Portal de recerca
Nutritional status, nutrient intake and use of enzyme supplements in paediatric patients with Cystic Fibrosis; a European multicentre study with reference to current guidelines
Data de publicació:
Autors de IIS La Fe
Autors aliens a IIS La Fe
- Hulst, JM
- Asseiceira, I
- Claes, I
- Garriga, M
- Woodcock, S
- Martins, T
- Boon, M
- Ruperto, M
- Walet, S
- Speziali, C
- Witters, P
- Barreto, C
- de Boeck, K
- MyCyFAPP Project
Grups d'Investigació
Abstract
Background: The New European guidelines have established the most updated recommendations on nutrition and pancreatic enzyme replacement therapy (PERT) in CF. In the context of MyCyFAPP project - a European study in children with CF aimed at developing specific tools for improvement of self-management - the objective of the current study was to assess nutritional status, daily energy and macronutrient intake, and PERT dosing with reference to these new guidelines. Methods: Cross sectional study in paediatric patients with CF from 6 European centres. SD-scores for weight-for-age (WFA), height-for-age (HFA) and body mass index-for-age (BMI) were obtained. Through a specific 4-day food and enzyme-dose record, energy and macronutrients intake and PERT-use (LU/g lipids) were automatically calculated by the,MyCyFAPP system. Comparisons were made using linear regression models. Results: The lowest quartiles, for BMI and HFA were between 0 and -1SD in all the centres with no significant differences, and 33.5% of the patients had a SD-score <0 for all three parameters. The minimum energy intake recommendation was not reached by 40% of the children and mean nutrients intake values were 14%, 51% and 34% of the total energy for protein, carbohydrates and lipids respectively. When assessed per centre, reported PERT doses were in the recommended range in only 13.8% to 46.6% of the patients; from 5.6% up to 82.7% of children were above the recommended doses and 3.3% to 75% were below. Conclusion: Among the 6 centres, a large variability and inconsistency with new guidelines on nutrition and PERT-use was found. Our findings document the lack of a general criterion to adjust PERT and suggest the potential benefit of educational and self-managerial tools to ensure adherence to therapies, both for clinical staff and families. They will be taken into account when developing these new tools during the next stages of MyCyFAPP Project. (C) 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
Dades de la publicació
- ISSN/ISSNe:
- 1569-1993, 1873-5010
- Tipus:
- Article
- Pàgines:
- 510-518
- PubMed:
- 28320633
- Factor d'Impacte:
- 1,587 SCImago ℠
- Quartil:
- Q1 SCImago ℠
JOURNAL OF CYSTIC FIBROSIS ELSEVIER SCIENCE BV
Cites Rebudes en Web of Science: 27
Documents
- No hi ha documents
Filiacions
Keywords
- Cystic fibrosis; Nutritional status; Pancreatic insufficiency; PERT; Nutritional requirements macronutrients; Guidelines; Paediatrics; Self-management; Telemedicine
Projectes associats
ANALISIS DE NUEVOS GENES IMPLICADOS EN LA PATOGENESIS DE LA ENFERMEDAD CELIACA
Investigador Principal: CARMEN RIBES KONINCKX
PI02/0573 . INSTITUTO DE SALUD CARLOS III . 2004
INNOVATIVE APPROACH FOR SELF-MANAGEMENT AND SOCIAL WELFARE OF CYSTIC FIBROSIS PATIENTS IN EUROPE: DEVELOPMENT, VALIDATION AND IMPLEMENTATION OF A TELEMATICS TOOL. MyCyFAPP
Investigador Principal: CARMEN RIBES KONINCKX
643806_H2020-PHC-2014-single-stage-MyCyFApp . COMISION EUROPEA . 2015
NUEVA ESTRATEGIA PARA LA DERIVACIÓN DE HEPATOCITOS FUNCIONALES DE MUJERES AFECTAS DE DEFICIENCIA DE ORNITINA TRANSCARBAMILASA
Investigador Principal: BERNARDO ROQUE BORT MARTÍ
SAF2014-51991-R . MINISTERIO DE ECONOMIA Y COMPETITIVIDAD . 2015
UN REGISTRO NO INTERVENCIONISTA A LARGO PLAZO PARA EVALUAR LA SEGURIDAD Y EFECTIVIDAD DE HUMIRA® (ADALIMUMAB) EN PACIENTES PEDIÁTRICOS CON ENFERMEDAD DE CROHN (EDC) ACTIVA ENTRE MODERADA Y GRAVE (CAPE).
Investigador Principal: ESTHER DONAT ALIAGA
P11-292 . 2015
ESTUDIO ABIERTO, MULTICÉNTRICO PARA EVALUAR LA FARMACOCINÉTICA, LA SEGURIDAD Y LA EFICACIA DE OMBITASVIR (OBV), PARITAPREVIR (PTV), RITONAVIR (RTV), CON O SIN DASABUVIR (DSV) Y CON O SIN RIBAVIRINA (RBV) EN PACIENTES PEDIÁTRICOS CON INFECCIÓN CRÓNICA POR EL VIRUS DE LA HEPATITIS C (VHC) GENOTIPO 1 O 4 (ZIRCON).
Investigador Principal: CARMEN RIBES KONINCKX
M14-748 . 2016
ESTUDIO DE SEGURIDAD Y CAPACIDAD NUTRICIONAL DE UNA NUEVA FORMULA HIPOALERGICA PARA LACTANTES CON DIVERSAS FORMAS DE ALERGIA A LAS PROTEINAS DE LA LECHE DE VACA.
Investigador Principal: CARMEN RIBES KONINCKX
HER-FHT-2009-01 . 2010
A MULTICENTRE, RANDOMISED, PLACEBO-CONTROLLED, DOUBLE-BLIND STUDY OF THE EFFICACY, SAFETY, AND PHARMACOKINETICS OF LUBIPROSTONE IN PAEDIATRIC SUBJECTS AGED >= 6 YEARS TO < 18 YEARS WITH FUNCTIONAL CONSTIPATION. / ESTUDIO MULTICENTRICO, ALEATORIZADO, CONTR OLADO CON PLACEBO Y CON DOBLE ANONIMATO (DOBLE CIEGO) DE LA EFICACIA, SEGURIDAD Y FARMACOCINETICA DE LA LUBIPROSTONA EN PACIENTES PEDIATRICOS CON EDAD . 6 ANOS Y < 18 ANOS CON ESTRENIMIENTO FUNCIONAL.
Investigador Principal: CARMEN RIBES KONINCKX
SAG/0211PFC-1131
A MULTICENTRE, LONG-TERM SAFETY, EFFICACY AND PHARMACOKINETICS STUDY OF LUBIPROSTONE IN PAEDIATRIC SUBJECTS AGED >= 6 TO <18 YEARS WITH FUNCTIONAL CONSTIPATION./ ESTUDIO MULTICÉNTRICO A LARGO PLAZO SOBRE LA SEGURIDAD, LA EFICACIA Y LA FARMACOCINÉTICA DE L UBIPROSTONA EN PACIENTES PEDIÁTRICOS DE EDADES COMPRENDIDAS ENTRE >= 6 AÑOS Y < 18 AÑOS CON ESTREÑIMIENTO FUNCIONAL.
Investigador Principal: CARMEN RIBES KONINCKX
SAG/0211PFC-11S1 . 2015
ESTUDIO FARMACOEPIDEMIOLÓGICO PARA ESTABLECER EL PERFIL DE SEGURIDAD A LARGO PLAZO DE TENOFOVIR DISOPROXIL FUMARATO (TENOFOVIR DF, VIREAD®) Y DESCRIBIR EL TRATAMIENTO DE LA TOXICIDAD RENAL Y ÓSEA ASOCIADA A TENOFOVIR DF EN ADOLESCENTES DE 12 A <18 AÑOS CO N HEPATITIS B CRÓNICA (HBC) EN EUROPA.
Investigador Principal: CARMEN RIBES KONINCKX
GS-EU-174-1403 . 2015