Bronchodilator Responsiveness in Cystic Fibrosis Children Treated for Pulmonary Exacerbations

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.

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

Journal of Investigative Medicine ◽

10.1136/jim-2019-001183 ◽

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.

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A first-year dornase alfa treatment impact on clinical parameters of patients with cystic fibrosis: the Brazilian cystic fibrosis multicenter study

Revista Paulista de Pediatria ◽

10.1590/s0103-05822013000400002 ◽

2013 ◽

Vol 31 (4) ◽

pp. 420-430 ◽

Cited By ~ 2

Author(s):

Tatiana Rozov ◽

Fernando Antônio A. e Silva ◽

Maria Angélica Santana ◽

Fabíola Villac Adde ◽

Rita Heloisa Mendes

Keyword(s):

Cystic Fibrosis ◽

Emergency Room ◽

Age Groups ◽

Forced Expiratory Volume ◽

First Year ◽

Age Group ◽

Emergency Room Visits ◽

Dornase Alfa ◽

Pulmonary Exacerbations ◽

One Year

OBJECTIVE: To describe the clinical impact of the first year treatment with dornase alfa, according to age groups, in a cohort of Brazilian Cystic Fibrosis (CF) patients. METHODS: The data on 152 eligible patients, from 16 CF reference centers, that answered the medical questionnaires and performed laboratory tests at baseline (T0), and at six (T2) and 12 (T4) months after dornase alfa initiation, were analyzed. Three age groups were assessed: six to 11, 12 to 13, and >14 years. Pulmonary tests, airway microbiology, emergency room visits, hospitalizations, emergency and routine treatments were evaluated. Student's t-test, chi-square test and analysis of variance were used when appropriated. RESULTS: Routine treatments were based on respiratory physical therapy, regular exercises, pancreatic enzymes, vitamins, bronchodilators, corticosteroids, and antibiotics. In the six months prior the study (T0 phase), hospitalizations for pulmonary exacerbations occurred in 38.0, 10.0 and 61.4% in the three age groups, respectively. After one year of intervention, there was a significant reduction in the number of emergency room visits in the six to 11 years group. There were no significant changes in forced expiratory volume in one second (VEF1), in forced vital capacity (FVC), in oxygen saturation (SpO2), and in Tiffenau index for all age groups. A significant improvement in Shwachman-Kulczychi score was observed in the older group. In the last six months of therapy, chronic or intermittent colonization by P. aeruginosa was detected in 75.0, 71.4 and 62.5% of the studied groups, respectively, while S. aureus colonization was identified in 68.6, 66.6 and 41.9% of the cases. CONCLUSIONS: The treatment with dornase alfa promoted the maintenance of pulmonary function parameters and was associated with a significant reduction of emergency room visits due to pulmonary exacerbations in the six to 11 years age group, with better clinical scores in the >14 age group, one year after the intervention.

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

Revista Paulista de Pediatria ◽

10.1590/1984-0462/;2019;37;4;00016 ◽

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.

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

Immunopathologia Persa ◽

10.15171/ipp.2020.06 ◽

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.

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Complete versus Limited Endoscopic Sinus Surgery for Chronic Rhinosinusitis in Adults with Cystic Fibrosis

Otolaryngology ◽

10.1177/0194599820904956 ◽

2020 ◽

Vol 162 (4) ◽

pp. 572-580 ◽

Cited By ~ 1

Author(s):

Daniel J. Lee ◽

Christopher M. K. L. Yao ◽

Jenna Sykes ◽

Leena Rizvi ◽

Elizabeth Tullis ◽

...

Keyword(s):

Cystic Fibrosis ◽

Lung Function ◽

Postoperative Period ◽

Endoscopic Sinus Surgery ◽

Hospital Admissions ◽

Care Center ◽

Sinus Surgery ◽

Oral Antibiotic ◽

Health Care Center ◽

Pulmonary Exacerbations

Objective To examine the effects of the extent of endoscopic sinus surgery (ESS) on pulmonary health, including the pulmonary exacerbations and lung function in patients with cystic fibrosis (CF). Study Design Retrospective cohort study. Setting Tertiary health care center. Subjects and Methods A retrospective review of patients with CF who underwent ESS at St. Michael’s Hospital between 1999 and 2016 was performed. Two groups of patients were identified based on the surgical extent: (1) complete (maxillary antrostomy, complete ethmoidectomy, sphenoidotomy, and frontal sinusotomy) and (2) limited (any ESS that involved less than complete). Primary outcomes included the number of pulmonary exacerbations (the use of oral or intravenous [IV] antibiotics), number of hospital admissions and hospital days during a 2-year pre- and postoperative period, and pulmonary function outcomes during a 1-year pre- and postoperative period. Results There were 70 procedures (30 complete and 40 limited) among 57 patients. Baseline characteristics were similar between the groups. Complete ESS group had a significant reduction in the oral antibiotic use compared to the limited ESS group (median, –1.0 [interquartile range (IQR), –2 to 0] in complete vs 0 [IQR, –1 to 1] in limited, P = .028). There was no difference in the use of IV antibiotics, number and duration of admissions, or rate of lung function change between the 2 groups. Conclusion Complete ESS may reduce mild forms of pulmonary exacerbations as shown in the decreased use of oral antibiotics. Overall, ESS does not significantly modify pulmonary outcomes in patients with CF.

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

American Journal of Rhinology and Allergy ◽

10.1177/19458924211059606 ◽

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.

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

PEDIATRICS ◽

10.1542/peds.90.5.703 ◽

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.

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Targeting of dornase alpha therapy in adult cystic fibrosis

Journal of the Royal Society of Medicine ◽

10.1177/014107689809100705 ◽

1998 ◽

Vol 91 (7) ◽

pp. 360-364 ◽

Cited By ~ 4

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.

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

Pediatric Exercise Science ◽

10.1123/pes.6.3.275 ◽

1994 ◽

Vol 6 (3) ◽

pp. 275-286 ◽

Cited By ~ 1

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.

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