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Laryngospasm

e3343bf0a689b5f974a852fa2e84a718What Is Laryngospasm?

Laryngospasm is an acute disorder of the larynx that is characterized by life-threatening spasms of the muscles of the larynx located in the throat.

Laryngospasm is a rare feature of hypocalcemia (low blood calcium level) characterized by severe alteration in nerve conduction and muscle contraction.

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

Diarrhea, Acute

Infant with Acute Diarrhea and Swollen Belly - Hallmarks of Celiac Disease.
Malnourished Infant With Acute Diarrhea and Swollen Belly.

What Is Acute Diarrhea?

Acute diarrhea is a small intestinal motility disorder characterized by excessively rapid movement of intestinal contents through the small intestine with excessive loss of fluid and electrolytes that leads rapidly to a life threatening hypokalemia (low potassium blood level) and acidosis.

Q: What is hypokalemia?

A: Hypokalemia is characterized by dehydration that may result in vascular collapse, muscular malfunction that may result in paralytic ileus (bowels do not move), paralysis (cannot stand), and respiratory hypoventilation (shallow breathing) or failure, metabolic acidosis resulting from diarrhea, and impaired nerve conduction.1

While diarrhea may be a common symptom of small bowel mucosal disease, the consequent malabsorption can lead to substantial malnutrition and nutrient deficiencies. The small intestine, unlike the colon, has been relatively inaccessible, and systematic evaluation is often necessary to identify and treat small intestinal mucosal diseases that lead to diarrhea. All patients with severe diarrhea or diarrhea associated with features suggestive of malabsorption may have a disease of the small intestinal mucosa that requires careful evaluation and targeted management.2

What Is Acute Diarrhea In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Kathleen Mahan and Sylvia Escott-Stump, ed. Krause’s Food, Nutrition & Diet Therapy, 10th Edition. Philadelphia, PA. USA: W.B. Saunders Company, 2000. []
  2. Murray JA1, Rubio-Tapia A. Diarrhoea due to small bowel diseases. Best Pract Res Clin Gastroenterol. 2012 Oct;26(5):581-600. doi: 10.1016/j.bpg.2012.11.013. []

Hypoprothrombinemia (Low Prothrombin Level)

bruiseWhat Is Hypoprothrombinemia?

Hypoprothrombinemia is a deficiency of prothrombin (clotting factor II) in the blood that is characterized by impaired hemostasis in response to trauma or a laceration.

Q: What is hemostasis and how is it altered by a deficiency of prothrombin?

A: Hemostasis encompasses the tightly regulated processes of blood clotting, platelet activation, and blood vessel repair.1

Prothrombin is a protein clotting factor present in blood that is involved in the first part of hemostasis, which is blood clotting or coagulation. Vitamin K is required for prothrombin production.

When a laceration or wound is sustained, prothrombin is converted to the enzyme thrombin. Thrombin in turn acts on fibrinogen to convert it to fibrin which then forms the framework of a clot to stop bleeding. Deficiency of prothrombin prevents this series of events and bleeding is not properly stopped.

After the clotting process of hemostasis would come the second part, platelet activation. Eventually, coagulation and platelet activation are switched off by blood-borne inhibitors.

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

Sources:
  1. Versteeg HH, Heemskerk JWM, Levi M,  Reitsma PH. New Fundamentals in Hemostasis. Physiological Reviews Published 1 January 2013Vol. 93no. 327-358DOI: 10.1152/physrev.00016.2011 []

Cancer, Post-Cricoid

Anatomy of Upper Airway and Throat. Courtesy Wikipedia.org
Anatomy of Upper Airway and Throat. Courtesy Wikipedia.org

What Is Post-Cricoid Carcinoma?

Post-cricoid carcinoma is a rare malignancy arising in the hypopharynx.

Q: What is the hypopharynx?

A: The hypopharynx is the lower portion of the pharynx that opens into the larynx. It is comprised of the postcricoid region, pyriform sinus, and posterior hypopharyngeal wall.

Tumors rarely appear in the posterior pharyngeal wall or postcricoid region without also involving the pyriform sinus. The lack of anatomic barriers between these sites and a propensity for these tumors to develop in the pyriform sinus and spread outward account for this phenomenon.

Overall prognosis for these tumors is poor. Because of the rich organic lymphatic and vascular networks, aggressive growth and early cervical metastases (compared with cancers at other head and neck sites) characterize these cancers.

A study investigating the clinical course of 21 patients with post-cricoid carcinoma found the 5-year cause-specific survival rate was 52%. All the patients who eventually died did so within 3 years of the treatment. Seven patients had distant metastases, representing a higher frequency as compared with that of patients with recurrence of the primary focus and cervical lymph node metastasis. All of these patients who had been treated by surgery died of the cancer.1

Unfortunately, hypopharyngeal cancers are discovered at a later stage than other head and neck cancers. The delay in diagnosis from symptom onset averages 10 months.2

What Is Post-Cricoid Carcinoma In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Asakage T, Nakao K, Ebihara Y, Fujishiro Y, Watanabe K. A clinical study of post-cricoid carcinoma. Acta Otolaryngol Suppl. 2007 Dec;(559):118-22. doi: 10.1080/03655230701599354. []
  2. http://emedicine.medscape.com/article/848359-overview []

Erosions in the Second Part of the Duodenum

What Are Erosions In The Second Part Of The Duodenum?

Erosions in the second part of the duodenum typically are superficial excoriations (sores) in the mucosal lining that do not penetrate into the muscle layer as does an ulcer.

Of note, most erosions develop in the first part of the duodenum rather than the second part.

Q: Are duodenal erosions serious?

A: Yes duodenal erosions are serious and can be life-threatening for the following reasons:

  • Duodenal erosions may occur together with gastric erosions, therefore they can jointly induce massive bleeding.
  • Duodenal erosions may be associated with duodenal ulcer but hemorrhage originates from the erosion and the ulcer itself does not bleed.
  • Duodenal erosion in itself might induce bleeding. The intensity of bleeding, if there is no hemorrhage from other places, is not so severe, as the one originating from gastric erosions.1

What Are Erosions In The Second Part Of The Duodenum In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Preisich P, Farkas I, Konyár EJ. The role of duodenal erosions in the occurrence of upper gastrointestinal haemorrhage. Scand J Gastroenterol Suppl. 1989;167:36-8. []

Immune Thrombocytopenic Purpura

Photo of Skin Rash in ITP Courtesy Wikipedia.
Photo of Skin Rash in ITP Courtesy Wikipedia.

What Is Immune Thrombocytopenic Purpura?

Immune 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:
  1. 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. []

Cancer Of The Esophagus 

A real photomicrograph of carcinoma of the esophagus. Panorama of 6 photos of a slide at 40x through the microscope. Some areas may appear blurry due to shallow DOF.
A real photomicrograph of carcinoma of the esophagus. Panorama of 6 photos of a slide at 40x through the microscope. Some areas may appear blurry due to shallow DOF.

What Is Cancer Of The Esophagus?

Cancer of the esophagus is a malignancy arising in the stratified squamous cell lining of any part of the esophagus and having a poor prognosis.

This tumor first invades the deeper layers of the esophagus which includes the layer beneath the mucosa (submucosa) and muscle before it may invade structures close to it including the aorta and trachea. Later, it may spread to organs further away such as the liver, lungs and bones.

Q: What is the stratified squamous cell lining of the esophagus?

A: Stratified squamous cells are flat epithelial cells that are composed of several layers, called the epithelium. This type of epithelium forms the surface mucosa that lines the inside of the esophagus.

The esophagus itself 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 in the throat 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.

Who is Affected in the General Population?

Esophageal cancer is the fifth leading cause of death in men from cancer worldwide.1

What Is Cancer Of The Esophagus In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Feldman M, Friedman LS, Brandt LJ. Sleisenger & Fordtran’s Gastrointestinal and Liver Disease. ed 9. Philadelphia: Saunders; 2010. pp. 745–767. []

Edema of the Small Intestine

Intestinal Edema in an 11 Month Old Baby. Courtesy: Nature.com
Intestinal Edema of Duodenum in an 11 Month Old Baby. Courtesy: Nature.com

What Is Small Intestinal Edema?

Small intestinal edema is characterized by fluid accumulation within the intestinal mucosa so that the intestinal wall appears thick and swollen.

Intestinal edema hampers peristalsis that can result in pain and gas build-up. Peristalsis is the normal rhythmic muscular wave-like action that moves residue along the gastrointestinal tract.

Q: What part of the small intestinal lining is swollen?

A: Any part of the small intestine may be affected. Some causes of edema include allergic reactions, enteropathies such as celiac disease, cow milk enteropthy, yeast infection, parasite infection, inflammatory bowel disease such as Crohn’s disease, and certain medications.

For example, the anti-hypertensive drugs known as Angiotension Converting Enzyme (ACE) inhibitors can cause intestinal angioedema and therefore the patient may present with gastrointestinal complaints.1

What Is Small Intestinal Edema In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. LoCascio E J,  Mahler  S A, and  Arnold TC. Intestinal Angioedema Misdiagnosed as Recurrent Episodes of Gastroenteritis. West J Emerg Med. Sep 2010; 11(4): 391–394. []

Neutropenia 

neutrophilWhat Is Neutropenia?

Neutropenia  is a blood disorder characterized by presence of an abnormally low number of neutrophils.

Neutrophils are white blood cells (leukocytes) that serves as the primary defense against infections by destroying bacteria in the blood. 

Specfically, neutrophils are a type of granulocyte that contain granules filled with potent chemicals to break down the microbes they ingest. Some of these chemicals, such as histamine, also contribute to inflammation and allergy.

The process of eating and digesting microbes is called phagocytosis. Neutrophils are phagocytes.1

Q: How do neutrophils eat microbes?

A: Segmented neutrophils  are the mature phagocytes that migrate through tissues to destroy microbes and respond to inflammatory stimuli. Segmented neutrophils comprise 40-75 % of the peripheral leukocytes. They are usually 9 to 16 µm in diameter. The nuclear lobes, normally numbering from 2 to 5, may be spread out so that the connecting filaments are clearly visible, or the lobes may overlap or twist. The chromatin pattern is coarse and clumped. The cytoplasm is abundant with a few nonspecific granules and a full complement of rose-violet specific granules.1

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

Sources:
  1. http://www.wadsworth.org/chemheme/heme/microscope/seg.htm [] []

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:…