Related Experiment Video

Updated: Aug 2, 2026

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
08:00

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study

Published on: April 11, 2018

[Nutritional intervention in children and adolescents with cystic fibrosis. Relationship with pulmonary function]

C Martínez-Costa1, A Escribano, F Núñez Gómez

  • 1Servicio de Pediatría, Hospital Clínico, Universidad de Valencia, España. ceciliam@comv.es

Nutricion Hospitalaria
|July 2, 2005
PubMed

Insights

Early nutritional intervention significantly improves weight and anthropometrics in pediatric cystic fibrosis (CF) patients. Good nutritional status is closely linked to better pulmonary function in CF.

Area of Science:

  • Pediatric Pulmonology
  • Nutritional Science
  • Genetic Diseases

Context:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, particularly the lungs and digestive system.
  • Pediatric patients with CF often face challenges in maintaining adequate nutritional status due to malabsorption and increased metabolic demands.
  • Early and continuous nutritional support is crucial for managing CF and its complications.

Purpose:

  • To evaluate the impact of early and consistent nutritional intervention on the nutritional status and pulmonary function of pediatric patients with cystic fibrosis (CF).
  • To explore the correlation between nutritional status and respiratory outcomes in young CF patients.
  • To determine the necessity and effectiveness of various nutritional support strategies in CF management.

Summary:

  • This study followed 19 pediatric CF patients (17 months to 18 years) over a mean of 10 years, assessing nutritional status (NS) and pulmonary function via regular clinical, anthropometrical, and spirometry evaluations.
  • Nutritional interventions included dietary recommendations, oral/invasive enteral nutrition (EN), and supplementation. Anthropometrical improvements, particularly in weight and skinfold thickness, were significant (p < 0.05).
  • Despite high rates of exocrine pancreatic failure (84%) and Pseudomonas aeruginosa colonization (73%), patients showed improved weight and a strong correlation between nutritional status (weight/height ratio) and pulmonary function (FVC, FEV1).

Impact:

  • Early and continuous nutritional intervention, including enteral nutrition (EN), is vital for improving anthropometric measures in pediatric CF patients.
  • Maintaining optimal nutritional status is directly correlated with better pulmonary function (FVC and FEV1) in children with CF.
  • Effective nutritional monitoring and support are essential for meeting the demands of CF and improving overall patient outcomes.
Abstract

More Related Videos

Design and Development of a Model to Study the Effect of Supplemental Oxygen on the Cystic Fibrosis Airway Microbiome07:38

Design and Development of a Model to Study the Effect of Supplemental Oxygen on the Cystic Fibrosis Airway Microbiome

Published on: August 3, 2021

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis05:56

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Acute Respiratory Failure-V01:29

Acute Respiratory Failure-V

The treatment for acute respiratory failure varies based on factors like the underlying cause, overall health, and severity. A collaborative healthcare team is essential for early detection, often through arterial blood gas analysis. Identifying the cause is the primary goal, with treatment strategies adjusted for ventilation/perfusion (V/Q) mismatch, shunting, or diffusion impairment.
Ensure that patients are monitored continuously for their response to therapy, including changes in...
Pulmonary Function Tests01:25

Pulmonary Function Tests

Pulmonary Function Tests (PFTs)
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
Inflammatory Bowel Disease III: Diagnostic Studies and Management I-Nutritional Therapy01:30

Inflammatory Bowel Disease III: Diagnostic Studies and Management I-Nutritional Therapy

Various diagnostic tests are employed in the diagnostic process for Inflammatory Bowel Disease (IBD), particularly to differentiate between Crohn's disease and ulcerative colitis.
Diagnostic studies
A colonoscopy is the definitive screening test, distinguishing ulcerative colitis from other colon diseases with similar symptoms. During a colonoscopy test, inflamed mucosa with exudate ulcerations can be observed, and biopsies are taken to determine the histologic characteristics of the colonic...
Chronic Kidney Disease III: Interprofessional Care01:28

Chronic Kidney Disease III: Interprofessional Care

Chronic kidney disease (CKD) requires collaborative and comprehensive management. CKD progresses through stages and can lead to end-stage kidney disease (ESKD) if untreated. Interprofessional collaboration and patient education are crucial, enabling patients to manage their health and improve their quality of life.Diagnostic approach for chronic kidney diseaseThe diagnosis of CKD primarily focuses on the glomerular filtration rate (GFR), which assesses kidney function by measuring how well...