P175 Nutritional status, lung function and adequacy of micronutrient supplementation in children with cystic fibrosis

2021 ◽  
Vol 20 ◽  
pp. S92
Author(s):  
T. Niseteo ◽  
S. Sila ◽  
A. Špoljarić ◽  
L. Marić ◽  
M. Bosanac ◽  
...  
2020 ◽  
Vol 19 ◽  
pp. S155-S156
Author(s):  
T. Odobasic Palkovic ◽  
D. Tjesic-Drinkovic ◽  
D. Tjesic-Drinkovic ◽  
I. Godic ◽  
I. Lalic ◽  
...  

Author(s):  
Jessica Ng ◽  
Florian Friedmacher ◽  
Caroline Pao ◽  
Paul Charlesworth

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.


2018 ◽  
Vol 17 ◽  
pp. S6
Author(s):  
M. Ashkenazi ◽  
N. Nathan ◽  
I. Sarouk ◽  
B.E. Bar Aluma ◽  
A. Dagan ◽  
...  

2019 ◽  
Vol 7 (1) ◽  
Author(s):  
B D Edwards ◽  
R Somayaji ◽  
J Greysson-Wong ◽  
C Izydorczyk ◽  
B Waddell ◽  
...  

Abstract Background Analysis of “emerging” pathogens in cystic fibrosis (CF) lung disease has focused on unique pathogens that are rare in other human diseases or are drug resistant. Escherichia coli is recovered in the sputum of up to 25% of patients with CF, yet little is known about the epidemiology or clinical impact of infection. Methods We studied patients attending a Canadian adult CF clinic who had positive sputum cultures for E coli from 1978 to 2016. Infection was categorized as transient or persistent (≥3 positive sputum cultures, spanning >6 months). Those with persistent infection were matched 2:1 with age, sex, and time-period controls without history of E coli infection, and mixed-effects models were used to assess pulmonary exacerbation (PEx) frequency, lung function decline, hospitalization, and intravenous antibiotic days. Results Forty-five patients (12.3%) had E coli recovered from sputum samples between 1978 and 2016, and 18 patients (40%) developed persistent infection. Nine patients (24%) had PEx at incident infection, and increased bioburden was predictive of exacerbation (P = .03). Risk factors for persistent infection included lower nutritional status (P < .001) and lower lung function (P = .009), but chronic infection with Pseudomonas aeruginosa was protective. There was no difference in annual lung function decline, need for hospitalization or intravenous antibiotics, or risk of PEx in patients with persistent infection. Conclusions Persistent E coli infection was frequent and was more common in CF patients with low nutritional status and lung function. However, this does not predict clinical decline. Multicenter studies would allow better characterization of the epidemiology and clinical impact of E coli infection.


2021 ◽  
Vol 16 (2) ◽  
pp. 22-34
Author(s):  
I. Sokolov ◽  
◽  
E.A. Roslavtseva ◽  
V.P. Chernevich ◽  
N.I. Burkina ◽  
...  

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


Author(s):  
Woolf Walker ◽  
Amanda Harris ◽  
Bruna Rubbo ◽  
Victoria Keenan ◽  
Amanda Friend ◽  
...  

2006 ◽  
Vol 5 ◽  
pp. S70
Author(s):  
J.W. Woestenenk ◽  
H.G.M. Arets ◽  
C.K. van der Ent ◽  
N.M. de Roos

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