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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. [↩]

Hypophosphatemia (Low Phosphate Blood Level)

Testing Thigh Strength. Courtesy Charlie Goldberg, M.D., UCSD School of Medicine
Testing Thigh Strength. Courtesy Charlie Goldberg, M.D., UCSD School of Medicine

What Is Hypophosphatemia?

Hypophosphatemia means the level of phosphates in the bloodstream is too low to meet metabolic needs of the body for this mineral.

Q: How important is phosphorus in metabolism?

A: Phosphorus is crucial to  life, being present in every cell of the body and constitutes 45% of skeletal bone weight along with 40% calcium needed to support the body as a framework.

A low blood phosphate level is characterized by alterations in blood acid-alkaline balance and serious neuromuscular, hematologic, renal, skeletal, and dental abnormalities.

Symptoms result primarily from decreased production of adenosine triphosphate (ATP), the main energy source in cells, and phosphocreatine, a secondary energy source for muscle contraction.

  • Acute phosphorus deficiency may precipitate rhabdomyolysis which is destruction of muscle.
  • Nervous system dysfunction is observed in severe hypophosphatemia.
  • Chronic phosphorus deficiency causes proximal myopathy (upper arms and thighs).1
  • Severe phosphorus deficiency has widespread and ultimately fatal consequences.

What Is Hypophosphatemia In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Takeda E, Ikeda S, Nakahashi O. Lack of phosphorus intake and nutrition. Clin Calcium. 2012 Oct;22(10):1487-91. [↩]

Cheilosis (Cracks in corners of mouth)

cheilosisWhat Is Cheilosis?

Cheilosis is a painful feature of nutritional deficiencies, especially riboflavin and/or pyridoxine deficiency, and less frequently, from vitamin B12, folic acid, and iron. It is characterized by redness of the lips with cracking and weeping in the corner of the mouth.

Q: What is the consequence of weeping at the corners of the mouth?

A: The open sores that develop at the corners of the mouth invite infection by candida albicans, a yeast organism, and less commonly by bacteria normally present on the skin such as staphylococcus aureus.

These sores make opening the mouth very painful and so restrict eating, talking, socializing, and dental care.

What Is Cheilosis In Celiac Disease and/or Gluten Sensitivity?

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?

Macroamylasemia

Depiction of pancreatic amylase molecule.
Depiction of pancreatic amylase molecule.

What Is Macroamylasemia?

Macroamylasemia is an acquired enzyme disorder that causes elevated levels of the enzyme amylase (hyperamylasaemia) in the bloodstream. It is characterized by altered amylase molecules that have become abnormally bound with plasma proteins in the bloodstream, commonly IgG (immunoglobulin G) and/or IgA (immunoglobulin A).

The resulting molecule is too large to be properly filtered by the kidneys and excreted in the urine as would be normal unbound amylase, causing sustained elevation of amylase levels in the plasma.

With normal kidney function, a hyperamylasemia without an increase in urine amylase suggests the diagnosis of macroamylasemia, and is confirmed by identifying the macromolecular components.1

Amylase is a an enzyme produced by the pancreas and the parotid glands to digest starch in the diet. Comparatively small amounts are also produced by other organs.

Q: How do the pancreas and parotid glands deliver their amylase for digestion of starch?

A: The parotid glands deliver their amylase into the mouth. The action of amylase in the mouth starts the breakdown of starch as it is chewed and moistened with saliva.

After the starchy food is liquified in the stomach, the mass empties into the duodenum. Here pancreatic amylase is delivered through the common bile duct into the duodenum where it turns starch into maltose sugar. The next step to final digestion of starch is carried out by enzymes in the small intestinal lining that split maltose into its component sugar which is glucose.

Glucose is a simple sugar that can be absorbed into the bloodstream for use in the body. Humans cannot live without adequate glucose.

Distinquishing macroamylasemia from hyperamylasemia due to pancreatic disease is necessary to prevent needless treatment and investigation for pancreatitis.2

What Is Macroamylasemia In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Torrent Vernetta A, Segarra Cantón O, Soler Palacín P, Segura Cardona RM, Infante Pina D. Macroamylasaemia in paediatrics. An Pediatr (Barc). 2008 Nov;69(5):439-41. [↩]
  2. Isham CA, Ridgeway NA, Hedrick R, Cate JC 4th. Screening for macroamylase in a community hospital. Clin Chem. 1984 May;30(5):741-2. [↩]

Bleeding / Swollen Gums

Vitamin C Deficiency: Clean Teeth with Swollen Gums.
Vitamin C Deficiency: Swollen Gums.

What Are Bleeding / Swollen Gums?

Bleeding and/or swollen gums is  an inflammatory disorder characterized by changes in gum integrity that may result in tooth loss.

Sponginess of the gum (swelling) indicates inflammation, called gingivitis. 

Q: What causes gingivitis?

A: Gingivitis that is caused by inadequate dental hygiene shows as pockets of swollen gum between or along the base of teeth where sticky plaque was allowed to build up. Plaque holds bacteria that inflames the gums where it occurs.

Gingivitis that is caused by vitamin C deficiency, pregnancy, certain drugs such as the anti-epileptic drug phenytoin, and leukemia affects the whole gum irrespective of mouth care.

What Are Bleeding / Swollen Gums 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… 

Macrolipasemia

Lipase Enzyme. Courtesy johnsonmatthey.
Lipase Enzyme. Courtesy Johnson Matthey Catalysts.

What Is Macrolipasemia?

Macrolipasemia is a rare enzyme disorder characterized by altered molecules of lipase, a pancreatic enzyme needed to digest fats, that are abnormally bound with serum antibody proteins. These antibodies are commonly immunoglobulin G (IgG) and/or less likely immunoglobulin A (IgA).

Q: What happens when lipase is bound to immunoglobulins (IgA and/or IgG)?

A: The resulting molecule is too large to be filtered by the kidneys and excreted in the urine, consequently these abnormal molecules build up in the plasma causing sustained elevation of lipase levels called macrolipasemia.

Macrolipasemia occurs with or without macroamylasemia, which is the binding of immunoglobulin A and/or G to amylase, a pancreatic enzyme needed to digest starches.1

What Is Macrolipasemia In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. La Villa G, Pantaleo P, Tarquini R, Cirami L, Perfetto F, Mancuso F, Laffi G. Multiple immune disorders in unrecognized celiac disease: a case report. World J Gastroenterol. 2003;9(6):1377-1380, Available at: http://www.wjgnet.com/1007-9327/9/1377.asp. Accessed Jan 3, 2005. [↩]

Oral Mucosal Lesions, Chronic (Mouth Soreness)

Canker Sore Inside Mouth. Notice The White Spot on This Person's Nail Showing Zinc Deficiency.
Inflammation/Sore Inside Mouth. Notice White Spot on Fingernail Nail Showing Zinc Deficiency.

What Are Chronic Oral Mucosal Lesions?

Chronic lesions of the oral mucosa are disorders of the mouth that are characterized by soreness and sores of the soft mucosal lining of the mouth.

What Are Chronic Oral Mucosal Lesions In Celiac Disease and/or Gluten Sensitivity?

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…