Cystic Fibrosis with Fibrosing Colonopathy in the Absence of Pancreatic Enzymes

1998 ◽  
Vol 1 (1) ◽  
pp. 74-78 ◽  
Author(s):  
Brenda L. Waters
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Enzyme ◽  
Bowel Dysfunction ◽  
Pancreatic Enzymes ◽  
Wall Thickening ◽  
Colonic Wall ◽  
Distended Abdomen ◽  
Luminal Narrowing ◽  
Fibrosing Colonopathy ◽  
Submucosal Fibrosis

Fibrosing colonopathy, characterized by dense submucosal fibrosis in the large bowel, is a disorder associated with bowel dysfunction in patients with cystic fibrosis who receive pancreatic enzyme supplementation. Most commonly, patients present with a distended abdomen and abdominal pain. Radiographs frequently demonstrate colonic wall thickening and luminal narrowing. Here I describe a neonate with cystic fibrosis who presented with both clinical and histological features of fibrosing colonopathy who had not received pancreatic enzymes. This report expands our understanding of the pathogenesis of 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

Severe Antibiotic-Associated Colitis in a Patient With Cystic Fibrosis and Colonic Wall Thickening

2002 ◽  
Vol 34 (2) ◽  
pp. 224-226 ◽  
Author(s):  
S. Schmitt-Grohé ◽  
E. Wiggert ◽  
J. Steffan ◽  
R. Handke ◽  
S. Zielen
Keyword(s):  
Cystic Fibrosis ◽  
Wall Thickening ◽  
Colonic Wall

Colonic Wall Thickening in Cystic Fibrosis Identified by Ultrasound

1996 ◽  
Vol 23 (4) ◽  
pp. 487-491 ◽  
Author(s):  
H. P. Haber ◽  
A. Lang ◽  
K. Drews ◽  
M. Stern
Keyword(s):  
Cystic Fibrosis ◽  
Wall Thickening ◽  
Colonic Wall

L-Arginine and Cystic Fibrosis

PEDIATRICS ◽  
1973 ◽  
Vol 51 (3) ◽  
pp. 586-588
Author(s):  
Ernest K. Cotton ◽  
Clive C. Solomons ◽  
Reuben Dubois ◽  
Michael D. Coburn ◽  
John Kattwinkel ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Older Patients ◽  
Pancreatic Insufficiency ◽  
Pancreatic Enzyme ◽  
Pancreatic Enzymes

Kattwinkel et al.1 state that "arginine is contraindicated in the treatment of cystic fibrosis." We do not accept this statement because their investigation was improperly controlled for studying arginine therapy in cystic fibrosis. Arginine enhances the activity of lipase and trypsin, and since pancreatic enzymes were completely absent in Kattwinkel's patients, it is not surprising to us that they failed to reduce steatorrhea. All they have proved is that their regimen of arginine without pancreatic enzyme supplements in older patients is ineffective in individuals who have complete pancreatic insufficiency.

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

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 Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis

Biomolecules ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 313
Author(s):  
Sławomira Drzymała-Czyż ◽  
Patrycja Krzyżanowska-Jankowska ◽  
Krzysztof Dziedzic ◽  
Aleksandra Lisowska ◽  
Szymon Kurek ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Cholesterol Biosynthesis ◽  
Pancreatic Insufficiency ◽  
Pancreatic Enzyme ◽  
Pancreatic Enzymes ◽  
Gas Chromatography Mass Spectrometry ◽  
Enzyme Replacement ◽  
Lipid Status ◽  
Pancreatic Enzyme Replacement Therapy ◽  
Sterol Profile

Background: Several factors could lead to lipid disturbances observed in cystic fibrosis (CF). This study aimed to assess sterol homeostasis in CF and define potential exogenous and endogenous determinants of lipid dysregulation. Methods: The study involved 55 CF patients and 45 healthy subjects (HS). Sterol concentrations (μg/dL) were measured by gas chromatography/mass spectrometry. CF was characterised by lung function, pancreatic status, liver disease and diabetes coexistence, Pseudomonas aeruginosa colonisation and BMI. CFTR genotypes were classified as severe or other. Results: Campesterol and β-sitosterol concentrations were lower (p = 0.0028 and p < 0.0001, respectively) and lathosterol levels (reflecting endogenous cholesterol biosynthesis) were higher (p = 0.0016) in CF patients than in HS. Campesterol and β-sitosterol concentrations were lower in patients with a severe CFTR genotype, pancreatic insufficiency and lower pancreatic enzyme dose (lipase units/gram of fat). In multiple regression analyses, β-sitosterol and campesterol concentrations were predicted by genotype and pancreatic insufficiency, whereas cholesterol and its fractions were predicted by phytosterol concentrations, age, dose of pancreatic enzymes, nutritional status and genotype. Conclusions: Independent determinants of lipid status suggest that malabsorption and pancreatic enzyme supplementation play a significant role in sterol abnormalities. The measurement of campesterol and β-sitosterol concentrations in CF patients may serve for the assessment of the effectiveness of pancreatic enzyme replacement therapy and/or compliance, but further research is required.

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