Fibrosing Colonopathy Revealing Cystic Fibrosis In A Neonate Before Any Pancreatic Enzyme Supplementation

2002 ◽  
Vol 35 (3) ◽  
pp. 356-359 ◽  
Author(s):  
Daniela Elena Serban ◽  
Petre Florescu ◽  
Nicolae Miu
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Enzyme ◽  
Enzyme Supplementation ◽  
Fibrosing Colonopathy

Pancreatic Enzyme Supplementation in Cystic Fibrosis Patients Before and After Fibrosing Colonopathy

1998 ◽  
Vol 26 (1) ◽  
pp. 80-84 ◽  
Author(s):  
John C. Stevens ◽  
Karen M. Maguiness ◽  
Judy Hollingsworth ◽  
Douglas K. Heilman ◽  
Sonny K. F. Chong
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Enzyme ◽  
Before And After ◽  
Enzyme Supplementation ◽  
Fibrosing Colonopathy

scholarly journals Ulceration of the Small and Large Bowel Mucosain Resection Specimens of Cystic Fibrosis Patients with Fibrosing Colonopathy

1998 ◽  
Vol 17 (1) ◽  
pp. 77-99 ◽  
Author(s):  
C. L. Lannon ◽  
S. A. Hinchliffe ◽  
J. D. Pope ◽  
L. M. Ball ◽  
D. Van Velzen
Keyword(s):  
Cystic Fibrosis ◽  
Fibrous Tissue ◽  
Pancreatic Enzyme ◽  
Three Dimensional ◽  
High Strength ◽  
Ascending Colon ◽  
Enzyme Preparations ◽  
Toxic Damage ◽  
Patients At Risk ◽  
Fibrosing Colonopathy

Fibrosing colonopathy (FC), observed in cystic fibrosis patients taking high-strength pancreatic enzyme preparations, is characterized by progressive obstruction of the ascending colon, with long-segment fusiform stenosis due to the deposition of submucosal fibrous tissue. The pathogenesis is uncertain, although direct toxic damage to the colonic mucosa by a constituent of such preparations has been proposed as an explanation. Mucosal defects and rectal bleeding have been observed by colonoscopy in cystic fibrosis patients at risk for and with evident FC. In a quantitative, observational study, mucosal defects were studied in six ileo-cecal resection specimens with FC confirmed by three independent pathologists' review. Representative areas (2.5-cm-long segments) were taken of terminal ileum, cecal colon, and ascending colon both at the site of most severe stenosis and at the most distal ascending colon site available; after processing with paraffin, the areas were serially sectioned at 500-μm intervals for the preparation of 5-μm sections for microscopical assessment. Marking in each section the area affected by (repairing) ulceration, and using three-dimensional reconstructions of the bowel lining, individual mucosal lesions were reconstructed. Using the reconstructed bowels, point scoring analysis of the area fraction affected by ulceration as well as the area of individual lesions was carried out. Lesions of variable age were found in the terminal

Adherence to Pancreatic Enzyme Supplementation: In Adolescents with Cystic Fibrosis

2012 ◽  
Vol 73 (4) ◽  
pp. 196-199 ◽  
Author(s):  
Colleen Faulkner ◽  
L. Janette Taper ◽  
Marjorie Scott
Keyword(s):  
Cystic Fibrosis ◽  
Newfoundland And Labrador ◽  
Health Centre ◽  
Pancreatic Enzyme ◽  
Self Report ◽  
Atlantic Canada ◽  
Rehabilitation Centre ◽  
Enzyme Supplementation ◽  
Positive Effect

Purpose: Levels of adherence to pancreatic enzyme supplementation were investigated in Atlantic Canada adolescents with cystic fibrosis (CF). Methods: Participants were recruited from CF clinics at the Izaak Walton Killam Health Centre in Halifax, Nova Scotia, and the Janeway Children’s Health & Rehabilitation Centre in St. John’s, Newfoundland and Labrador. Self-report questionnaires were mailed to potential participants (n=51) by clinic staff and completed surveys (n=9) were mailed to the principal investigator. Results: Nine adolescents (mean age 15.2 ± 1.9 years) participated in the study. The adherence survey indicated that the majority perceived themselves to be adherent to taking enzymes with meals (67%), but only 44% perceived themselves to be adherent to taking enzymes with snacks. Recorded amounts of enzymes, taken over three days, indicated that 67% of participants were actually adherent to taking enzymes with meals and 56% with snacks. Including those who correctly predicted non-adherence, 56% and 44% of participants accurately predicted their adherence to taking enzymes with meals and snacks, respectively. Conclusions: Adherence rates in the literature vary because of differences in definition and measurement. In the CF population, adherence has been shown to have a positive effect on quality of life. Results for this small group of patients suggest that Atlantic Canada adolescents with CF are able to estimate correctly their adherence to taking pancreatic enzymes, but definite conclusions cannot be made because of the small number of respondents.

scholarly journals Pancreatic enzyme supplementation in cystic fibrosis.

1993 ◽  
Vol 68 (1) ◽  
pp. 150-150 ◽  
Author(s):  
C M Hill ◽  
C J Rolles ◽  
P Keegan ◽  
R A Chand
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Enzyme ◽  
Enzyme Supplementation

Influence of pancreatic status on circulating plasma sterols in patients with cystic fibrosis

2020 ◽  
Vol 58 (10) ◽  
pp. 1725-1730
Author(s):  
Monica Gelzo ◽  
Paola Iacotucci ◽  
Concetta Sica ◽  
Renato Liguori ◽  
Marika Comegna ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Intestinal Absorption ◽  
Cholesterol Biosynthesis ◽  
Pancreatic Insufficiency ◽  
Pancreatic Enzyme ◽  
Adult Patients ◽  
Control Group ◽  
Preliminary Study ◽  
Sterol Profile ◽  
Enzyme Supplementation

AbstractBackgroundPatients with cystic fibrosis (CF) have a reduced intestinal absorption of cholesterol and in a preliminary study we observed differences in plasma sterol profile between patients with pancreatic sufficiency (PS) and those with pancreatic insufficiency (PI). Therefore, we hypothesized that the sterol analysis may contribute to study the digestion and absorption state of lipids in patients with CF. To this aim we evaluated plasma sterols in a significant number of adult patients with CF in relation to the pancreatic status.MethodsBeside cholesterol, we measured phytosterols and lathosterol as markers of intestinal absorption and hepatic biosynthesis, respectively, by gas-chromatography in plasma of adult CF patients with pancreatic sufficiency (PS-CF, n = 57), insufficiency (PI-CF, n = 97) and healthy subjects (control group, CT, n = 71).ResultsPI-CF patients had cholesterol and phytosterols levels significantly lower than PS-CF and CT (p < 5 × 10−10) suggesting a reduced intestinal absorption of sterols related to PI. Instead, lathosterol was significantly higher in PI-CF patients than PS-CF and CT (p < 0.0003) indicating an enhanced cholesterol biosynthesis. In PI-CF patients, phytosterols positively correlate with vitamin E (p = 0.004). Both the classes of molecules need cholesterol esterase for the intestinal digestion, thus the reduced levels of such lipids in serum from PI-CF patients may depend on a reduced enzyme activity, despite the pancreatic enzyme supplementation in all PI-CF patients.ConclusionsA plasma sterols profile may be useful to evaluate the metabolic status of lipids in adult patients with CF and could help to manage the pancreatic enzyme supplementation therapy.

High-Dose Pancreatic-Enzyme Supplements and Fibrosing Colonopathy in Children with Cystic Fibrosis

1997 ◽  
Vol 336 (18) ◽  
pp. 1283-1289 ◽  
Author(s):  
Stacey C. FitzSimmons ◽  
Greg A. Burkhart ◽  
Drucy Borowitz ◽  
Richard J. Grand ◽  
Thomas Hammerstrom ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Enzyme ◽  
High Dose ◽  
Fibrosing Colonopathy
Sign in / Sign up

Export Citation Format

Share Document