scholarly journals Targeting of dornase alpha therapy in adult cystic fibrosis

1998 ◽  
Vol 91 (7) ◽  
pp. 360-364 ◽  
Author(s):  
M J Ledson ◽  
Z Wahbi ◽  
R P Convery ◽  
C Cowperthwaite ◽  
D P Heaf ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Forced Expiratory Volume ◽  
Intravenous Antibiotic ◽  
Average Improvement ◽  
Clinical Indices ◽  
Dornase Alpha ◽  
Selection For ◽  
Alpha Therapy ◽  
Antibiotic Courses

Although dornase alpha (recombinant human DNase) can thin the viscid pulmonary secretions of cystic fibrosis (CF), clinical trials in groups of unselected patients have shown only modest average improvements in pulmonary function. The product is very expensive, so in conjunction with purchasers we designed selection criteria and a protocol for a 2-week trial to target CF individuals who might gain most benefit. Treatment was to be continued in those showing ≥10% improvement in pulmonary function. Those who had a trial of dornase alpha were followed up for 2 years. Of 25 patients who had a 2-week trial of dornase alpha, 17 met the criteria for continuation (average gain in forced expiratory volume 37%). The 11 of these who were still alive at 2 years had a greater initial average FEV1 improvement than those who had died (45% versus 22%), and still had an average improvement of 31% at 2 years. The 8 patients who did not meet the criteria for continuation were older and had required fewer intravenous antibiotic courses. All these were alive at 2 years with unchanged clinical indices. This method of selection for dornase alpha treatment allows targeting to those who gain most benefit without disadvantaging the remaining patients. Furthermore, production of such guidelines in conjunction with purchasers obviates funding difficulties and allows rational prescribing.

scholarly journals BODY MASS INDEX AND ALBUMIN LEVELS ARE ASSOCIATED WITH PULMONARY FUNCTION PARAMETERS IN PEDIATRIC SUBJECTS WITH CYSTIC FIBROSIS

2019 ◽  
Vol 37 (4) ◽  
pp. 414-418
Author(s):  
Miriam Isabel Souza dos Santos Simon ◽  
Gabriele Carra Forte ◽  
Paulo José Cauduro Marostica
Keyword(s):  
Body Mass Index ◽  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Body Mass ◽  
Univariate Analysis ◽  
Cross Sectional Study ◽  
Forced Expiratory Volume ◽  
Tertiary Referral Hospital ◽  
Cross Sectional ◽  
One Year

ABSTRACT Objective: To evaluate the association of body mass index (BMI) and albumin with pulmonary function in cystic fibrosis (CF) pediatric subjects. Methods: This is a cross-sectional study with clinically stable CF’s subjects. Clinical (pulmonary function) and nutritional evaluation (body mass index and albumin) were performed. Univariate analysis was performed using simple linear correlations. Regression analysis was performed using an exit level of p<0.05. Results: Seventy-eight CF’s subjects (mean age 12.8±3.8 years) with mean albumin 4.2±0.4 mg/dL, predicted forced expiratory volume in 1 second (FEV1%) 80.8±22.6 and BMI median percentile 51.2 (1.3-97.7). In the multiple regression models, albumin, age and BMI percentile were associated with pulmonary function. Subjects with lower than 25 BMI percentile had 12.2% lower FEV1%. An albumin increase of 0.1 mg was associated with 2.7% increase in predicted FEV1%, and one year increase in age was associated with reduction in 1.2% of predicted FEV1%. Conclusions: BMI percentile, albumin and age were independently associated with predicted FEV1% in a tertiary referral hospital.

scholarly journals Nutritional impact of antipseudomonas intravenous antibiotic courses in cystic fibrosis

1997 ◽  
Vol 76 (5) ◽  
pp. 437-440 ◽  
Author(s):  
P Vic ◽  
S Ategbo ◽  
F Gottrand ◽  
V Launay ◽  
G A Loeuille ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Intravenous Antibiotic ◽  
Antibiotic Courses

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 Nutritional impact of antipseudomonas intravenous antibiotic courses in cystic fibrosis

1998 ◽  
Vol 78 (2) ◽  
pp. 194-194
Author(s):  
G L BRIARS ◽  
S A MCNAUGHTON ◽  
G J CLEGHORN ◽  
R W SHEPHERD
Keyword(s):  
Cystic Fibrosis ◽  
Intravenous Antibiotic ◽  
Antibiotic Courses

scholarly journals Bronchodilator Responsiveness in Cystic Fibrosis Children Treated for Pulmonary Exacerbations

2021 ◽  
Author(s):  
Mordechai Pollak ◽  
Michelle Shaw ◽  
David Wilson ◽  
Melinda Solomon ◽  
Felix Ratjen ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Hospital Admissions ◽  
Forced Expiratory Volume ◽  
Clinical Value ◽  
Significant Drop ◽  
End Of Treatment ◽  
Repeated Events ◽  
Pulmonary Exacerbations ◽  
Response Testing

Background: Cystic fibrosis (CF) pulmonary exacerbations (PEx) are associated with significant drop in pulmonary function. The clinical value of measuring bronchodilator (BD) responsiveness during treatment for PEx to monitor or predict recovery of lung function is unclear. Methods: A retrospective analysis of spirometry with BD response testing obtained during hospital admissions for PEx in pediatric CF patients. Repeated events were included for patients with BD testing during multiple admissions. Results: 249 spirometries with BD testing in 102 patients were completed around day 7 (day 4-10) of hospital admission for treatment of CF PEx. Median (IQR) forced expiratory volume in one second (FEV1) was 70.6% predicted (58.1, 84.6) prior to the PEx event (best FEV1 in 6 months prior to admission), 54.4% (41.5, 66.9) at admission, 62.3% (48.4, 74.7) around day 7 of admission and 67.1% predicted (53.8, 78.2) at end of treatment. BD response around day 7 correlated poorly with FEV1 prior to PEx (r=-0.16, p=0.02), and did not correlate with recovery to baseline FEV1 at end of treatment (r=0.08, p=0.22). Only 23/249 (9%) patients had a BD response of ≥12 % and 200 ml. BD response was not related to age or severity of lung disease and led to an immediate change in clinical management in only 4 cases. CONCLUSIONS: BD response in CF patients treated for PEx is poorly correlated with baseline pulmonary function and does not correlate with recovery of FEV1 with treatment. These data suggest that routine testing for BD response is not indicated during PEx.

Endoscopic Sinus Surgery for Cystic Fibrosis: Variables Influencing Sinonasal and Pulmonary Outcomes

2021 ◽  
pp. 194589242110596
Author(s):  
Keven S. Y. Ji ◽  
Dennis Frank-Ito ◽  
Ralph Abi Hachem ◽  
Khalil Issa ◽  
Carrie Johnson ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Revision Surgery ◽  
Endoscopic Sinus Surgery ◽  
Stepwise Regression ◽  
Care Center ◽  
Tertiary Care ◽  
Forced Expiratory Volume ◽  
Older Age ◽  
Sinus Surgery

Background Endoscopic sinus surgery is a well-established treatment for chronic rhinosinusitis in patients with cystic fibrosis, though its benefits seem to be limited to improving sinonasal symptoms rather than affecting lung function. Objective This study aims to identify clinical and demographic factors that may influence sinonasal and pulmonary outcomes after surgery. Methods This is a six-year retrospective analysis of adult cystic fibrosis patients who underwent endoscopic sinus surgery at a tertiary care center. 22-Item Sino-Nasal Outcomes Test scores and mean forced expiratory volume data at baseline and three to six months after surgery were analyzed using t-test and stepwise regression with the following covariates: age, gender, lung transplant, revision surgery, and pseudomonas on sinus culture. Results 119 surgeries were performed on 88 patients, with 69% on patients with transplant. The overall mean (Standard Deviation) improvement in 22-Item Sino-Nasal Outcomes Test score was 9.42 (18.15) for the entire cohort ( P < .001). Pseudomonas on culture was associated with less improvement in sinonasal scores ( P = .002). There was no significant change in forced expiratory volume after surgery ( P = .94). Revision surgery ( P = .004) and older age ( P = .007) were associated with less favorable change of pulmonary function on stepwise regression ( P = .002). There was no correlation between change in sinonasal scores and pulmonary function. Conclusion Although surgery was associated with a clinically and statistically significant improvement in sinonasal scores in cystic fibrosis patients, patients with pseudomonas may experience less benefit. Revision surgery and older age may be associated with less favorable pulmonary outcomes. Awareness of such variables may help when deciding which cystic fibrosis patients should undergo surgery.

Wheezing in Infants with Cystic Fibrosis: Clinical Course, Pulmonary Function, and Survival Analysis

PEDIATRICS ◽  
1992 ◽  
Vol 90 (5) ◽  
pp. 703-706
Author(s):  
Joseph Reisman ◽  
Mary Corey ◽  
Gerard Canny ◽  
Henry Levison ◽  
Eitan Kerem ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Flow Rate ◽  
Forced Expiratory Volume ◽  
Common Finding ◽  
Study Cohort ◽  
Expiratory Phase ◽  
Significant Difference ◽  
Forced Expiratory Flow ◽  
Expiratory Flow

Wheezing is a common finding in infants with cystic fibrosis (CF). This study was undertaken to determine the prevalence of wheezing in infants with CF and to compare the clinical outcome of those who wheezed in infancy with that of those who did not. The study cohort included 229 CF patients born between 1965 and 1979 with CF diagnosed before 2 years of age. Fifty-seven (25%) had physician-documented wheezing during the first 2 years of life. Wheezing had resolved by the age of 2 years in 50% of the patients and by the age of 4 years in 75%. Although wheezing seemed to be linked to a family history of allergy and asthma, the frequency of the ΔF508 mutation was similar to that of the non-wheezers. There was no significant difference in survival at the age of 13 years between the two groups. At the age of 7 years, patients who had wheezed had significantly lower forced expiratory flow rate at mid-expiratory phase (85 ± 34% predicted) compared with those with no wheezing history (101 ± 34% predicted). At the age of 13 years, forced expiratory volume in 1 second values was lower in the wheezing group (69 ± 24% predicted vs 78 ± 21% predicted), as was forced expiratory flow rate at mid-expiratory phase (56 ± 33% predicted vs 69 ± 30% predicted). In conclusion, although wheezing in infants with CF seems to have diminished with age, pulmonary function abnormalities were more evident at 7 and 13 years of age in the group that wheezed than in the group that did not.

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.

Decreased Antibiotic Utilization After Sinus Surgery in Cystic Fibrosis Patients With Lung Transplantation

2019 ◽  
Vol 33 (4) ◽  
pp. 354-358 ◽  
Author(s):  
Tracy Z. Cheng ◽  
Kevin J. Choi ◽  
Adam L. Honeybrook ◽  
Rasheedat T. Zakare-Fagbamila ◽  
Alice L. Gray ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Respiratory Tract ◽  
Pulmonary Function ◽  
Lung Transplantation ◽  
Respiratory Tract Infections ◽  
Forced Expiratory Volume ◽  
Sinus Surgery ◽  
Least Squares Regression ◽  
Antibiotic Utilization ◽  
Tract Infections

Background Patients with cystic fibrosis (CF) who have undergone lung transplantation frequently require hospitalizations and antibiotic treatments for respiratory tract infections. Although endoscopic sinus surgery (ESS) improves sinonasal quality of life in CF patients, it is unclear if ESS offers additional benefit in terms of antibiotics for pulmonary infection, hospitalization, and pulmonary function. Objective To determine whether ESS impacts antibiotic use or hospitalizations for pulmonary indications or pulmonary function in CF patients after lung transplantation. Methods This is a single-institution retrospective study of all patients who underwent lung transplantation for CF from 2005 to 2017. Patients who underwent ESS at least 1 year after transplant were included. Paired bivariate analyses were performed to determine whether there was a difference in the frequency and length of hospitalizations for pulmonary indications, number of antibiotic courses (intravenous and oral) for pulmonary exacerbations, and forced expiratory volume in 1 second (FEV1) slope in the 6 months before versus after ESS. Perioperative antibiotics and hospitalizations were not included in the analyses. Least squares regression was utilized to analyze FEV1 trends. Results A total of 20 patients underwent 36 ESS during the study period. There was significantly higher antibiotic utilization in the 6 months before ESS (0.89 ± 1.03) compared to the 6 months after ESS (0.33 ± 0.53) ( P = .002). The frequency and length of hospitalizations, FEV1 slope, and FEV1 trend before and after ESS were not significantly different. Conclusion Our results suggest that ESS is associated with a reduction in the frequency of antibiotic utilization for respiratory tract infections in lung transplant recipients with CF. A prospective study is needed to investigate these relationships further.

scholarly journals Vitamin D intake, serum 25-hydroxy vitamin D and pulmonary function in paediatric patients with cystic fibrosis: a longitudinal approach

2018 ◽  
Vol 121 (2) ◽  
pp. 195-201 ◽  
Author(s):  
Nyanza K. L. M. Timmers ◽  
Rebecca K. Stellato ◽  
Cornelis K. van der Ent ◽  
Roderick H. J. Houwen ◽  
Janna W. Woestenenk
Keyword(s):  
Cystic Fibrosis ◽  
Vitamin D ◽  
Body Weight ◽  
Pulmonary Function ◽  
Significant Relationship ◽  
Forced Expiratory Volume ◽  
Vitamin D Supplementation ◽  
Vitamin D Intake ◽  
Nutritional Guidelines ◽  
25 Hydroxy Vitamin D

AbstractPancreatic-insufficient children with cystic fibrosis (CF) receive age-group-specific vitamin D supplementation according to international CF nutritional guidelines. The potential advantageous immunomodulatory effect of serum 25-hydroxy vitamin D (25(OH)D) on pulmonary function (PF) is yet to be established and is complicated by CF-related vitamin D malabsorption. We aimed to assess whether current recommendations are optimal for preventing deficiencies and whether higher serum 25(OH)D levels have long-term beneficial effects on PF. We examined the longitudinal relationship between vitamin D intake, serum 25(OH)D and PF in 190 CF children during a 4-year follow-up period. We found a significant relationship between total vitamin D intake and serum 25(OH)D (β = 0·02; 95 % CI 0·01, 0·03; P = 0·000). However, serum 25(OH)D decreased with increasing body weight (β = –0·79; 95 % CI –1·28, –0·29; P = 0·002). Furthermore, we observed a significant relationship between serum 25(OH)D and forced expiratory volume in 1 s (β = 0·056; 95 % CI 0·01, 0·102; P = 0·018) and forced vital capacity (β = 0·045; 95 % CI 0·008, 0·082; P = 0·017). In the present large study sample, vitamin D intake is associated with serum 25(OH)D levels, and adequate serum 25(OH)D levels may contribute to the preservation of PF in children with CF. Furthermore, to maintain adequate levels of serum 25(OH)D, vitamin D supplementation should increase with increasing body weight. Adjustments of the international CF nutritional guidelines, in which vitamin D supplementation increases with increasing weight, should be considered.

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