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 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 Impact of chronic khat (Catha edulis Forsk) chewing on pulmonary function test and oxygen saturation in humans: A comparative study

2019 ◽  
Vol 7 ◽  
pp. 205031211882461 ◽  
Author(s):  
Gashaw Garedew Woldeamanuel ◽  
Teshome Gensa Geta
Keyword(s):  
Lung Function ◽  
Pulmonary Function ◽  
Oxygen Saturation ◽  
Vital Capacity ◽  
Forced Expiratory Volume ◽  
Mean Values ◽  
Khat Chewing ◽  
Significant Difference ◽  
The Mean

Background: Chronic consumption of khat affects many organ systems and leads to various health disturbances in the chewers. Few studies examined the acute effects of khat ingestion on lung function parameters. However, studies which assessed the long-term effects of khat chewing on pulmonary function parameters and oxygen saturation are lacking. Objective: The aim of this study was to assess the impact of chronic Khat chewing on pulmonary function parameters and oxygen saturation among chronic Khat chewers in Wolkite, Ethiopia. Methods: A community-based comparative cross-sectional study was conducted in Wolkite, Ethiopia from 1 June 2018 to 15 August 2018. A total of 324 participants, 162 khat chewers and 162 non-chewers were included in the study. The data were collected through face-to-face interview by trained data collectors. British Medical Research Council respiratory questionnaire was used to assess respiratory symptoms. A spirometer was used to assess various lung function parameters. Moreover, oxygen saturation of hemoglobin was measured using pulse oximeter. Data were entered into CSPro version 6.2 and analyzed using SPSS version 23. Results: This study showed statistically significant (p < 0.05) reduction in the mean values of forced vital capacity, forced expiratory volume in first second and maximum ventilation volume among khat chewers as compared to non-chewers. There was no significant difference in the mean values of other lung function parameters between the two groups. Similarly, there was no significant difference (p = 0.642) in mean oxygen saturation of hemoglobin (SaO2) across the two groups. Conclusion: It is evident from this study that long-term khat consumption is associated with decreased mean forced vital capacity, forced expiratory volume in first second and maximum ventilation volume. Hence, there is a need for further study to strengthen the current findings and to explore the mechanisms of khat chewing effect on lung function parameters.

P356 Can nurse-provided education affect improvement of lung function and nutritional status in adult patients with cystic fibrosis?

2020 ◽  
Vol 19 ◽  
pp. S155-S156
Author(s):  
T. Odobasic Palkovic ◽  
D. Tjesic-Drinkovic ◽  
D. Tjesic-Drinkovic ◽  
I. Godic ◽  
I. Lalic ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Nutritional Status ◽  
Adult Patients

scholarly journals PREVALENCE OF HEPATIC STEATOSIS AMONG CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS AND ITS ASSOCIATION WITH NUTRITIONAL STATUS

2019 ◽  
Vol 37 (4) ◽  
pp. 435-441
Author(s):  
Amanda Oliva Gobato ◽  
Ana Carolina Junqueira Vasques ◽  
Antonio Fernando Ribeiro ◽  
Roberto Massao Yamada ◽  
Gabriel Hessel
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Children And Adolescents ◽  
Hepatic Steatosis ◽  
Statistical Tests ◽  
The Body ◽  
Significant Difference ◽  
Group A ◽  
The Mean ◽  
Group B

ABSTRACT Objective: To determine the prevalence of hepatic steatosis (HS) in children and adolescents with cystic fibrosis (CF) and associate it with nutritional status. Methods: Cross-sectional study with children and adolescents with CF diagnosis. Weight and height were used to calculate the body mass index (BMI) and subsequent classification of the nutritional status. The midarm circumference (MAC), triceps skinfold thickness (TSF) and midarm muscle circumference (MAMC) were used to evaluate body composition. Abdominal ultrasonography was performed for diagnosis of HS. The statistical tests used were Student’s t test, Mann-Whitney test and chi-square test with significance level of 5%. Results: 50 patients with CF were evaluated, 18 (36%) were diagnosed with HS (Group A) and 32 (64%) without HS (Group B). The mean age of Group A was 13,2±4,9 years old and Group B 11,7±4,9; for BMI, the value for Group A was 18,0±4,1 and Group B was 15,7±3,8; the TSF of Group A was 8,4±3,5 mm and Group B was 7,0±2,5 mm. For these variables, there was no significant difference between the groups. The mean of MAC and MAMC differed significantly between the groups, being higher in the HS group, with p values of 0,047 and 0,043. Conclusions: The frequency of HS in patients with CF is high and it is not related to malnutrition, according to the parameters of BMI, TSF and MAMC. The values of MAC and MAMC indicated a greater reserve of muscle mass in patients with HS.

scholarly journals Gastroesophageal Reflux Disease and Need for Antireflux Surgery in Children with Cystic Fibrosis: A Systematic Review on Incidence, Surgical Complications, and Postoperative Outcomes

2020 ◽  
Author(s):  
Jessica Ng ◽  
Florian Friedmacher ◽  
Caroline Pao ◽  
Paul Charlesworth
Keyword(s):  
Cystic Fibrosis ◽  
Postoperative Complications ◽  
Gastroesophageal Reflux Disease ◽  
Lung Function ◽  
Gastroesophageal Reflux ◽  
Nutritional Status ◽  
Reflux Disease ◽  
Antireflux Surgery ◽  
Published Data ◽  
Refractory Gerd

Abstract Introduction Gastroesophageal reflux disease (GERD) is associated with accelerated decline in lung health in children with cystic fibrosis (CF). Thus, antireflux surgery (ARS) is offered to a selected CF cohort with refractory GERD, but outcomes remain poorly investigated. This study aimed to determine the incidence of GERD in children with CF and to evaluate complications and outcomes of ARS. Materials and Methods A systematic literature-based search was conducted using various online databases according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The number of GERD cases in pediatric CF cohorts who underwent diagnostic investigation(s) was recorded. Data on postoperative complications and outcomes (including symptoms, lung function, and nutritional status) following ARS were analyzed. Results Ten articles (n = 289 patients) met the defined inclusion criteria (51% male; age range, 0.5 month–36 years). The overall incidence of GERD was 46% (range, 19–81%), derived from seven studies (n = 212 patients). Four publications (n = 82 patients) reported on ARS due to uncontrolled GERD. All ARSs were Nissen fundoplication (majority with gastrostomy placement). Major postoperative complications occurred in 15 (18%) patients, two required redo-ARS. Median follow-up time was 2 years (range, 3 months–6 years); 59% showed symptom improvement, and pulmonary exacerbations and decline in lung function were reduced. Nutritional status mainly improved in milder CF cases. There were no deaths related to ARS. Conclusion Approximately half of pediatric CF patients have GERD. Published data for children with CF are limited and heterogeneous in terms of GERD diagnosis and outcomes following ARS. However, ARS has shown to slow the deterioration of lung function in CF.

scholarly journals Integrating latent classes in the Bayesian shared parameter joint model of longitudinal and survival outcomes

2020 ◽  
Vol 29 (11) ◽  
pp. 3294-3307
Author(s):  
Eleni-Rosalina Andrinopoulou ◽  
Kazem Nasserinejad ◽  
Rhonda Szczesniak ◽  
Dimitris Rizopoulos
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Latent Class ◽  
Joint Model ◽  
Forced Expiratory Volume ◽  
Optimal Number ◽  
Latent Classes ◽  
Joint Models ◽  
Number Of Classes ◽  
Strength Of Association

Cystic fibrosis is a chronic lung disease requiring frequent lung-function monitoring to track acute respiratory events (pulmonary exacerbations). The association between lung-function trajectory and time-to-first exacerbation can be characterized using joint longitudinal-survival modeling. Joint models specified through the shared parameter framework quantify the strength of association between such outcomes but do not incorporate latent sub-populations reflective of heterogeneous disease progression. Conversely, latent class joint models explicitly postulate the existence of sub-populations but do not directly quantify the strength of association. Furthermore, choosing the optimal number of classes using established metrics like deviance information criterion is computationally intensive in complex models. To overcome these limitations, we integrate latent classes in the shared parameter joint model through a fully Bayesian approach. To choose the optimal number of classes, we construct a mixture model assuming more latent classes than present in the data, thereby asymptotically “emptying” superfluous latent classes, provided the Dirichlet prior on class proportions is sufficiently uninformative. Model properties are evaluated in simulation studies. Application to data from the US Cystic Fibrosis Registry supports the existence of three sub-populations corresponding to lung-function trajectories with high initial forced expiratory volume in 1 s ( FEV1), rapid FEV1 decline, and low but steady FEV1 progression. The association between FEV1 and hazard of exacerbation was negative in each class, but magnitude varied.

scholarly journals 1014 Relationship Between Lung Function and Obstructive Sleep Apnea in Cystic Fibrosis

SLEEP ◽  
2020 ◽  
Vol 43 (Supplement_1) ◽  
pp. A385-A385
Author(s):  
A Shakkottai ◽  
S Z Nasr ◽  
F Hassan ◽  
L M O’Brien ◽  
R D Chervin
Keyword(s):  
Cystic Fibrosis ◽  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Lung Function ◽  
Forced Expiratory Volume ◽  
Apnea Hypopnea Index ◽  
Lung Function Decline ◽  
Obstructive Sleep ◽  
Larger Sample ◽  
Training Grant

Abstract Introduction The frequency of obstructive sleep apnea (OSA) may be high among patients with cystic fibrosis (CF), a life-shortening, genetic respiratory disease that affects approximately 30,000 Americans. Yet, the potential relationship between OSA and lung function has not been thoroughly explored. Methods Single-center retrospective review of polysomnography (PSG) results from 2009-2017 in referred patients with CF and available pulmonary function data (PFTs) obtained at time of PSG and at 3, 6, 9, and 12-months prior. Results Mean ages were 11.1±3.9 (sd) and 37.1±14.1 years, among 18 children and 16 adults, respectively. Mean body mass index (BMI) was normal in both groups (62.5±26.6% in children; 25.1±6.4 kg/m2 in adults). Twenty-six subjects (76%) had OSA (apnea-hypopnea index &gt;1 in children, ≥5 in adults). Mean forced expiratory volume in 1 second percent predicted (FEV1 PPD) was higher among subjects with vs. without OSA at PSG and at each time-point in the year prior, independent of age and BMI at PSG (longitudinal mixed effects model, β=19.0, SE=8.1, p=0.028). While FEV1 PPD remained unchanged in the non-OSA group, FEV1 PPD at PSG was lower, in comparison to the year prior in subjects with OSA, with the greatest difference observed at 9-months prior to PSG (2-sample t-test, difference of -6.6% vs 0.6% in OSA vs. non-OSA groups respectively, p=0.078). Conclusion The PFTs, as daytime markers of CF lung disease severity, do not seem to reliably predict risk for OSA. In our sample, CF patients with vs. without OSA had better PFTs at baseline but they also showed a greater tendency for decline in PFTs over the year prior to OSA diagnosis. Larger sample size and longer duration of assessment may help, going forward, to assess any potential adverse impact of OSA on lung function decline. Support NIH Training Grant (T32NS007222, F32HL145915)

Airway reactivity and lung function in triiodothyronine-induced thyrotoxicosis

1985 ◽  
Vol 58 (5) ◽  
pp. 1485-1488 ◽  
Author(s):  
R. S. Irwin ◽  
M. R. Pratter ◽  
D. H. Stivers ◽  
L. E. Braverman
Keyword(s):  
Lung Function ◽  
Pulmonary Function ◽  
Respiratory Symptoms ◽  
Pulmonary Function Tests ◽  
Forced Expiratory Volume ◽  
Base Line ◽  
Airway Reactivity ◽  
Percent Change ◽  
Serum T3 ◽  
The Mean

To evaluate the possible relationship between asthma and hyperthyroidism, airway reactivity and lung function were prospectively compared in healthy volunteers before, during, and after liothyronine (triiodothyronine, T3)-induced hyperthyroidism. Base-line evaluation of the 10 subjects included clinical evaluation, thyroid and pulmonary function tests, and airway reactivity assessed by methacholine inhalational challenge (MIC). All studies were normal. During T3-induced hyperthyroidism, no subject developed respiratory symptoms or changes in pulmonary function or airway reactivity. The mean percent change in forced expiratory volume at 1 s from base line (delta FEV1) of -2.4 +/- 3.0 after MIC was not significantly different from that obtained before T3 administration (-1.4 +/- 1.5, P greater than 0.2). When all serum T3 concentrations and delta FEV1 values before, during and after T3-induced hyperthyroidism were compared, there was no significant correlation. We conclude that T3-induced hyperthyroidism of 3-wk duration has no effect on airway reactivity or lung function in normal volunteers.

scholarly journals Lung function over the life course of paediatric and adult patients with cystic fibrosis from a large multi-centre registry

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Arul Earnest ◽  
Farhad Salimi ◽  
Claire E. Wainwright ◽  
Scott C. Bell ◽  
Rasa Ruseckaite ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Mixed Model ◽  
Linear Mixed Model ◽  
Pancreatic Insufficiency ◽  
Forced Expiratory Volume ◽  
Rate Of Change ◽  
Data Registry ◽  
Mortality Outcome ◽  
The Impact

Abstract A key measure of lung function in people with Cystic Fibrosis (CF) is Forced Expiratory Volume in the first second FEV1 percent predicted (FEV1pp). This study aimed to address challenges in identifying predictors of FEV1pp, specifically dealing with non-linearity and the censoring effect of death. Data was obtained from a large multi-centre Australian Cystic Fibrosis Data Registry (ACFDR). A linear mixed model was used to study FEV1pp as the endpoint. There were 3655 patients (52.4% male) included in our study. Restricted cubic splines were used to fit the non-linear relationship between age of visit and FEV1pp. The following predictors were found to be significant in the multivariate model: age of patient at visit, BMI z-score, age interaction with lung transplantation, insulin dependent diabetes, cirrhosis/portal hypertension, pancreatic insufficiency, Pseudomonas aeruginosa infection and baseline variability in FEV1pp. Those with P. aeruginosa infection had a lower mean difference in FEV1pp of 4.7 units, p < 0.001 compared to those who did not have the infection. Joint modelling with mortality outcome did not materially affect our findings. These models will prove useful for to study the impact of CFTR modulator therapies on rate of change of lung function among patients with CF.

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