What Is Vitiligo? V itiligo is a pigmentation disorder of the skin characterized by permanent loss of melanocytes in defined areas and, in some patients, antibodies to melanin. Vitiligo has significant psychological impact if occurring before…
Sucrose intolerance is the inability to digest sucrose, a widely available sugar, while sucrosemia is the abnormal presence of sucrose in the bloodstream.
Q: Why cannot the body digest sucrose?
A: Sucrose, such as cane or beet sugar, is a double molecule sugar which must first be digested before being absorbed from the gut into the bloodstream. That is, sucrose must be split into its component single molecules of fructose and glucose, which are then properly absorbed.
The inability to properly digest sucrose results directly from low production and activity of sucrase in the small intestine. Sucrase is the specific enzyme that splits or digests sucrose.
Undigested sucrose does not remain idle. Its presence acts osmotically to draw water from the body into the intestine, causing watery diarrhea.
Meanwhile, microbiota (normal bacteria) in the colon eagerly ferment the abnormally present sucrose that arrives from the small intestine. Fermentation generates short-chain fatty acids and hydrogen gas, which results in bloating pain.1
In sucrosemia, sucrose molecules abnormaly pass through an unhealthy small intestinal lining and enter the bloodstream where there presence is abnormal. Sucrose in the blood is filtered out by the kidneys and excreted in urine.
Positive response to a breath hydrogen test (BHT), involving 1 – 3 hours of time post ingestion of sucrose test dose, signifies malabsorption in the small intestine and fermentation in the colon. If BHT is positive before 60 minutes, the result implies bacteria is abnormally present in the small intestine, causing fermentation there. Endoscopy is used to measure sucrase activity in tissue samples.
What Is Sucrose Intolerance And Sucrosemia In Celiac Disease and/or Gluten Sensitivity?
Mucosa in refractory celiac disease immunostained sequentially for CD3 (alkaline phosphatase-blue) and CD8 (peroxidase-brown). Most intraepithelial lymphocytes are CD3+, CD8-. Courtesy pubcan.org
What Is Refractory Celiac Disease?
R efractory celiac disease, formerly called refractory sprue, is a severe complication characterized by persistence of symptoms and intestinal inflammation despite gluten free diet after 12 months.1
Refractory celiac disease appears in two forms, ulcerative jejunitis (RCD I) and cryptic intestinal T-cell lymphoma (RCD II).
Patients with RCD I seem to profit from immunosuppressive treatment, but positive response to corticosteroid treatment does not exclude underlying enteropathy–associated T-cell lymphoma (EATL).
Patients with RCD II have a high percentage of abberant T-cells and is usually resistant to medical therapies. The presence of an aberrant clonal intraepithelial T-cell population has led to the designation of refractory celiac disease with this population as a cryptic intestinal T-cell lymphoma, characterized by frequent dissemination to the blood and the entire gastrointestinal lining.2
Refractory sprue may occur after an initial response to gluten free diet or without any evidence of preexisting celiac disease. All other causes of malabsorption must be excluded, such as collagenous colitis.
In a subgroup of patients with enteropathy-associated T-cell lymphoma (EATL) there is progressive deterioration of a refractory form of celiac disease. The prognosis is poor, although some patients respond to corticosteroids and immunosuppressive agents.3
A nationwide Finnish study showed that patients of male gender, older age, severe symptoms or seronegativity (negative antibody result) at the diagnosis of celiac disease are at risk of future refractory coeliac disease and should be followed up carefully.4
Chorea has been described as a paraneoplastic phenomenon in patients with non-Hodgkin’s lymphoma and has been described as associated with lymphoma arising from a background of refractory celiac disease. The finding of chorea in association with celiac disease should prompt a search for possible underlying intestinal T-cell lymphoma.5
How Prevalent Is Refractory Celiac Disease?
Sources:
Murray JA, The widening spectrum of celiac disease. American Journal of Clinical Nutrition. Mar 1999;69 (3):354-365. [↩]
Culliford AN, Green PH. Refractory sprue. Current Gastroenterology Reports. Oct 2003;5(5):373-8. [↩]
Culliford AN, Green PH. Refractory sprue. Current Gastroenterology Reports. Oct 2003;5(5):373-8. [↩]
Ilus T, Kaukinen K, Virta LJ, Huhtala H, Mäki M, Kurppa K, Heikkinen M, Heikura M, Hirsi E, Jantunen K, Moilanen V, Nielsen C, Puhto M, Pölkki H, Vihriälä I, Collin P. Refractory coeliac disease in a country with a high prevalence of clinically-diagnosed coeliac disease. Aliment Pharmacol Ther. 2014 Feb;39(4):418-25. doi: 10.1111/apt.12606. [↩]
Kitiyakara T, Jackson M, Gorard DA. Refractory coeliac disease, small-bowel lymphoma and chorea. J R Soc Med. 2002 Mar;95(3):133-4. [↩]
I mmune thrombocytopenia purpura (ITP), formerly called idiopathic, is an immune mediated bleeding disorder characterized by destruction of circulating platelets (thrombocytes) by autoantibodies in the presence of normal bone marrow and without other conditions that cause bleeding problems including many medications.
Q: How do autoantibodies destroy platelets?
A: Autoantibodies bind to normal platelet surface antigens (targets) which forms a complex that is cleared from blood by the reticuloendothelial system. Consequently, this condition results in an abnormal decrease in the number of platelets circulating in blood plasma.
Platelets are cells that are needed to stop bleeding. A normal platelet count is in the range of 150,000 to 450,000. With ITP, the platelet count is less than 100,000. By the time significant bleeding occurs, the patient may have a platelet count of less than 10,000. The lower the platelet count, the greater the risk of bleeding.
Because of inability of blood to properly clot due to lack of sufficient platelets, a distinctive purplish rash develops under the skin from blood leakage and an appearance of many small red spots of varying size can cover the skin. Depending on the number of platelets, there may be minimal to extensive bleeding in various parts of the body. Bleeding in the brain is the worst complication, causing death in approximately 1% of affected children and 5% of affected adults.
Immune thrombocytopenic purpura is common in childhood, on average 50 cases per 1,000,000 per year with same incidence in boys as girls. It may follow a viral infection or immunization and is caused by an inappropriate response of the immune system to cross react against the body’s platelets.1
The incidence of immune thrombocytopenic purpura in adults is approximately 66 cases per 1,000,000 per year. Older persons and persons with a previous history of hemorrhage are at increased risk of severe bleeding in adult ITP.
Spontaneous remission occurs in most children (80%). Remission in adults usually requires treatment.
What Is Immune Thrombocytopenic Purpura In Celiac Disease and/or Gluten Sensitivity?
Sources:
Dogan M, Sal E, Akbayram S, Peker E, Cesur Y, Oner AF. Concurrent celiac disease, idiopathic thrombocytopenic purpura and autoimmune thyroiditis. Clin Appl Thromb Hemost. 2011 Nov-Dec;17(6):E13-6. doi: 10.1177/1076029610378502. Epub 2010 Aug 3. [↩]
A fiery red, smooth, burning tongue is an alteration in tongue tissue that is characteristic of advanced niacin deficiency.1
Q: How does niacin deficiency cause the tongue to be red and sore?
A: Niacin is an essential B vitamin that is required for a healthy tongue and by all body cells as well. Deficiency first shows in tissues with rapid cellular turnover, such as mucosal cells of the tongue.
When absorbed from the small intestines, niacin (the form in food) becomes the active form niacinamide. Niacinamide is converted by the body into co-enzymes which are present in all cells. These enzymes function in oxidation-reduction reactions essential for release of energy from carbohydrates, fats, and proteins and are needed as components for more than 200 enzymes involved in metabolism.
In addition to producing energy, niacinamide is essential for healthy skin and the mucosal lining of the digestive tract, normal functioning of the brain and nervous system, and production of steroid hormones from adrenal glands and hormones from sex glands.
What Is A Fiery Red, Smooth, Burning Tongue In Celiac Disease and/or Gluten Sensitivity?
Areas of the Pharynx Where Cancer May Occur. Courtesy Wikimedia.
What Is Cancer Of The Pharynx?
C ancer of the pharynx is a malignant growth of stratified squamous cells that line the pharynx, or throat.
Q: What are stratified squamous cells that line the pharynx?
A: Stratified squamous cells are thin, flat epithelial cells consisting of several layers forming the surface mucosa that protects underlying layers of the pharynx. These cells lie very close together and have no blood vessels.
The pharynx is the upper passageway of the throat where swallowing food and fluids from the mouth first pass on the way to the esophagus and breathing air passes from the nose and mouth to and from the lungs.
The pharynx provides for speech production, adequate taste reception, and proper hearing through equalization of air pressure in the eustachian tubes.
The pharynx has three areas and pharyngeal cancer can occur in any of them: the nasopharynx behind the nose, the oropharynx behind the mouth, and the hypopharynx or laryngopharynx, just above the larynx. Tonsils, adenoids, and other lymph tissue lie at the back of the throat.
Unfortunately, cancers of the pharynx tend not to be detected until late in their course when symptoms become evident. This makes the prognosis poor. See symptom below.
What Is Cancer Of The Pharynx In Celiac Disease and/or Gluten Sensitivity?
A bdominal 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?
L actose intolerance is a well known symptom of carbohydrate malabsorption characterized by inability to properly digest lactose, the sugar in milk, due to low lactase digesting activity in the small intestine.
Lactase is an enzyme produced by specialized cells in the tips of villi within the brush border of the small intestinal lining that digests lactose.
Lactose is a disaccharide, or double sugar, made up of a molecule of glucose and a molecule of galactose (simple sugars). Lactose cannot be absorbed as is into the body unless it is broken down by lactase into glucose and galactose.
Q: How does lactose cause diarrhea?
A: If lactose is not broken down, undigested lactose moves into the colon where it acts osmotically to draw water from the walls of the colon into the lumen, which causes diarrhea by the unnatural amount of water. How much water? Can be 2 liters! Meanwhile, the normal population of colonic bacteria ferment the undigested lactose, generating short-chain fatty acids and hydrogen gas, resulting in bloating pain and flatulence.
Testing for intolerance is based on the action of lactose to increase intestinal permeability.1 Positive response to a breath hydrogen test (BHT), involving 1 – 3 hours of time post ingestion of lactose test dose, signifies malabsorption in the small intestine and fermentation in the colon. If BHT is positive before 60 minutes, the result implies bacteria is abnormally present in the small intestine, causing fermentation there.
Endoscopy is used to measure activity of lactase in a tissue sample.
Treatment is avoidance of milk and dairy products that contain lactose. Nevertheless, milk treated with lactase enzyme can be safely consumed. Fermented milk products like yogurt or kefir are safe because the lactose has been digested by organisms. If there is also a problem with milk protein, then no milk is acceptable that comes from the cow or animal that is causing the allergy.
What Is Lactose Intolerance In Celiac Disease and/or Gluten Sensitivity?
Sources:
Farhadi A, Banan A, Fields J, Keshavarzian A. Intestinal barrier: an interface between health and disease. Journal of Gastroenterology and Hepatology. 2003;18:479-91. [↩]
Underarm showing skin darkening, which is a feature of Addison’s Disease
What Is Primary Addison’s Disease?
A ddison’s disease is an autoimmune destruction of the adrenal glands by autoantibodies that target the adrenal cortex, or outer part of these glands, and is characterized by a slow progressive failure of the adrenal glands to adequately produce its steroid hormones.
Symptoms of adrenal fatigue or failure may not develop until the majority of adrenal tissue is destroyed. When untreated, progression leads to coma, called Addisonian crisis, which is a medical emergency.
There are two adrenal glands each located on top of a kidney and enclosed in a connective tissue capsule. Each is a small, triangular shape that is made of two parts: the outer region and the inner region.
The inner region, called the adrenal medulla, produces epinephrine and norepinephrine chemicals that are needed to deal with stress.
The outer region, called the adrenal cortex, produces adrenocortical (steroid) hormones and releases them into the bloodstream in response to pituitary stimulating hormone from the brain.
Q: What is the function of steroid hormones produced by the adrenal glands?
A: Functions of the three steroid hormones produced by the adrenal glands are:
Glucocorticoids restrain inflammation and metabolism of carbohydrates, fats and proteins to maintain a normal glucose blood level. The major glucocorticoid is hydrocortisone.
Mineralocorticoids regulate the retention and excretion of fluids and electrolytes by the kidneys. The most important mineralocorticoid is aldosterone.
Androgen (testosterone) is a male sex hormone.
Secondary adrenal insufficiency may develop from other causes that are not immune related such as chronic infections, tumor, and medications.
What Is Addison’s Disease In Celiac Disease and/or Gluten Sensitivity?