample flow during MBW measurement. In vitro validation of triplicate functional residual capacity (FRC) assessments was undertaken under dry ambient conditions using lung models ranging from 90 to 267 ml, with tidal volumes of 28-79 ml, and respiratory rates 20-60 per minute. The relative mean (SD, 95% confidence interval) error of triplicate FRC determinations by washout was -0.26 (1.84, -3.86 to +3.35)% and by wash-in was 0.57 (2.66, -4.66 to +5.79)%. The standard deviations [mean (SD)] of percentage error among FRC triplicates were 1.40 (1.14) and 1.38 (1.32) for washout and wash-in, respectively. The novel methodology presented achieved FRC accuracy as outlined by current MBW consensus recommendations (95% of measurements within 5% accuracy). Further clinical evaluation is required, but this new technique, using existing commercially available equipment, has exciting potential for research and clinical use.

Bone mineral density and its contributing factors in Egyptian children with cystic fibrosis

Cystic fibrosis (CF) is an autosomal recessive disorder that, despite advances in medical care, continues to be a life-limiting disease. With increase in life expectancy of the CF population, bone disease has emerged as a common complication. The aim of the study was to determine bone mineral density (BMD) and total body composition (TBC) in a sample of Egyptian children with CF and assess the contributing factors that might be related to BMD deficits. This was a cross-sectional case–control study that included 15 children with CF who were of a mean age of 6.3±3.68 years (2.5–15 years) and were diagnosed by sweat chloride testing. All CF children were subjected to detailed history taking, thorough clinical examination, laboratory investigations, and pulmonary function tests. They also underwent growth, puberty, and nutrition evaluation. BMD and TBC were evaluated using dual-energy X-ray absorptiometry. CF children had significantly decreased mean BMD and TBC compared with the control group. An overall 26.66% of these patients had osteopenia and one patient was 3 years old. They also showed delayed growth when compared with Egyptian standards, and most of them (> 80%) presented with recurrent chest infections, malabsorption, undernutrition, and treatment with inhaled steroids for more than 6 months. We demonstrated the presence of osteopenia in our CF patients that presented early in life. Improving nutritional status, correcting malabsorption, and limiting chest infections are necessary for prevention. Annual assessment of BMD and body composition should be initiated early in life to target those who need preventive treatment against osteoporosis and reduce the risk for fractures later in life.

Asymmetric dimethylarginine and related metabolites in exhaled breath condensate of children with cystic fibrosis.

Asymmetric dimethylarginine (ADMA) competitively inhibits nitric oxide synthase (NOS). Its levels in specimens from murine models and asthmatic patients are related to inflammation and oxidative stress. Patients with cystic fibrosis(CF) reportedly have higher arginase activity, lower NO production and NOS expression than healthy controls. The objective was to assess the role of ADMA and related metabolites as disease biomarkers in exhaled breath condensate (EBC) of pediatric CF patients, compared with age-matched healthy controls (HC). A longitudinal design was conceived and 34 CF patients (21 stable, 13 at the onset of exacerbation) and 16 HC were enrolled. All CF patients underwent clinical examination, spirometry and EBC collection at enrolment; the same tests were performed also after an antibiotic course in those patients with exacerbation. Metabolites levels in EBC were measured with an ultra-performance liquid chromatography and tandem mass spectrometry technique. All CF patients had ADMA levels (expressed as ratio to tyrosine) similar to those in HC (median 0.0112, IQR 0.0103-0.0120 and median 0.0114, IQR 0.0090-0.0128, respectively; P = 0.983), while a significant increase in the citrulline/tyrosine ratio was found in CF patients (median 0.6419, IQR 0.5738-0.6899 in CF vs median 0.4176, IQR 0.2986-0.5082 in HC; P = 0.00003). No differences in ADMA levels emerged between stable patients and those with exacerbation. ADMA and related aminoacids were measured simultaneously for the first time in EBC from CF patients. Higher citrulline/tyrosine ratios were found in CF children with normal ADMA levels, suggesting a dysregulated ADMA metabolism in these patients.

62 Evaluation of nasal microbiota in cystic fibrosis children

Results: The percentage share of NFGNB other than PA was 28%. The most common isolated NFGNB were: Stenotrophomonas 10%, Achromobacter 5%, non-aeruginosa Pseudomonas 5%, Sphingomonas 3%, Acinetobacter 2% and others 3%. NFGNB showed variable sensitivity to carbapenems (42–89%), fluoroquinolones (13–100%), aminoglycosides (22–100%), cephalosporins (23–78%), colistin (84–100%), co-trimoxazole (75–97%). Conclusion: We observed significant increase of NFGNB other than PA in colonization of the airways of CF pts. Prolonged incubation and using a selective media increases the frequency of NFGNB isolation. Mass spectrometry is a reliable alternative to conventional biochemical tests used in identification of rarely isolated NFGNB. For some NFGNB species it is required to determine the MIC values to interpret their susceptibility.

238 Evaluation of adherence to azithromycin in parents with cystic fibrosis children

238 Evaluation of adherence to azithromycin in parents with cystic fibrosis children

230 Prevention is better than cure: assessing oral health behaviours in cystic fibrosis (CF) children

230 Prevention is better than cure: assessing oral health behaviours in cystic fibrosis (CF) children

Molecular diagnosis of two Chinese cystic fibrosis children and literature review

To investigate the clinical manifestations and molecular features of cystic fibrosis in Chinese children. A retrospective analysis of two pediatric cystic fibrosis cases diagnosed by gene test in Beijing Children's Hospital, Capital Medical University from 2010 to 2015, and Chinese cystic fibrosis reported patients searched of"cystic fibrosis, Chinese"on Chinese databases (CNKI, Wanfang Data) and PubMed from 1975 to 2015.The clinical manifestations and molecular features were analyzed. One of the two newly diagnosed cystic fibrosis cases was a 10-year old girl who suffered from reccurent cough with expectoration and associated with cirrhosis.Sweat tests showed increased chloride twice with the lower level of 306.82 mmol/L.The other was an 8-month old boy with reccurent pneumonia from neonate, failure to thrive and fatty diarrhea.Two children had various degrees of bronchiectasis and massive sticky secretion on the bronchoscopy.They had no family history and their parents had no consanguineous marriage.CFTR mutations of c. 595C>T and c. 2290C>T were found in gene tests.On the database, twenty-one reports involving thirty-six Chinese patients (16 males and 20 females) were retrieved.Together with this group of 2 cases, a total of 38 cases were involved.The age at diagnosis was 4 months to 28 years with a median age of 10 years.All patients had reccurent respiratory infections, twenty-seven cases (71%) had malnutrition, fifteen (39%)had chronic diarrhea, and 16 cases (42%) had other digestive manifestations, including jaundice (4 cases), hepatomegaly (11 cases), ascites (2 cases) and pancreatic atrophy (3 cases). Five cases had a positive family history and six cases had a suspicious family history.Consanguineous marriage was found in three families.Sweat test revealed elevated chloride (52-327 mmol/L) in 28 cases.Eight of the 16 patients who performed pancreatic exocrine function examination showed pancreatic insufficiency.Eighteen of the 20 patients described the image manifestation of bronchiectasis.The age at death of 13 patients(34%) was 4 months to 25 years with a median of 11 years.Twenty-nine rare CFTR mutations were found in 25 tested patients, and c. 1766+ 5G>T(5 cases), c.2083dupG and c. 2684G>A (3 cases) held the top three mutation patterns. Respiratory symptoms and bronchiectasis are the main manifestations of Chinese cystic fibrosis patients, although some of them may be associated with malnutrition, digestive symptoms and pancreatic insufficiency. CFTR mutations are various and rare in Caucasians.

Evaluation of children with cystic fibrosis by impulse oscillometry when stable and at exacerbation.

Pulmonary function tests are important in the diagnosis and follow-up of airway disease in cystic fibrosis (CF). Conventional spirometry for which repeated forced expiration maneuver are needed is considered as the main method. Impulse oscillometry (IOS) is a non-invasive method, which needs minimal cooperation. We performed a prospective cross-sectional study to determine the pulmonary function in CF children with IOS, and evaluate the IOS measurements during acute exacerbation. We hypothesized that IOS can detect lung function impairment in CF patients and detect changes during acute exacerbations. CF patients aged 3-18 years were included as the study group and healthy children of the same age formed the control group. The patients were evaluated three times at 3-month intervals. Patients aged >6 years performed both spirometry and IOS while patients aged ≤6 years performed only IOS. In patients with acute exacerbation, spirometry and IOS were performed both at and after the exacerbation. Forty-nine CF patients and 45 healthy children were enrolled to the study. Sixteen patients were admitted with acute exacerbation during the study. The resistance values (R5-10-15-20 Hz) as well as Z5, Fres, and AX values of the patients were higher than those of the control group, while reactance values (X5-10-15-20 Hz) were lower. R values as well as Z5, Fres, and AX values increased during exacerbation and decreased after treatment; X (10-15 Hz) values decreased during exacerbation and increased after recovery (P < 0.05). IOS may be useful to evaluate pulmonary functions and detect acute exacerbations in CF patients. Pediatr Pulmonol. 2016;51:1151-1158. © 2016 Wiley Periodicals, Inc.

Respiratory sleep disturbance in children and adolescents with cystic fibrosis

Sleep disturbance has been described in cystic fibrosis (CF) patients as relevant to clinical and lung function predictive factors helping to improve the diagnosis and early intervention. Related paediatric studies are scarce. ObjectiveTo describe respiratory sleep disturbance (RSD) and its association with spirometric indices in a population of CF children. A second aim was to determine if spirometric indices and wake-time SpO2 are predictors of sleep disturbance. MethodsA cross-sectional study involving 33CF paediatric patients. All participants underwent in-lab polysomnography (PSG), pulse oximetry and spirometry. A standardized sleep questionnaire was completed for each patient. Two subgroups were considered: I – Normal (FEV1>−1.64 z-score); II – Obstructed (FEV1≤−1.64 z-score). ResultsParticipant's median age was 12 (6–18) years, 16 (48.5%) were male. Twenty-nine patients (87.9%) presented sleep complaints. Sleep efficiency was reduced; sleep latency and waking after sleep onset (WASO) increased. N1 increased, N2, N3, REM and awakenings were normal. The apnoea–hypopnoea index was 0.6/h (sd 0.9); respiratory disturbance index (RDI) was 6.6/h (sd 5.2). Mean awaking (97% (sd 1.1)) and sleep SpO2 (95% (sd 2.7)) were normal; mean nocturnal oximetry desaturation index was 2.36/h; minimal nocturnal SpO2 was 89% (sd 4.1).We found associations between mean nocturnal SPO2 and mean values of FEV1 (r=0.528; p=0.002) and FEF25–75 (r=0.426; p=0.013). There were significant differences in nocturnal SpO2 between normal and obstructed patients (p<0.000). PSG data correlated with the questionnaire answers for night awakenings and WASO (p=0.985) and difficult breathing during sleep and RDI (p=0.722).This study points to most CF children having sleep complaints, and highlights the correlation between subjective assessment of sleep and PSG and spirometric results. Awake-time SpO2 and spirometric values are possible risk predictors for nocturnal desaturation.