FATTY-ACID COMPOSITION OF SERUM-LECITHINS IN CYSTIC-FIBROSIS PATIENTS WITHOUT STEATORRH$OElig;A

The Lancet ◽  
1978 ◽  
Vol 312 (8095) ◽  
pp. 903 ◽  
Author(s):  
C GALABERT
PEDIATRICS ◽  
1989 ◽  
Vol 83 (4) ◽  
pp. 632-632
Author(s):  
JOEL BITMAN ◽  
MARGIT HAMOSH ◽  
D. L. WOOD ◽  
L. M. FREED ◽  
P. HAMOSH

This report from Golembeski and Emery adds to the small quantity of literature in this field. Drs Golembeski and Emery claim that they are presenting information that counters our statement,"The abnormalities in fatty acid composition of the cystic fibrosis milk may contraindicate its use for the nursing infant." However, no data regarding fatty acid composition were presented. In their Table, only total lipids are shown. In our study, we acknowledged that mean total lipids were sufficient to supply the energy needs of the nursing infant.


2008 ◽  
Vol 54 (2) ◽  
pp. 388-395 ◽  
Author(s):  
Thierry Charles Coste ◽  
Gladys Deumer ◽  
Gregory Reychler ◽  
Patrick Lebecque ◽  
Pierre Wallemacq ◽  
...  

Abstract Background: Some but not all studies have reported abnormal polyunsaturated fatty acid composition in cystic fibrosis (CF) patients. We investigated the influence of pancreatic status and sex on the fatty acid profile in plasma and erythrocyte membranes in patients with CF. Methods: After a 1-step transesterification with acetyl chloride on plasma and washed erythrocyte membranes, we quantified fatty acid methyl esters by use of GC-MS in 124 CF patients and 80 age-matched healthy controls. In the CF group, mean (SD) age was 17.5 (11.3) years, and 51.6% were male. Pancreatic insufficiency was diagnosed in 78% of the CF population. Results: A decrease in docosahexaenoic acid concentrations was observed in CF patients independently of pancreatic status. Pancreatic insufficient CF patients displayed lower concentrations of linoleic acid and arachidonic acid and higher concentrations of dihomo-γ-linolenic acid and eicosatrienoic acid (mead acid) in plasma and erythrocyte membranes compared with healthy controls and pancreatic sufficient CF patients. Male CF patients had significantly lower docosahexaenoic acid and higher eicosatrienoic acid in plasma and erythrocyte membranes compared with female CF patients. Conclusions: These results support the concept that multiple abnormalities of polyunsaturated fatty acid composition participate in the CF disease phenotype and that pancreatic status plays a major role in such abnormalities. Moreover, patient sex influences the polyunsaturated fatty acid spectrum in CF, with more marked abnormalities in males.


PEDIATRICS ◽  
1976 ◽  
Vol 57 (4) ◽  
pp. 480-486
Author(s):  
Ian M. Campbell ◽  
Douglas N. Crozier ◽  
Robert B. Caton

Impaired oxygen supply and deteriorating health, in cystic fibrosis patients, correlates with abnormal changes in the fatty acid composition of blood lipids. As the proportion of oleates increases and that of linoleates decreases, erythrocyte membrane interference with the formation of intracellular oxyhemoglobin increases and arterial oxygen pressure decreases. The physical-chemical basis for these changes seems to be that oleic and linoleic acid differ in their ability to undergo reversible oxygenation in response to changes in oxygen pressure. The oxygen complex of linoleic acid dissociates at relatively high pressures, whereas that of oleic dissociates only at low pressures. Accordingly, excessive substitution of oleic for linoleic acid in membrane lipids would be expected to decrease the intracellular oxygen pressure to a level where hemoglobin oxygenation and any other oxygen-requiring processes would be impaired.


2007 ◽  
Vol 51 (6) ◽  
pp. 541-549 ◽  
Author(s):  
Stephanie Van Biervliet ◽  
Griet Vanbillemont ◽  
Jean-Pierre Van Biervliet ◽  
Dimitri Declercq ◽  
Eddy Robberecht ◽  
...  

PEDIATRICS ◽  
1989 ◽  
Vol 83 (4) ◽  
pp. 631-632
Author(s):  
DAVID J. GOLEMBESKI ◽  
MAURICE G. EMERY

In response to the article by Bitman et al concerning the lipid composition in the breast milk of women with cystic fibrosis, we would like to share information about a recent case from our clinic and present information that counters the statement, "abnormalities in the fatty acid composition of cystic fibrosis milk may contraindicate its use for the nursing infant."


The Lancet ◽  
1977 ◽  
Vol 310 (8052) ◽  
pp. 1302-1304 ◽  
Author(s):  
VanS. Hubbard ◽  
G.Dewey Dunn ◽  
PaulA. DI SANT'AGNESE

2018 ◽  
Vol 31 (5) ◽  
pp. 455-466
Author(s):  
Ana Lucia Pereira da CUNHA ◽  
Ana Carolina Carioca da COSTA ◽  
Zilton VASCONCELOS ◽  
Maria das Graças TAVARES DO CARMO ◽  
Celia Regina Moutinho de Miranda CHAVES

ABSTRACT Objective To analyze erythrocyte fatty acid composition and its association with serum cytokine levels in pediatric cystic fibrosis patients. Methods A cross-sectional study was performed at a reference center in Rio de Janeiro, Brazil. We have included all pediatric patients aged 5-19 years with confirmed cystic fibrosis diagnosis. Erythrocyte fatty acid composition and serum cytokine (TNF-α, IL-1β, IL-6 and IL-8) and C-reactive protein levels were measured. The cut-off point to determine essential fatty acids deficiency was the linoleic acid concentration of <21%. Results Twenty-six children (<10 years old) and thirty-one adolescents were studied. Most patients were female and heterozygous for DF508 mutation and suffered from exocrine pancreatic insufficiency. Both children and adolescents had lower linoleic acid concentration (<21%). TNF-α was the only pro-inflammatory marker whose levels were increased; the increase was greater in children. An association between fatty acid composition in erythrocytes and cytokines IL-1β and IL-6 was observed (p<0.05). Conclusion The pediatric cystic fibrosis patients studied presented a deficiency of essential fatty acids, and an association between fatty acid profile in erythrocytes and serum pro-inflammatory cytokines was observed. These findings highlight the importance of this type of assessment that may open new possibilities for studying pathophysiology and treating cystic fibrosis patients, such as the dietary supplementation with n-3 fatty acids (eicosapentaenoic and docosahexaenoic acids). However, further longitudinal studies are needed for better clarification of the imbalance in lipid metabolism and inflammation in cystic fibrosis


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