scholarly journals Nutritional status, nutrient intake and use of enzyme supplements in paediatric patients with Cystic Fibrosis; a European multicentre study with reference to current guidelines

2017 ◽  
Vol 16 (4) ◽  
pp. 510-518 ◽  
Author(s):  
Joaquim Calvo-Lerma ◽  
Jessie M. Hulst ◽  
Inês Asseiceira ◽  
Ine Claes ◽  
Maria Garriga ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Multicentre Study ◽  
Nutrient Intake ◽  
Paediatric Patients ◽  
Current Guidelines

scholarly journals DeltaF508 CFTR Hetero- and Homozygous Paediatric Patients with Cystic Fibrosis Do Not Differ with Regard to Nutritional Status

Nutrients ◽  
2021 ◽  
Vol 13 (5) ◽  
pp. 1402
Author(s):  
Aleksandra Mędza ◽  
Katarzyna Kaźmierska ◽  
Bartosz Wielgomas ◽  
Lucyna Konieczna ◽  
Ilona Olędzka ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Serum Levels ◽  
The Body ◽  
Erythrocyte Membranes ◽  
Paediatric Patients ◽  
Patient Groups ◽  
The Mean ◽  
Body Mass Indexes ◽  
Homozygous Patient

The purpose of this study was to compare the nutritional status between deltaF508 CFTR hetero- and homozygous paediatric patients with cystic fibrosis. We assessed the percentage profiles of fatty acids measured in erythrocyte membranes and the serum levels of vitamins A, D3, E and K1 in the studied groups. We also measured the weights and heights and calculated the body mass indexes (BMIs). The studied groups consisted of 34 heterozygous and 30 homozygous patients. No statistically significant differences were found in the serum vitamins or erythrocyte membrane fatty acid profiles between the hetero- and homozygous patient groups, except for heptadecanoic acid (p = 0.038). The mean percentiles of height, weight and BMI did not differ significantly between the two groups. The homozygous and heterozygous paediatric patients with cystic fibrosis were similar in terms of their nutritional statuses.

Long‐term clinical outcome of cystic fibrosis paediatric patients presenting with meconium ileus

2020 ◽  
Vol 109 (12) ◽  
pp. 2738-2739
Author(s):  
Ioanna Loukou ◽  
Maria Moustaki ◽  
Marina Plyta ◽  
Konstantinos Douros
Keyword(s):  
Cystic Fibrosis ◽  
Clinical Outcome ◽  
Meconium Ileus ◽  
Paediatric Patients

Defining DIOS and Constipation in Cystic Fibrosis With a Multicentre Study on the Incidence, Characteristics, and Treatment of DIOS

2010 ◽  
Vol 50 (1) ◽  
pp. 38-42 ◽  
Author(s):  
Roderick H Houwen ◽  
Hubert P van der Doef ◽  
Isabelle Sermet ◽  
Anne Munck ◽  
Bruno Hauser ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Multicentre Study

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.

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