Intestinal Inflammation in Cystic Fibrosis

Overview

Journal Arch Dis Child

Specialty Pediatrics

Date 2000 May 9

PMID 10799435

Citations 47

Authors

R L Smyth

N M Croft

U OHea

T G Marshall

A Ferguson

Affiliations

Soon will be listed here.

Abstract

Background: There is controversy about whether the inflammatory response observed in the cystic fibrosis (CF) lung occurs secondary to bacterial infection or is caused by a dysregulation of the inflammatory response associated with the basic cellular defect of CF.

Aims: To study the inflammatory response in the gastrointestinal tract of children with CF; and to investigate whether there is increased inflammation in the gastrointestinal tract of CF children with fibrosing colonopathy.

Methods: Whole gut lavage was performed on 21 pancreatic insufficient children with CF, who were clinically well, five children with CF and fibrosing colonopathy, and 12 controls. Intestinal outputs of plasma derived proteins (albumin, alpha(1) antitrypsin, IgG), secretory immunoglobulins (IgA and IgM), cellular constituents (eosinophil cationic protein and neutrophil elastase), and cytokines (interleukin 8 and interleukin 1beta) were measured.

Results: Compared to controls, the 21 CF patients, with no intestinal complications, had increased intestinal outputs of albumin, IgG, IgM, eosinophil cationic protein, neutrophil elastase, interleukin 1beta, and interleukin 8. Similar values were obtained for the CF patients with fibrosing colonopathy.

Conclusions: These data suggest that there is immune activation in the gastrointestinal mucosa of children with cystic fibrosis, which may result from the basic cellular defect. Fibrosing colonopathy does not appear to be associated with increased inflammation.

Citing Articles

Fatty acid abnormalities in cystic fibrosis-the missing link for a cure?.

Drzymala-Czyz S, Walkowiak J, Colombo C, Alicandro G, Storrosten O, Kolsgaard M iScience. 2024; 27(11):111153.

PMID: 39620135 PMC: 11607544. DOI: 10.1016/j.isci.2024.111153.

Normalization of Cystic Fibrosis Immune System Reverses Intestinal Neutrophilic Inflammation and Significantly Improves the Survival of Cystic Fibrosis Mice.

Scull C, Hu Y, Jennings S, Wang G Cell Mol Gastroenterol Hepatol. 2024; 19(2):101424.

PMID: 39510500 PMC: 11720009. DOI: 10.1016/j.jcmgh.2024.101424.

Intestinal Inflammation and Regeneration-Interdigitating Processes Controlled by Dietary Lipids in Inflammatory Bowel Disease.

Kwon S, Khan M, Kim S Int J Mol Sci. 2024; 25(2).

PMID: 38279309 PMC: 10816399. DOI: 10.3390/ijms25021311.

Fecal dysbiosis and inflammation in intestinal-specific Cftr knockout mice on regimens preventing intestinal obstruction.

Young S, Woode R, Williams E, Ericsson A, Clarke L Physiol Genomics. 2023; 56(3):247-264.

PMID: 38073491 PMC: 11283905. DOI: 10.1152/physiolgenomics.00077.2023.

Cystic Fibrosis-Related Gut Dysbiosis: A Systematic Review.

Caley L, White H, de Goffau M, Floto R, Parkhill J, Marsland B Dig Dis Sci. 2023; 68(5):1797-1814.

PMID: 36600119 DOI: 10.1007/s10620-022-07812-1.

References

Balough K, McCubbin M, Weinberger M, Smits W, Ahrens R, Fick R . The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis. Pediatr Pulmonol. 1995; 20(2):63-70. DOI: 10.1002/ppul.1950200203. View

Bonfield T, Panuska J, Konstan M, Hilliard K, Hilliard J, Ghnaim H . Inflammatory cytokines in cystic fibrosis lungs. Am J Respir Crit Care Med. 1995; 152(6 Pt 1):2111-8. DOI: 10.1164/ajrccm.152.6.8520783. View

Croft N, Marshall T, Ferguson A . Direct assessment of gastrointestinal inflammation and mucosal immunity in children with cystic fibrosis. Postgrad Med J. 1996; 72 Suppl 2:S32-6. View

Koller D, Gotz M, Wojnarowski C, Eichler I . Relationship between disease severity and inflammatory markers in cystic fibrosis. Arch Dis Child. 1996; 75(6):498-501. PMC: 1511810. DOI: 10.1136/adc.75.6.498. View

Noah T, Black H, Cheng P, Wood R, Leigh M . Nasal and bronchoalveolar lavage fluid cytokines in early cystic fibrosis. J Infect Dis. 1997; 175(3):638-47. DOI: 10.1093/infdis/175.3.638. View

Zahm J, Gaillard D, Dupuit F, Hinnrasky J, Porteous D, Dorin J . Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice. Am J Physiol. 1997; 272(3 Pt 1):C853-9. DOI: 10.1152/ajpcell.1997.272.3.C853. View

Maiuri L, Raia V, De Marco G, COLETTA S, De Ritis G, Londei M . DNA fragmentation is a feature of cystic fibrosis epithelial cells: a disease with inappropriate apoptosis?. FEBS Lett. 1997; 408(2):225-31. DOI: 10.1016/s0014-5793(97)00347-5. View

Miele L, Xing M, Frizzell R, Mukherjee A . Cystic fibrosis gene mutation (deltaF508) is associated with an intrinsic abnormality in Ca2+-induced arachidonic acid release by epithelial cells. DNA Cell Biol. 1997; 16(6):749-59. DOI: 10.1089/dna.1997.16.749. View

Troncone R, Caputo N, Campanozzi A, Cucciardi M, Esposito V, Russo R . Gut lavage IgG and interleukin 1 receptor antagonist:interleukin 1 beta ratio as markers of intestinal inflammation in children with inflammatory bowel disease. Gut. 1997; 41(1):60-5. PMC: 1027229. DOI: 10.1136/gut.41.1.60. View

10.

Troncone R, Caputo N, Esposito V, Campanozzi A, Campanozzi F, Auricchio R . Increased concentrations of eosinophilic cationic protein in whole-gut lavage fluid from children with inflammatory bowel disease. J Pediatr Gastroenterol Nutr. 1999; 28(2):164-8. DOI: 10.1097/00005176-199902000-00014. View

11.

Anderson C, LANGFORD R . Bacterial content of small intestine of children in health, in coeliac disease, and in fibrocystic disease of pancreas. Br Med J. 1958; 1(5074):803-6. PMC: 2027979. DOI: 10.1136/bmj.1.5074.803. View

12.

FREYE H, Kurtz S, Spock A, CAPP M . LIGHT AND ELECTRON MICROSCOPIC EXAMINATION OF THE SMALL BOWEL OF CHILDREN WITH CYSTIC FIBROSIS. J Pediatr. 1964; 64:575-9. DOI: 10.1016/s0022-3476(64)80351-6. View

13.

Falchuk Z, Taussig L . IgA synthesis by jejunal biopsies from patients with cystic fibrosis and hereditary pancreatitis. Pediatrics. 1973; 51(1):49-54. View

14.

Morin C, Roy C, LASALLE R, Bonin A . Small bowel mucosal dysfunction in patients with cystic fibrosis. J Pediatr. 1976; 88(2):213-6. DOI: 10.1016/s0022-3476(76)80984-5. View

15.

ANTONOWICZ I, Lebenthal E, SHWACHMAN H . Disaccharidase activities in small intestinal mucosa in patients with cystic fibrosis. J Pediatr. 1978; 92(2):214-9. DOI: 10.1016/s0022-3476(78)80007-9. View

16.

Conley M, Delacroix D . Intravascular and mucosal immunoglobulin A: two separate but related systems of immune defense?. Ann Intern Med. 1987; 106(6):892-9. DOI: 10.7326/0003-4819-106-6-892. View

17.

Suter S, Schaad U, Roux-Lombard P, Girardin E, Grau G, Dayer J . Relation between tumor necrosis factor-alpha and granulocyte elastase-alpha 1-proteinase inhibitor complexes in the plasma of patients with cystic fibrosis. Am Rev Respir Dis. 1989; 140(6):1640-4. DOI: 10.1164/ajrccm/140.6.1640. View

18.

Wilmott R, Kassab J, Kilian P, Benjamin W, Douglas S, Wood R . Increased levels of interleukin-1 in bronchoalveolar washings from children with bacterial pulmonary infections. Am Rev Respir Dis. 1990; 142(2):365-8. DOI: 10.1164/ajrccm/142.2.365. View

19.

OMahony S, Barton J, Crichton S, Ferguson A . Appraisal of gut lavage in the study of intestinal humoral immunity. Gut. 1990; 31(12):1341-4. PMC: 1378753. DOI: 10.1136/gut.31.12.1341. View

20.

OMahony S, Arranz E, Barton J, Ferguson A . Dissociation between systemic and mucosal humoral immune responses in coeliac disease. Gut. 1991; 32(1):29-35. PMC: 1379209. DOI: 10.1136/gut.32.1.29. View