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Primary Sclerosing Cholangitis 

A 3D Image From Magnetic Resonace Cholangiography. NIHMS
A 3D Image From Magnetic Resonace Cholangiography showing biliary tree. NIHMS

What Is Primary Sclerosing Cholangitis?

Primary sclerosing cholangitis (PSC) is an uncommon, slowly progressive bile duct disease that results in stagnation or build-up of bile in the liver, called cholestasis.

Primary sclerosing cholangitis is characterized by sclerosis, or scarring inflammation in bile ducts both within the liver (intra-hepatic ducts), and outside the liver (extra-hepatic ducts), causing progressive narrowing and, eventually, obliteration of the bile ducts.

Primary sclerosing cholangitis comes under the umbrella term autoimune liver disease in which the end result is immune-mediated hepatocellular (liver cell) or hepatobiliary (bile duct) injury.1

Q: What happens when scarred bile ducts can no longer transport bile out of the liver?

A: Bile that cannot be removed from the liver by the biliary duct system backs up and damages the liver, causing cirrhosis.

Bile is continually made by the liver from phospholipids salt, cholesterol, and aging blood cells that it removes from circulation to be carried out of the liver. Bile also carries away waste products produced by normal metabolism and toxic substances that are removed by the liver for eventual elimination in stool. As such, bile must continually flow out of the liver to prevent build-up in the liver.

Bile is a greenish brown liquid made by the liver. Bile ducts carry it out of the liver to the gall bladder for storage until needed to aid in the digestion and absorption of fat from the small intestine. Bile emulsifies fat eaten in the diet so that the pancreatic enzyme called lypase can break it down into its fatty acid and glycerol components.

The liver is the largest organ within the body. It lies mostly in the upper part of the abdomen on the right side just under the diaphragm. About 70% of liver tissue is made up of cube shaped cells called hepatocytes that do the main work of the liver. Other cells (epithelial) form structure and are arranged in single layers around blood vessels, sinusoids, and bile ducts. 

Build-up of bile in the liver is the end result of the inflammatory process in primary sclerosing cholangitis, that by swelling and scarring of bile ducts impedes and eventually prevents bile flow out of the liver, leading to liver failure. There is no curative treatment available for primary sclerosing cholangitis, besides liver transplantation.2

The appearance of the intrahepatic and extrahepatic biliary ducts can be assessed by use of cholangiography, and magnetic resonance (MR) imaging is the best way to identify patients.  See image above.3

MR cholangiography offers a noninvasive method of obtaining images of the biliary system without the use of a contrast agent. There is no radiation exposure. Pulse sequences can be chosen to obtain bright bile or black bile cholangiograms. Image processing algorithms can be selected to obtain a three-dimensional representation of biliary anatomy and pathology, and those images can be rotated in any plane so that ductal anatomy and pathology can be seen to best advantage.4

There is no cure for primary sclerosing cholangitis but there are symptom treatments one of which is supplementation for low levels of vitamins A,D,E, and K. Liver transplant is the only effective option.

What Is Primary Sclerosing Cholangitis In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Trivedi PJ, Adams DH. Mucosal immunity in liver autoimmunity: a comprehensive review. J Autoimmun. 2013 Oct;46:97-111. doi: 10.1016/j.jaut.2013.06.013. [↩]
  2. Kummen M, Schrumpf E, Boberg KM. Liver abnormalities in bowel diseases. Best Pract Res Clin Gastroenterol. 2013 Aug;27(4):531-42. doi: 10.1016/j.bpg.2013.06.013. [↩]
  3. Eaton JE, Talwalkar JA, Lazaridis KN, Gores GJ, Lindor KD. Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management. Gastroenterology. 2013 Sep;145(3):521-36. doi: 10.1053/j.gastro.2013.06.052. Epub 2013 Jul 1. [↩]
  4. Meakem TJ 3rd, Schnall MD. Magnetic resonance cholangiography. Gastroenterol Clin North Am. 1995 Jun;24(2):221-38. [↩]

Milk Intolerance – Bovine Beta Casein Enteropathy

Cows Milk enteropathyWhat Is Milk Intolerance (Bovine Beta Casein Enteropathy)?

Bovine beta casein enteropathy is characterized by raised serum IgA antibodies to bovine beta casein found by blood testing and damage to mucosa of the jejunum found by biopsy.

The jejunum is the second section of the small intestine, between the duodenum and the ileum.

Mucosa damage is similar to damage caused by gluten.

Q: What is bovine beta casein?

A: Bovine beta casein is a protein found in cow’s milk.

An immune reaction to protein in milk is totally different from lactose intolerance. Lactose intolerance develops from deficiency of an intestinal enzyme called lactase which is required to digest lactose, the sugar in milk.

What Is Milk Intolerance In Celiac Disease and/or Gluten Sensitivity?

Dysphagia (Difficulty Swallowing)

What Is Dysphagia? Dysphagia is a functional upper digestive disorder characterized by difficulty in swallowing or food getting stuck and swallowing that takes longer than 10 seconds to complete. Q: What determines the symptoms? A:… 

Abdominal Distention, Chronic  (Bloating)

chronic abdominal distention celiac disease gluten symptomWhat Is Chronic Abdominal Distention?

Abdominal distention, or bloating, is characterized by enlargement in normal size of abdomen not due to fatty tissue.

Q: Why does the abdomen enlarge?

A: The abdomen enlarges because the bowel is dysfunctional. Loops of bowel usually lack normal peristalsis (rhythmic wave-like muscle movement) trapping gas in stagnant loops of bowel, inflammation swells loops of bowel also trapping gas, and dysbiosis overproduces gas. All these factors distend the abdomen.

What Is Chronic Abdominal Distention In Celiac Disease and/or Gluten Sensitivity?

Ulceration And Stenosis, Postbulbar Duodenal

What Is Postbulbar Duodenal Ulceration/Stenosis?

Postbulbar duodenal ulceration is a chronic inflammatory disorder characterized by thickening of the intestinal lining with excoriation penetrating the muscularis mucosae (muscle layer of the intestine) associated with villous atrophy.

The ulcer is well-defined. Stenosis due to thickening of the area narrows the hollow inside of the duodenum which impairs passage of foodstuffs and may lead to obstruction.

Q: Is postbulbar duodenal ulceration painful?

A: Postbulbar duodenal ulceration may not cause symptoms but usually causes gnawing pain that is relieved by eating food. After eating there develops nausea and bloating.

Complications include obstruction, bleeding, and perforation of the duodenal lining. Ulcers do not usually become malignant.

What Is Postbulbar Duodenal Ulceration/Stenosis In Celiac Disease and/or Gluten Sensitivity?

Cancer, Esophageal Small Cell

esophageal small cell carcinomaWhat Is Esophageal Small Cell Cancer (Carcinoma)?

Esophageal small cell carcinoma is a rare and aggressive malignancy arising in the esophagus with a poor prognosis.

Q: Where does the cancer occur in the esophagus?

A: Esophageal cancer can occur anywhere in the esophagus.

The esophagus is a muscular tube that transports swallowed substances to the stomach. It begins at the cricoid cartilage (Adam’s apple) as a continuation of the pharynx and ends at the lower esophageal sphincter (LES).

The LES is a circular muscle surrounding the junction of the esophagus and stomach. The LES opens to allow swallowed food and liquids to enter the stomach and closes to prevent their travelling back into the esophagus.

Dysphagia (difficulty swallowing) occurs when the inside diameter of the esophagus constricts to less than 14 millimeters. First there is difficulty swallowing solid food then liquids and saliva due to progressive growth of the cancer.

What Is Esophageal Small Cell Carcinoma In Celiac Disease and/or Gluten Sensitivity?

Abdominal Pain, Chronic or Recurrent

What Is Chronic Or Recurrent Abdominal Pain? Abdominal pain is characterized by pain anywhere in the abdominal cavity between the chest and groin. This region is often referred to as the belly. Abdominal pain, or… 

Esophageal Motor Abnormalities – Food Sticks In Esophagus

Beaded Barium Swallow Shows Spasms of Espophagus. Courtesy Quizlet.com
Beaded Barium Swallow Shows Spasms of Espophagus. Courtesy Quizlet.com

What Are Esophageal Motor Abnormalities?

Esophageal motor abnormalities are disorders of the esophagus involving altered muscular motility characterized by impaired esophageal peristalsis, or the progressive wave-like movement that occurs automatically when food or fluids are swallowed.

Q: What is altered motility?

A: Altered motility describes the lack of coordinated muscular movements or weak muscular movement needed to rapidly propel food and fluids through the highly muscular esophagus from the throat to the stomach.

The esophagus is a tube-like organ that begins at the cricoid cartilage (Adam’s apple) as a continuation of the pharynx and ends at the lower esophageal sphincter (LES).

The LES is a very strong, circular muscle surrounding the junction of the esophagus and stomach. The LES opens to allow swallowed food and liquids to enter the stomach and closes to prevent their travelling back into the esophagus.

Failure of the lower esophageal sphincter to remain tightly closed after swallowing allows stomach contents to reenter the esophagus giving symptoms of heartburn.

On the other hand, failure of the LES to relax traps the contents of the esophagus so that it cannot quickly empty into the stomach as it should. Because the esophagus is not designed to hold food/fluids, the abnormal expansion results in pain, anxiety and possibly vomiting and choking.  If the contents are aspirated into the windpipe upon choking, there will be coughing with attempts to clear it.

What Are Esophageal Motor Abnormalities In Celiac Disease and/or Gluten Sensitivity?

Aphthous Ulcers (Canker Sores) 

Aphthous_ulcer[1]What Are Aphthous Ulcers?

Aphthous ulcers, also called canker sores, are a chronic disorder of soft mouth tissue characterized by small, painful purpuric, papular, or erosive lesions that are often surrounded by erythematous (red) margins.1

Q: What soft mouth tissues develop canker sores?

A: Canker sores can erupt on the mucosal surface of the inside lip, sides of mouth, under the tongue and along the side of the tongue. When they are forming, the area swells into a papule, or small bump. Later, the surface erodes and a crater forms with firm pus.

What Are Aphthous Ulcers In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Lahteenoja H, Toivanen A, Viander M, Maki M, Irjala K, Raiha I, Syrjanen S. Oral mucosal changes in coeliac patients on a gluten-free diet. European Journal of Oral Sciences. Oct 1998;106(5):899,8p. [↩]

Carbohydrate Malabsorption

What Is Carbohydrate Malabsorption? Carbohydrate malabsorption is a digestive disorder characterized by the inability to properly digest and absorb carbohydrates within the small intestine to supply needed energy to the body. Q: What carbohydrates should be normally…