scholarly journals Continuous versus Intermittent Infusions of Ceftazidime for Treating Exacerbation of Cystic Fibrosis

2009 ◽  
Vol 53 (9) ◽  
pp. 3650-3656 ◽  
Author(s):  
Dominique Hubert ◽  
Evelyne Le Roux ◽  
Thibaud Lavrut ◽  
Benoit Wallaert ◽  
Philippe Scheid ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Continuous Infusion ◽  
Antibiotic Treatment ◽  
Forced Expiratory Volume ◽  
Trough Concentrations ◽  
The Mean ◽  
Treatment Procedures ◽  
Susceptibility Profiles ◽  
Infusion Regimen

ABSTRACT The present multicenter, randomized crossover study compared the safety and efficacy of continuous infusion with those of short infusions of ceftazidime in patients with cystic fibrosis. Patients with chronic Pseudomonas aeruginosa colonization received two successive courses of intravenous tobramycin and ceftazidime (200 mg/kg of body weight/day) for pulmonary exacerbation administered as thrice-daily short infusions or as a continuous infusion. The primary endpoint was the variation in the forced expiratory volume in 1 s (FEV1) during the course of antibiotic treatment. Sixty-nine of the 70 patients enrolled in the study received at least one course of antibiotic treatment. The improvement in FEV1 at the end of therapy was not statistically different between the two treatment procedures (+7.6% after continuous infusion and +5.5% after short infusions) but was better after continuous ceftazidime treatment in patients harboring resistant isolates (P < 0.05). The interval between the course of antibiotic treatments was longer after the continuous infusion than after the short infusion of ceftazidime (P = 0.04). The mean serum ceftazidime concentration during the continuous infusion was 56.2 ± 23.2 μg/ml; the mean peak and trough concentrations during the short infusions were 216.3 ± 71.5 and 12.1 ± 8.7 μg/ml, respectively. The susceptibility profiles of the P. aeruginosa isolates remained unchanged and were similar for both regimens. Quality-of-life scores were similar whatever the treatment procedure, but 82% of the patients preferred the continuous-infusion regimen. Adverse events were not significantly different between the two regimens. In conclusion, the continuous infusion of ceftazidime did not increase its toxicity and appeared to be as efficient as short infusions in patients with cystic fibrosis as a whole, but it gave better results in patients harboring resistant isolates of P. aeruginosa.

scholarly journals Is FEV1 associated with quality of life in children with cystic fibrosis? An original article

2019 ◽  
Vol 6 (1) ◽  
pp. e06-e06
Author(s):  
Rohola Shirzadi ◽  
Safoura Navaei ◽  
Mohammadreza Modaresi ◽  
Farzad Masiha
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Medical Center ◽  
Cross Sectional Study ◽  
Forced Expiratory Volume ◽  
Psychological Status ◽  
Cross Sectional ◽  
The Mean

Introduction: Cystic fibrosis (CF) is a serious genetic life-shortening disease. Quality of life (QoL) measurement related to CF children is a relatively new field of research, which includes the patient’s perspective in research and clinical practice. Objectives: This study aimed to evaluate the QoL in children with CF and its association with FEV1 (forced expiratory volume in 1 second). Patients and Methods: This cross-sectional study was carried out on 7-14 years old children with CF attending children’s medical center from March 2017 to March 2018. Throat swab cultures and spirometry evaluation was performed for all patients. FEV1 was determined and the 6-min walk test (6MWT) was conducted. The patient’s psychological status was assessed using the Persian version of pediatric QoL inventory. ANOVA, t test, and chisquare tests were used for data analysis. Results: Seventy-six subjects with the mean age of 10.49±3.18 years were studied since 59% of them (n=44) were boys. The mean total QoL was 65.34±17.73. Patients with lower pulmonary function had a lower QoL. There was a significant association between FEV1 and school and emotional functioning (P=0.005 and P=0.002, respectively). A significant association was found between SPO2 (peripheral capillary oxygen saturation) reduction after 6MWT and FEV1 decline (P=0.001). Additionally, a significant association was detected between FEV1 and the distance walked during 6MWT (P=0.030). Conclusion: Regarding the association between pulmonary function and QoL in CF patients and lower QoL score in our study, the importance of assessing pulmonary function in these patients should not be neglected.

scholarly journals 0960 Perception of Sleep Importance in Children With Cystic Fibrosis

SLEEP ◽  
2020 ◽  
Vol 43 (Supplement_1) ◽  
pp. A364-A365
Author(s):  
K Reiling ◽  
A Patel
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Sleep Problems ◽  
Pulmonary Function Tests ◽  
Poor Sleep ◽  
Airway Clearance ◽  
The Mean ◽  
Nocturnal Cough ◽  
Perception Of Sleep

Abstract Introduction Children with cystic fibrosis (CF) are known to have poor sleep efficiency and increased nighttime awakenings secondary to factors such as nocturnal cough and time spent on therapies for airway clearance. Studies have shown that children with poor lung function have a higher Pediatric Daytime Sleepiness Score (PDSS). An increase in sleep disturbance has been associated with poorer perceived health in children with CF. There have been limited studies to date that report the perception of sleep importance in CF patients. We aim to characterize the perception of sleep importance in children with CF as measured through a self-reported questionnaire and identify barriers to sleep. Methods After IRB approval, subjects with CF aged 3-17 years were prospectively recruited from routine pulmonology clinic visits (n=28, 17 male). A questionnaire was provided consisting of 35 questions regarding sleep practices, perception of sleep importance, and PDSS. Recent pulmonary function tests (PFTs) were also collected. Results The mean PDSS was 11.3, with a range of 4 to 24. The questionnaire responses were as follows: 82% of participants reported sleep as “very important” overall, 92% reported sleep being “very important” for health, and 75% reported sleep being “very important” for lung function. In addition, 39% reported airway clearance as part of their nighttime routine and 89% reported utilizing electronic screens 2 hours prior to bed. The most frequent barriers to sleep identified were technology and bedtime resistance (14% each), and homework, excitability, and vest/airway treatments (11% each). 86% of participants had at least one symptom of disordered sleep. Conclusion Screening for sleep problems in the CF population may be beneficial and may contribute to improved quality of life. Further recruitment is ongoing. Support  

scholarly journals FACTORS ASSOCIATED TO QUALITY OF LIFE IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS

2020 ◽  
Vol 38 ◽  
Author(s):  
Nelbe Nesi Santana ◽  
Célia Regina Moutinho de Miranda Chaves ◽  
Christine Pereira Gonçalves ◽  
Saint Clair dos Santos Gomes Junior
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Children And Adolescents ◽  
Functional Capacity ◽  
Clinical Status ◽  
Forced Expiratory Volume ◽  
Cross Sectional ◽  
Function Test

ABSTRACT Objective: To verify the association between quality of life, functional capacity and clinical and nutritional status in children and adolescents with cystic fibrosis (CF). Methods: Cross-sectional study, including patients from eight to 18 years old with CF. Quality of life, functional capacity, nutritional status and clinical status were evaluated with the Cystic Fibrosis Questionnaire; the 6-minute walk test (6MWT) and manual gripping force (MGF); the height percentiles for age and body mass index for age and respiratory function test, respectively. Pearson and Spearman correlation tests and logistic regression were used to analyze the data. Results: A total of 45 patients, 13.4±0.5 years old, 60% female, 60% colonized by Pseudomonas aeruginosa and 57.8% with at least one F508del mutation participated in the study. When assessing the perception of quality of life, the weight domain reached the lowest values, and the digestive domain, the highest. In the pulmonary function test, the forced expiratory volume of the first second was 77.3±3.3% and the 6MWT and MGF presented values within the normal range. There was an association between quality of life and functional capacity, nutritional status and clinical status of CF patients. Conclusions: The study participants had good clinical conditions and satisfactory values of functional capacity and quality of life. The findings reinforce that the assessment of quality of life may be important for clinical practice in the management of treatment.

scholarly journals Sputum and plasma adiponectin levels in clinically stable adult cystic fibrosis patients with CFTR I1234V mutation

2019 ◽  
Author(s):  
Atqah AbdulWahab ◽  
Mona Allangawi ◽  
Merlin Thomas ◽  
Ilham Bettahi ◽  
Siveen K. Sivaraman ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Adiponectin Level ◽  
Pancreatic Insufficiency ◽  
Forced Expiratory Volume ◽  
Healthy Controls ◽  
Cross Sectional ◽  
Plasma Adiponectin ◽  
Inflammatory Status ◽  
Significant Difference ◽  
The Mean

Abstract Background: Cystic fibrosis (CF) lung disease is associated with chronic inflammation leading to progress in lung function. Adiponectin is a predominantly anti-inflammatory adipokine that may have a role in CF lung. This study aims to determine total sputum and total plasma adiponectin levels in clinically stable adults CF patients with CFTR I1234V mutation, compared to plasma adiponectin levels in healthy controls and to investigate their correlations with body mass index (BMI) and spirometry in patients with CF. Methods: A cross-sectional study comprises 17 CF patients and 18 healthy controls. Adiponectin levels were measured by magnetic bead-based multiplex assay. Results: The mean age of adult CF patients was 22.9 years± 3.8 (18-30) and 76.5% CF patients had pancreatic sufficiency. The mean BMI in healthy controls was slightly higher than CF patients. The mean sputum adiponectin level was significantly lower than plasma adiponectin levels in CF patients and healthy controls (p<0.001), whereas no significant difference in plasma adiponectin levels between CF patients and healthy controls. The mean sputum adiponectin level was observed to be higher in CF patients with pancreatic insufficiency. Sputum adiponectin level was correlated positively with plasma adiponectin level in CF patients (r= 0.47, p=0.06). Sputum and plasma adiponectin levels in CF patients were correlated negatively with BMI and percentage predicted forced expiratory volume in 1 second (FEV1) and Forced vital capacity (FVC). Conclusions: Sputum adiponectin may provide a minimally invasive tool in the assessment of an inflammatory status in CF patients. Further larger study to address any difference in sputum and plasma adiponectin levels among CF patients with pancreatic sufficiency versus pancreatic insufficiency.

scholarly journals Cystic Fibrosis Mortality in Childhood. Data from European Cystic Fibrosis Society Patient Registry

2018 ◽  
Vol 15 (9) ◽  
pp. 2020 ◽  
Author(s):  
Anna Zolin ◽  
Anna Bossi ◽  
Natalia Cirilli ◽  
Nataliya Kashirskaya ◽  
Rita Padoan
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Standard Deviation Score ◽  
Logistic Models ◽  
Forced Expiratory Volume ◽  
High Income ◽  
Patient Registry ◽  
Middle Income

Data collected in the European Cystic Fibrosis Society Patient Registry (ECFSPR) database were used to investigate whether risk factors for death in childhood and adolescents CF patients have different impact in countries of different income. In this way, it is possible to recognize where interventions could improve the quality of care and survival in these patients. We matched deceased and alive patients by age, country, year of follow-up. Multivariable logistic models were developed. In the years of this study, the ECFSPR collected information on 24,416 patients younger than 18 years: 7830 patients were from countries with low/middle income and 16,586 from countries with high income; among these the dead are 102 and 107 (p < 0.001), respectively. The use of oxygen, forced expiratory volume in one second (FEV1) below 40% and BMI standard deviation score (SDS) below −2 represent risk factors for death. However, some patients from countries with high income remain alive even if their values of FEV1% and BMI-SDS were low, and some deceased patients from countries with high income had high values of FEV1% (>60%). Evaluation of mortality in pediatric age may reflect the availability of resources for CF diagnosis and treatment in some countries.

scholarly journals Procalcitonin predicts the severity of cystic fibrosis pulmonary exacerbations and readmissions in adult patients: a prospective cohort study

2020 ◽  
Vol 68 (4) ◽  
pp. 856-863
Author(s):  
Kristina L Bailey ◽  
Peter J Murphy ◽  
Olena K Lineberry ◽  
Matthew R Haack ◽  
John D Dickinson ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Antibiotic Treatment ◽  
Hospital Admissions ◽  
Clinical Care ◽  
Forced Expiratory Volume ◽  
Pulmonary Exacerbation ◽  
Inclusion Criteria ◽  
Exclusion Criteria ◽  
Pulmonary Exacerbations ◽  
Time Of Admission

Patients with cystic fibrosis (CF) experience multiple pulmonary exacerbations throughout their lifetime, resulting in repeated antibiotic exposure and hospital admissions. Reliable diagnostic markers to guide antibiotic treatment in patients with CF, however, are lacking. Given that the CF airway is characterized by persistent and frequent bacterial infection, our goal was to determine if procalcitonin (PCT) could be used as a severity and prognostic marker of CF exacerbation. We enrolled 40 participants at the time of diagnosis of CF pulmonary exacerbation. Inclusion criteria: age ≥19 years with exacerbation requiring antibiotics as determined by the treating physician. Exclusion criteria: antibiotics initiated more than 48 hours prior to enrollment, and pregnancy. Blood samples were collected on enrollment day and after 7–10 days of treatment. Of the 40 patients enrolled, 23 (57.5%) had detectable levels of PCT (≥0.05 ng/mL). PCT levels were significantly associated with pulmonary exacerbation scores (p=0.01) and per cent decrease in forced expiratory volume in 1 second (FEV1) (p=0.01) compared with the best in the last 12 months. Those who had worsening PCT during treatment had less improvement in FEV1 (p=0.001) and were more likely to be readmitted to the hospital sooner (p<0.0001). Likewise, those who had a detectable PCT at the time of admission were more likely to be readmitted sooner (p=0.03). PCT elevation during antibiotic treatment is associated with less improvement in FEV1 and earlier readmission. A detectable PCT level occurs only in more severe CF exacerbations. Multicenter trials are needed to confirm whether PCT may play a role in the clinical care of patients with CF.

Nocturnal hyperalimentation via a gastrostomy tube in children with cystic fibrosis

2021 ◽  
Vol 16 (2) ◽  
pp. 22-34
Author(s):  
I. Sokolov ◽  
◽  
E.A. Roslavtseva ◽  
V.P. Chernevich ◽  
N.I. Burkina ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Nutritional Status ◽  
Body Weight Gain ◽  
Forced Expiratory Volume ◽  
Gastrostomy Tube ◽  
Percutaneous Endoscopic Gastrostomy Tube ◽  
Endoscopic Control ◽  
Low Profile ◽  
The Mean

Objective. To assess the efficacy of nocturnal hyperalimentation via a low-profile gastrostomy tube for in the improvement of nutritional status and lung function in children with severe cystic fibrosis (CF). Patients and methods. We used nocturnal hyperalimentation with a nutrient solution via a low-profile gastrostomy tube installed under endoscopic control to improve nutritional status of 16 CF children with severe protein-calorie malnutrition aged between 5.5 and 17.5 years. The mean duration of nocturnal hyperalimentation was 1.3 years (range: 7 months–54 months). The nutritional status was evaluated using the WHOAntho Plus software; pulmonary function was evaluated by assessing forced expiratory volume in 1 minute (FEV1) and the number of bronchopulmonary exacerbations per year. Results. All children demonstrated an improvement in their weight and height. After the first 12 months, the mean body weight gain was 5 kg; during the second and third years, children gained 4 kg and nearly 2 kg, respectively. The mean increase in height was 7 cm in the first year, 10 cm in the second year, and 4 cm in the third year. All patients except one had an increase in FEV1 and fewer exacerbations. Complications after gastrostomy were rare and easily addressed. Conclusion. Nocturnal hyperalimentation via a percutaneous endoscopic gastrostomy tube is an effective and safe method of improving nutritional status in children with CF, which delays the progression of bronchopulmonary lesions and lung function reduction, improves the prognosis and quality of life. Key words: cystic fibrosis, children, nutritional status, lung function, enteral nutrition, low-profile gastrostomy tube, nocturnal hyperalimentation

scholarly journals Changes in Pediatric Health-Related Quality of Life in Cystic Fibrosis After IV Antibiotic Treatment for Pulmonary Exacerbations

2009 ◽  
Vol 17 (1) ◽  
pp. 49-55 ◽  
Author(s):  
Avani C. Modi ◽  
Crystal S. Lim ◽  
Kimberly A. Driscoll ◽  
Carrie Piazza-Waggoner ◽  
Alexandra L. Quittner ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Antibiotic Treatment ◽  
Health Related Quality ◽  
Pediatric Health ◽  
Related Quality ◽  
Health Related ◽  
Pulmonary Exacerbations

Unique Pattern of Pulmonary Function after Exercise in Patients with Cystic Fibrosis

1994 ◽  
Vol 6 (3) ◽  
pp. 275-286 ◽  
Author(s):  
Ted. A. Kaplan ◽  
Gina Moccia ◽  
Robert M. McKey
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Time Course ◽  
Forced Expiratory Volume ◽  
Distinct Pattern ◽  
Degree Pattern ◽  
Function Test ◽  
The Mean ◽  
Exercise Induced ◽  
Mean Time

The purpose of this study was to assess the incidence, degree, pattern, and time course of pulmonary function test (PFT) changes measured after a dynamic exercise challenge. Forced expiratory maneuvers were performed before and serially after a 7-min run-walk in 44 patients with cystic fibrosis (CF). Twenty-four patients met at least one PFT criterion for exercise-induced bronchospasm (EIB) within 21 min after exercise, and 38 patients had >5% increase in a PFT parameter at some point after exercise. The mean time of greatest increase in PFTs occurred sooner than the greatest decrease, which was later than for 22 patients with asthma or allergic rhinitis found to have EIB. Age was inversely related to percentage fall in forced expiratory volume in 1 s (FEV,) after exercise. These results demonstrate a distinct pattern of PFTs measured after exercise in most patients with CF, with an early bronchodilation followed by a significant decrease in PFTs.

scholarly journals Liver Dysfunction may be Associated with QOL in Adults with CF

2019 ◽  
Vol 2 (1) ◽  
Author(s):  
Megana Rao, BS ◽  
Regina Weber, BS ◽  
Naga Chalasani, MD
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Alkaline Phosphatase ◽  
Liver Disease ◽  
Transient Elastography ◽  
Liver Stiffness ◽  
Future Research ◽  
Co Morbidity ◽  
The Mean

Background: More individuals with cystic fibrosis (CF) are living into their adulthood due to significant advances in medical care. The adult CF clinic at Indiana University cares for one of the largest adult populations with CF. Liver disease is common in children and adults with CF. However, the characteristics and consequences of CF liver disease (CFLD) in adults are not well understood. In this ongoing, IRB approved study, we systematically characterized the liver manifestations and their health related quality of life in adults with well characterized CF. Methods: Individual patient demographics and clinical data were collected for study participants. Participants completed two quality of life study questionnaires: Chronic Liver Disease Questionnaire (CLDQ) and Revised Cystic Fibrosis Questionnaire (CFQ-R). 10mL of blood was collected for future research. Finally, participants underwent a bedside transient elastography via FibroScan®. Results: There were 50 patients, with a mean age of 30.9 years, enrolled in the study. Pancreatic insufficiency was the most common co-morbidity, as it affected 90% of patients. The mean AST was 23.55 units/L, mean ALT was 25.20 units/L, and mean alkaline phosphatase level was 117.73 units/L. From the FibroScan®, the median liver stiffness measurement (LSM) was 4.65 kPa and the mean controlled attenuation parameter (CAP) was 219.52 dB/M. The mean CLDQ score was 5.343. Conclusion: This ongoing study reveals that increased alkaline phosphatase and CAP scores are associated with a poor QOL. More data is needed to further understand the pathophysiology behind CFLD. Use of noninvasive imaging, noninvasive markers, and the CLDQ may aid in early identification of adult CFLD.    

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