Cystic fibrosis (abdominal manifestations)
Updates to Article Attributes
Abdominal manifestations in cystic fibrosis are common, varied and nearly all organ systems can be affected.
Only 39% of patients with cystic fibrosis (CF) have pulmonary symptoms as their sole complaint 1. 7% of CF patients do not present until adulthood.
For a general discussion of cystic fibrosis, and a discussion of its other manifestations, please refer to:
- cystic fibrosis (parent article)
Hepatobiliary manifestations
Liver parenchymal disease
- 40% of CF patients will develop liver disease
- 1-8% progress to portal hypertension
- up to 40% progress to focal cirrhosis and up to 12% progress to multilobular cirrhosis 5, but development of HCC is unusual
- pathophysiology is multifactorial
- fatty infiltration seen in 30% of CF patients at biopsy, 30
- 50-50% at imaging and 60% at autopsy 2
Biliary tree
- gallstones: 12-24% of CF patients
- sclerosing cholangitis
- intrahepatic ductal strictures seen in 100% of patients with CF and liver disease and may correspond histologically to focal biliary fibrosis seen in 78% of CF patients >24 years old
- microgallbladder: seen in up to 30% of CF patients at autopsy 3
Pancreatic manifestations
Exocrine gland insuffciency affects 85 - 90-90% of all CF patients and results from inspissated secretions leading to proximal duct obstruction with subsequent acinar disruption and replacement by fibrous tissue and fat.
Endocrine dysfunction occurs in 30 - 50-50% of CF patients
- fatty replacement: most common manifestation of the pancreas, which can progress to complete pancreatic lipomatosis
- acute pancreatitis: occurs in patients with residual pancreatic exocrine function
- pancreatic calcifications occur in 7% of patients
- pancreatic cysts and cystosis: typically microscopic 3mm diameter
- pancreatic duct strictures
Gastrointestinal manifestations
-
gastro-oesophageal reflux and associated complications such as Barrett oesophagus: thought to be secondary to chronic cough, hyperinflation and
diaphgramaticdiaphragmatic depression - gastric and duodenal ulcerations: likely related to impaired bicarbonate secretion
- distal intestinal obstruction syndrome (DIOS)
-
intussusception
: typically ileocolic and may result from adherent fecal residue or enlarged lymphoid follicles4. A4; a chronically distended appendix may be the lead point - despite the common distention of the appendix by inspissated secretions, the reported prevalence of acute appendicitis in CF patients is lower than that in the general population
- colon: often abnormal in patients with CF, with proximal colonic wall thickening, pericolonic fat proliferation and mesenteric fat infiltration
- pneumatosis intestinalis: confined to the colon and typically coincides with the development of obstructive lung disease
- rectal mucosal prolapse: typically in young children in whom CF diagnosis not yet made or young adults that are non compliant with treatment
- gastrointestinal tract malignancies: of which colorectal carcinoma is most common
Renal manifestations
-<p><strong>Abdominal manifestations in cystic fibrosis</strong> are common, varied and nearly all organ systems can be affected.</p><p>Only 39% of patients with <a href="/articles/cystic-fibrosis">cystic fibrosis</a> (CF) have pulmonary symptoms as their sole complaint <sup>1</sup>. 7% of CF patients do not present until adulthood.</p><p>For a general discussion of cystic fibrosis, and a discussion of its other manifestations, please refer to :</p><ul><li>- +<p><strong>Abdominal manifestations in cystic fibrosis</strong> are common, varied and nearly all organ systems can be affected.</p><p>Only 39% of patients with <a href="/articles/cystic-fibrosis">cystic fibrosis</a> (CF) have pulmonary symptoms as their sole complaint <sup>1</sup>. 7% of CF patients do not present until adulthood.</p><p>For a general discussion of cystic fibrosis, and a discussion of its other manifestations, please refer to:</p><ul><li>
-<li>up to 40 % progress to focal <a href="/articles/cirrhosis">cirrhosis </a>and up to 12 % progress to multilobular cirrhosis <sup>5</sup>, but development of HCC is unusual</li>- +<li>up to 40% progress to focal <a href="/articles/cirrhosis">cirrhosis </a>and up to 12% progress to multilobular cirrhosis <sup>5</sup>, but development of HCC is unusual</li>
-<li>fatty infiltration seen in 30% of CF patients at biopsy, 30 - 50% at imaging and 60% at autopsy <sup>2</sup>- +<li>fatty infiltration seen in 30% of CF patients at biopsy, 30-50% at imaging and 60% at autopsy <sup>2</sup>
-<li>microgallbladder : seen in up to 30% of CF patients at autopsy <sup>3</sup>- +<li>microgallbladder: seen in up to 30% of CF patients at autopsy <sup>3</sup>
-</ul><h4>Pancreatic manifestations</h4><p>Exocrine gland insuffciency affects 85 - 90% of all CF patients and results from inspissated secretions leading to proximal duct obstruction with subsequent acinar disruption and replacement by fibrous tissue and fat.</p><p>Endocrine dysfunction occurs in 30 - 50% of CF patients</p><ul>- +</ul><h4>Pancreatic manifestations</h4><p>Exocrine gland insuffciency affects 85-90% of all CF patients and results from inspissated secretions leading to proximal duct obstruction with subsequent acinar disruption and replacement by fibrous tissue and fat.</p><p>Endocrine dysfunction occurs in 30-50% of CF patients</p><ul>
-<a href="/articles/pancreatic-lipomatosis">fatty replacement</a> : most common manifestation of the pancreas, which can progress to complete <a href="/articles/pancreatic-lipomatosis">pancreatic lipomatosis</a>- +<a href="/articles/pancreatic-lipomatosis">fatty replacement</a>: most common manifestation of the pancreas, which can progress to complete <a href="/articles/pancreatic-lipomatosis">pancreatic lipomatosis</a>
-<a href="/articles/acute-pancreatitis">acute pancreatitis</a> : occurs in patients with residual pancreatic exocrine function</li>- +<a href="/articles/acute-pancreatitis">acute pancreatitis</a>: occurs in patients with residual pancreatic exocrine function</li>
-<li>pancreatic cysts and cystosis : typically microscopic 3mm diameter</li>- +<li>pancreatic cysts and cystosis: typically microscopic 3mm diameter</li>
-<a href="/articles/gastro-oesophageal-reflux-disease">gastro-oesophageal reflux</a> and associated complications such as <a href="/articles/barretts-oesophagus">Barrett oesophagus</a> : thought to be secondary to chronic cough, hyperinflation and diaphgramatic depression</li>-<li>gastric and duodenal ulcerations : likely related to impaired bicarbonate secretion</li>- +<a href="/articles/gastro-oesophageal-reflux-disease">gastro-oesophageal reflux</a> and associated complications such as <a href="/articles/barretts-oesophagus">Barrett oesophagus</a>: thought to be secondary to chronic cough, hyperinflation and diaphragmatic depression</li>
- +<li>gastric and duodenal ulcerations: likely related to impaired bicarbonate secretion</li>
-<a href="/articles/intussusception">intussusception</a> <sup>4</sup> : typically ileocolic and may result from adherent fecal residue or enlarged lymphoid follicles. A chronically distended appendix may be the lead point</li>- +<a href="/articles/intussusception">intussusception</a>: typically ileocolic and may result from adherent fecal residue or enlarged lymphoid follicles <sup>4</sup>; a chronically distended appendix may be the lead point</li>
-<li>colon : often abnormal in patients with CF, with proximal colonic wall thickening, pericolonic fat proliferation and mesenteric fat infiltration</li>- +<li>colon: often abnormal in patients with CF, with proximal colonic wall thickening, pericolonic fat proliferation and mesenteric fat infiltration</li>
-<a href="/articles/pneumatosis-intestinalis">pneumatosis intestinalis</a> : confined to the colon and typically coincides with the development of obstructive lung disease</li>-<li>rectal mucosal prolapse : typically in young children in whom CF diagnosis not yet made or young adults that are non compliant with treatment</li>-<li>gastrointestinal tract malignancies : of which <a href="/articles/colorectal-carcinoma">colorectal carcinoma</a> is most common</li>- +<a href="/articles/pneumatosis-intestinalis">pneumatosis intestinalis</a>: confined to the colon and typically coincides with the development of obstructive lung disease</li>
- +<li>rectal mucosal prolapse: typically in young children in whom CF diagnosis not yet made or young adults that are non compliant with treatment</li>
- +<li>gastrointestinal tract malignancies: of which <a href="/articles/colorectal-carcinoma">colorectal carcinoma</a> is most common</li>