NETwork of Linoleic Acid Supplementation in Cystic Fibrosis

Summary

Undernutrition is a common problem in patients with cystic fibrosis (CF) despite international consensus that the patients shall be given 120-200% of energy recommendations. Studies imply that one problem might be that the patients are not compensated for the essential fatty acid deficiency (linoleic acid, LA), which is well known in these patients. This deficiency is shown not to be due to fat malabsorption, but related to an increased turnover of arachidonic acid, a transformation product of LA. This abnormality is related to mutations associated with a more severe clinical phenotype. The most common and typical symptom of LA deficiency is poor growth. Studies in animals have further indicated that many of the symptoms in CF are related to the deficiency. A series of recent prospective studies from Wisconsin corroborate the importance of LA for growth. In Sweden LA has been supplemented to most patients since the late 70´, and the condition of patients have been among the leading in the world regarding growth, pulmonary function and survival. Short-term studies have shown better effect of LA supplementation compared to similar supply of energy without including extra LA. There are few long-term studies, performed before the gene was identified, giving very heterogeneous patient groups in regard to genotype, but with some positive results on growth and physiology. It´s of interest that modern personalized extremely expensive therapy with correctors and potentiators for Cystic Fibrosis Transmembrane Conductance Regulator may influence lipid metabolism. LA might thus tentatively be a cheap adjuvant to this modern therapy, but this has to be specially studied.

The aim of the study is to find if there are differences in clinical and metabolic outcome between two groups, blindly given similar amount of extra calories, in one group consisting of linoleic acid.The benefit for the patients would be great if the expected positive effect can be proved in the planned study. The treatment will be cheap and without adverse effects. From socioeconomic point of view is would be a great advantage.

Conditions

• Cystic Fibrosis

Interventions

DIETARY_SUPPLEMENT

linoleic acid supplementation

Oils given daily at morning meal with extra enzymes

DIETARY_SUPPLEMENT

oleic acid supplementation

Oils given at morning meal with extra enzymes

Sponsors & Collaborators

• European Society of Pediatric Gastroenterology, Hepatology and Nutrition

  collaborator OTHER

• Karolinska Institutet

  lead OTHER

Principal Investigators

• Jaroslaw Walkowiak, MD,PhD · University of Poznan, CF center, Poland

• Carla Colombo, MD,PhD · University of Milan, CF center, Italy

• Egil Bakkeheim, MD, PhD · University of Oslo, CF center, Norway

• Raffaele Badolato, MD, PhD · University of Brescia, CF center, Italy

• Christine Rönne-Hansen, Md, PhD · Lund University, CF center, Sweden

Study Design

Allocation

RANDOMIZED

Purpose

TREATMENT

Masking

QUADRUPLE

Model

PARALLEL

Eligibility

Min Age

5 Years

Max Age

15 Years

Sex

ALL

Healthy Volunteers

No

Timeline & Regulatory

Start

2021-10-25

Primary Completion

2024-10-31

Completion

2024-11-05

Countries

• Italy
• Norway
• Poland

Study Locations