Nutritional status and pulmonary function in patients with cystic fibrosis with and without Burkholderia cepacia colonization: role of specialist dietetic support

1996 ◽  
Vol 9 (3) ◽  
pp. 173-179 ◽  
Author(s):  
Daniel Peckham ◽  
Clare Leonard ◽  
Simon Range ◽  
Alan Knox
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Pulmonary Function ◽  
Burkholderia Cepacia

Diabetes Mellitus in Patients With Cystic Fibrosis: Effect on Survival

PEDIATRICS ◽  
1990 ◽  
Vol 86 (3) ◽  
pp. 374-377
Author(s):  
J. Reisman ◽  
M. Corey ◽  
G. Canny ◽  
H. Levison
Keyword(s):  
Diabetes Mellitus ◽  
Cystic Fibrosis ◽  
Survival Analysis ◽  
Nutritional Status ◽  
Pulmonary Function ◽  
Survival Data ◽  
Diabetes Diagnosis ◽  
Patients With Diabetes ◽  
Insulin Dependent ◽  
Sick Children

Patient data obtained from the cystic fibrosis clinic of the Hospital for Sick Children (Toronto, Canada) over the period 1977 to 1988 were analyzed to compare the diabetic and nondiabetic cystic fibrosis patients. The pulmonary function, nutritional status, and survival data for 713 patients who attended the clinic over the 11-year period are reported. Insulin-dependent diabetes was found to exist in 37 (5.2%) of 713 patients. The patient age at time of diabetes diagnosis ranged from 2 to 34 years, with a mean ± SD of 20.0 ± 7.4 years. Patients who died in both the diabetic and nondiabetic groups had worse pulmonary and nutritional status than the surviving patients, but there were no significant differences between the diabetic and nondiabetic groups in those who died or in those who remained alive. Survival analysis showed a similar prognosis in the diabetic and nondiabetic groups. It is concluded that cystic fibrosis patients with diabetes are, for their age, not different from patients without diabetes with respect to pulmonary function, nutritional status, and survival.

scholarly journals The role of nutrition and pancreatic enzyme replacement therapy in children with cystic fibrosis

2021 ◽  
Vol 4 (2) ◽  
pp. 84-93
Author(s):  
Muzal Kadim ◽  
William Cheng
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Pancreatic Insufficiency ◽  
Pancreatic Enzyme ◽  
Nutritional Intervention ◽  
Enzyme Replacement ◽  
Pancreatic Enzyme Replacement Therapy

Background Cystic fibrosis (CF) is an inherited genetic disorder with high mortality and morbidity. CF is strongly correlated with malnutrition due to higher energy losses, pancreatic insufficiency, chronic inflammation, higher resting energy expenditure, and feeding problems. Malnutrition in CF patients associated with worse survival. Thus, appropriate and prompt nutritional intervention should be addressed to reduced malnutrition in CF patients. Methods The literature search was performed on 9 August 2021 in four major databases such as MEDLINE, EBSCOhost, Cochrane Reviews, and Web of Sciences to find the role of nutrition and pancreatic enzyme replacement therapy in pediatrics population with cystic fibrosis. Recent findings In recent decades, early nutritional management and pancreatic enzyme replacement therapy (PERT) have been shown to improve CF patient’s outcomes. Nutrition should be given in higher calories compared to healthy individuals with close and regular nutritional status monitoring. High protein and fat diets are essential for CF patient’s overall survival. Adequate level of micronutrients should be ensured to avoid morbidity caused by micronutrients deficiency. Regular pancreatic insufficiency screening should be done annually in order to start PERT early.  Further research focusing on body composition, growth chart, protein intake, and PERT are needed to further improve the management of CF patient. Conclusion Nutritional intervention and PERT play an important role in prolonging CF patient survival. Both treatments should be initiated early with nutritional status close monitoring and tailored to each individual. Collaboration with parents and children is critical to warrant that CF patients followed the dietary advice.

scholarly journals Correlation between pulmonary function and nutritional status, age, and bacterial colonization in cystic fibrosis (CF) patients

2008 ◽  
Vol 7 ◽  
pp. S63
Author(s):  
S. Lubovich ◽  
V. Rodriguez ◽  
S. Zaragoza ◽  
C. Kofman ◽  
L. Galanternik ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Pulmonary Function ◽  
Bacterial Colonization

scholarly journals Cedecea davisae’s Role in a Polymicrobial Lung Infection in a Cystic Fibrosis Patient

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Thayer G. Ismaael ◽  
Eleana M. Zamora ◽  
Faisal A. Khasawneh
Keyword(s):  
Cystic Fibrosis ◽  
Staphylococcus Aureus ◽  
Burkholderia Cepacia ◽  
Airway Disease ◽  
Lung Infection ◽  
Emerging Pathogen ◽  
The Family ◽  
Healthcare Associated ◽  
Airway Colonization

Chronic airway colonization and infection are the hallmark of cystic fibrosis (CF).Staphylococcus aureus, Pseudomonas aeruginosa, andBurkholderia cepaciaare well-documented bacterial culprits in this chronic suppurative airway disease. Advanced molecular diagnostics have uncovered a possible role of a larger group of microorganisms in CF.Cedeceais a member of the family Enterobacteriaceae and is an emerging pathogen. We present a case of a polymicrobial healthcare-associated pneumonia in a CF patient caused byCedecea davisae, among other bacteria.

scholarly journals Endoscopic sinus surgery in patients with cystic fibrosis: a systematic review and meta-analysis of pulmonary function

2012 ◽  
Vol 50 (4) ◽  
pp. 360-369
Author(s):  
K.I. Macdonald ◽  
A. Gipsman ◽  
A. Magit ◽  
M. Fandino ◽  
E. Massoud ◽  
...  
Keyword(s):  
Systematic Review ◽  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Endoscopic Sinus Surgery ◽  
Meta Analysis ◽  
Pulmonary Function Tests ◽  
Sinus Surgery ◽  
Intravenous Antibiotics

Introduction: The role of endoscopic sinus surgery (ESS) in patients with cystic fibrosis (CF) is not clearly defined. Objective: TO perform a systematic review of subjective and objective outcomes of ESS in CF. Methods: A systematic review was performed using the keywords 'sinusitis,' 'sinus surgery,' 'nasal polyps' and 'cystic fibrosis.' The quality of papers was assessed using the NICE scoring scale. Outcomes included safety, subjective symptoms, objective endoscopy scores, days spent in hospital, courses of antibiotics, and pulmonary function tests (PFTs). Results: Nineteen studies involving 586 patients were included in the review. There were four prospective cohort trials, and three were rated as good quality. There were no major complications attributable to ESS. There was consistent evidence in four cohort studies of improved sinonasal symptoms, including nasal obstruction, facial pain, headaches, rhinorrhea and olfaction. Three studies reported conflicting results in post-operative endoscopy scores. Three studies showed a decrease in days spent in hospital, and two showed a significant decrease in courses of intravenous antibiotics. A recent study, however, did not show a difference in either days spent in hospital or courses of antibiotics. Pulmonary function tests were not improved by ESS in six cohort trials, and one small study found significant improvement. A meta-analysis of FEV1 scores confirmed no significant difference. Conclusion: THE most consistent findings of this review were that ESS in patients with CF is safe, produces symptomatic benefit, and does not consistently improve PFTs. There were more conflicting results with regards to endoscopy scores, days spent in hospital, and courses of intravenous antibiotics. Future prospective studies, utilizing validated quality of life, symptom and endoscopy scales, are needed to further elucidate the role of ESS in the management of chronic rhinosinusitis in CF patients.

scholarly journals Cystic fibrosis and the role of the physiotherapist

1981 ◽  
Vol 37 (3) ◽  
pp. 72-74
Author(s):  
Anne Miot ◽  
J. M. Pettifor ◽  
I. Reef
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Function ◽  
Close Contact ◽  
Point Of View ◽  
Inherited Disease ◽  
Family Situation ◽  
Respiratory Involvement ◽  
The Family ◽  
Pulmonary Pathology

Cystic fibrosis is an inherited disease, the major problems being pancreatic malabsorption and progressive respiratory involvement, although numerous other organs may also be involved. The pulmonary pathology and the role of the physiotherapist in the treatment of cystic fibrosis is discussed. The physiotherapist plays an important role in the management of the child with cystic fibrosis, not only from the point of view of maintaining maximal pulmonary function but also because she comes into close contact with the family and can help in optimising the acceptance of the child into the family situation.

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