Literature DB >> 11045840

Energy supplements rich in linoleic acid improve body weight and essential fatty acid status of cystic fibrosis patients.

G Steinkamp1, H Demmelmair, I Rühl-Bagheri, H von der Hardt, B Koletzko.   

Abstract

BACKGROUND: Patients with cystic fibrosis who have steatorrhea frequently are underweight and have essential fatty acid (EFA) depletion, which is associated with a poor clinical course. It has been stated that poor EFA status is difficult to correct in patients with cystic fibrosis, and an impaired EFA metabolism with reduced synthesis of long-chain polyunsaturated fatty acids has been proposed. In this study, the effects of an oral energy supplement rich in linoleic acid were investigated in patients with cystic fibrosis who had a body weight below 95% of normal for height.
METHODS: Thirty-six patients (16 girls) more than 4 years of age were randomized either to a control group (n = 20, age 13.3 +/- 3.8 years, mean +/- SD) receiving intensive dietary counseling only, or an intervention group (n = 16, age, 10.4 +/- 4.3 years) treated for 3 months with dietary counseling plus 628 +/- 254 mL (= kcal) per day of an energy supplement rich in fat (31% of energy) and linoleic acid (16% of energy).
RESULTS: In contrast to the control group, the patients with supplemented diets achieved significant increases of energy intake (2189 +/- 731 kcal/day vs. 2733 +/- 762 kcal/day), weight for height (82.8% +/- 8.6% vs. 84.8% +/- 9.6% of normal), and body fat (5.1 +/- 1.7 kg vs. 5.8 +/- 2.2 kg) as well as the initially low values of plasma phospholipid linoleic acid (11.8% +/- 1.1% vs. 17.6% +/- 1.6% of total phospholipid fatty acids) and its main metabolite arachidonic acid (4.4% +/- 0.4% vs. 5.9% +/- 0.3%).
CONCLUSIONS: Patients with cystic fibrosis with low body weight and poor EFA status benefit from EFA-rich energy supplements and can synthesize arachidonic acid from the precursor linoleic acid.

Entities:  

Mesh:

Substances:

Year:  2000        PMID: 11045840     DOI: 10.1097/00005176-200010000-00016

Source DB:  PubMed          Journal:  J Pediatr Gastroenterol Nutr        ISSN: 0277-2116            Impact factor:   2.839


  9 in total

1.  Effect of Oral Lipid Matrix Supplement on Fat Absorption in Cystic Fibrosis: A Randomized Placebo-Controlled Trial.

Authors:  Virginia A Stallings; Joan I Schall; Asim Maqbool; Maria R Mascarenhas; Belal N Alshaikh; Kelly A Dougherty; Kevin Hommel; Jamie Ryan; Okan U Elci; Walter A Shaw
Journal:  J Pediatr Gastroenterol Nutr       Date:  2016-12       Impact factor: 2.839

Review 2.  Newborn screening for cystic fibrosis.

Authors:  Jack K Sharp; Michael J Rock
Journal:  Clin Rev Allergy Immunol       Date:  2008-12       Impact factor: 8.667

3.  Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis.

Authors:  Drucy Borowitz; Karen A Robinson; Margaret Rosenfeld; Stephanie D Davis; Kathryn A Sabadosa; Stephanie L Spear; Suzanne H Michel; Richard B Parad; Terry B White; Philip M Farrell; Bruce C Marshall; Frank J Accurso
Journal:  J Pediatr       Date:  2009-12       Impact factor: 4.406

4.  Enteral tube feeding for cystic fibrosis.

Authors:  Deirdre Shimmin; Jacqueline Lowdon; Tracey Remmington
Journal:  Cochrane Database Syst Rev       Date:  2019-07-22

5.  Mechanisms of lipid malabsorption in Cystic Fibrosis: the impact of essential fatty acids deficiency.

Authors:  N Peretti; V Marcil; E Drouin; E Levy
Journal:  Nutr Metab (Lond)       Date:  2005-05-03       Impact factor: 4.169

6.  Determinants of Serum Glycerophospholipid Fatty Acids in Cystic Fibrosis.

Authors:  Sławomira Drzymała-Czyż; Patrycja Krzyżanowska; Berthold Koletzko; Jan Nowak; Anna Miśkiewicz-Chotnicka; Jerzy A Moczko; Aleksandra Lisowska; Jarosław Walkowiak
Journal:  Int J Mol Sci       Date:  2017-01-18       Impact factor: 5.923

7.  Brazilian Guidelines for Nutrition in Cystic Fibrosis.

Authors:  Lenycia de Cassya Lopes Neri; Miriam Isabel Souza Dos Santos Simon; Valéria Laguna Salomão Ambrósio; Eliana Barbosa; Monique Ferreira Garcia; Juliana Ferreira Mauri; Renata Rodrigues Guirau; Mirella Aparecida Neves; Carolina de Azevedo Pedrosa Cunha; Marcelo Coelho Nogueira; Anna Carolina Di Creddo Alves; Jocemara Gurmini; Maria de Fatima Servidoni; Matias Epifanio; Rodrigo Athanazio
Journal:  Einstein (Sao Paulo)       Date:  2022-04-01

8.  Energy-dense, low-volume paediatric oral nutritional supplements improve total nutrient intake and increase growth in paediatric patients requiring nutritional support: results of a randomised controlled pilot trial.

Authors:  Gary P Hubbard; Catherine Fry; Katy Sorensen; Catherine Casewell; Lydia Collins; Annaruby Cunjamalay; Michelle Simpson; Amanda Wall; Elmarie Van Wyk; Matthew Ward; Sophie Hallowes; Hannah Duggan; Jennifer Robison; Helen Gane; Lucy Pope; Jennifer Clark; Rebecca J Stratton
Journal:  Eur J Pediatr       Date:  2020-03-13       Impact factor: 3.183

Review 9.  Is the ENaC Dysregulation in CF an Effect of Protein-Lipid Interaction in the Membranes?

Authors:  Birgitta Strandvik
Journal:  Int J Mol Sci       Date:  2021-03-08       Impact factor: 5.923

  9 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.