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Osteopenia In Childhood 

sclerodermaWhat Is Scleroderma?

S cleroderma is a chronic skin manifestation of progressive systemic sclerosis characterized by generalized thickened, edematous skin firmly bound to subcutaneous tissue which causes limited movement.

Systemic sclerosis a connective tissue disease that involves destructive changes in the skin, blood vessels, muscles, and internal organs. The course can be mild or it can be fatal. Cardiopulmonary complications from fibrosis are the most common cause of death.

Gastrointestinal problems mainly due to fibrosis affect 50 to 90% of patients.1

Q: Is there a cure for scleroderma?

A: There is no cure for scleroderma. Treatment is aimed at improving symptoms.

  • Heartburn (acid reflux) can be treated with antacid drugs.
  • Scleroderma kidney disease can be treated with blood pressure medications called “angiotensin converting enzyme inhibitors” (ACE inhibitors). These can often effectively control kidney damage if started early and use of these drugs has been a major advance for treating scleroderma.
  • Muscle pain and weakness can be treated with anti-inflammatory drugs such as prednisone, intravenous immunoglobin (IVIg), and/or immunosuppressive medications. Physical therapy may be useful to maintain joint and skin flexibility.2

 

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

Sources:
  1. Forbes A, Marie I. Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology (Oxford). 2009 Jun;48 Suppl 3:iii36-9. doi: 10.1093/rheumatology/ ken485. [↩]
  2. http://www.rheumatology.org [↩]

Glycogenic Acanthosis (Esophageal Plaques)

What Is Intraepithelial Lymphocytosis In Normal Small Bowel Samples? I ntraepithelial lymphocytosis is characterized by an abnormal increase in the numbers of intraepithelial lymphocytes (IELs) that are present in the small intestinal mucosa. Q: What… 

Hypotonia

In this photo, the spleen is the darkish oval organ in the lower middle of this photo. In your body, it is located in your upper abdoman to the left of your stomach under your ribs.
The spleen is the darkish oval organ in the lower middle of this photo.

What Is Hyposplenism?

H yposplenism is the condition resulting from having lost spleen tissue, called atrophy of the spleen. Spleen atrophy impairs splenic functions or activities because there are insufficient tissues to do the work required.

Q: What splenic functions are impaired?

A: The spleen, apart from acting as a phagocytic filter, thus removing aging and damaged cells, is crucial in regulating immune homeostasis by linking innate and adaptive immunity, and in protecting against infections by encapsulated bacteria.1

Impaired function of the spleen therefore increases risk of infections with encapsulated bacteria because of inability to mount a proper defense and to filter and remove bacteria from the circulation.

The spleen is a highly vascular and solid organ about the size of a fist. It has a delicate structure inside that is enclosed by fibrous, elastic layers consisting of connective tissue.

The tissues within are made up of two different types of tissues, called white pulp and red pulp. White pulp carries out lymphoid functions. Red pulp filters and cleanses the blood. The spleen is situated above the stomach on the left side of the upper abdomen and firmly fixed in place by ligaments and ribs.

The spleen is an important organ of the lymph system, having the largest collection of lymph tissue in the body. It functions to produce antibodies (immunoglobulins) and white blood cells (T-cells and B-cells), help control the amount of blood in the body, keep body fluids in balance, destroy and filter out old and damaged cells2 and salvage the iron needed for producing new blood cells, and lastly, clear bacteria through production of substances that enable phagocytosis (engulfing bacteria and other unwanted particles, such as antigens, from blood).

Children and adults with hyposplenism are at risk for overwhelming infections. Management of hypospenism is directed towards preventing pneumonia by immununization against pneumonia and meningitis and treating bacterial infections as they arise, which may require hospitalization. For some patients, life-long treatment with antibiotics, such as erythromycin and penicillin, are recommended.

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

Sources:
  1. Di Sabatino A, Brunetti L, Carnevale Maffè G, Giuffrida P, Corazza GR. Is it worth investigating splenic function in patients with celiac disease? World J Gastroenterol. 2013 Apr 21;19(15):2313-8. doi: 10.3748/wjg.v19.i15.2313. [↩]
  2. http://www.nlm.nih.gov/medlineplus/spleendiseases.html [↩]

Delayed Puberty In Girls 

sick woman on bed, symptom of cold, flu, insomnia, stress, headache, hangover, dizzinessWhat Is Hypokalemic Rhabdomyolysis?

H ypokalemic rhabdomyolysis is an acute and sometimes fatal disease due to its rapid progression of muscle destruction when untreated.

It is characterized by the accumulation of by-products of skeletal muscle destruction in the renal (kidney) tubules and producing acute kidney failure caused by rapid potassium loss.

This condition puts you in bed because the legs muscles cannot support the body and arms are too weak to move.

What Is Hypokalemic Rhabdomyolysis In Celiac Disease and/or Gluten Sensitivity and Dermatitis Herpetiformis?

Juvenile Diabetes (Type 1 Diabetes Mellitus)

Muscle pain in celiac disease and gluten sensitivityWhat Is Chronic Muscle Pain And Tenderness?

M uscle pain and tenderness, also called myalgia, is a disorder of muscle tissue.

Muscle pain that is ongoing or chronic can result from a variety of disorders, infections, overuse, injury, as an unwanted effect of certain medications, toxic substances, celiac disease, and certain nutritional deficiencies.

What Is Muscle Pain and Tenderness In Celiac Disease and/or Gluten Sensitivity?

Chronic Bullous Dermatosis of Childhood or Linear IgA Dermatosis

Muscle spasm and cramping in celiac disease and gluten sensitivity symptomWhat Are Muscle Spasm, Muscle Cramps?

M uscle spasm and muscle cramps are  disorders of muscle function caused by painful involuntary contractions of affected skeletal muscles characterized by limited movement.

Q: What is the difference between muscle spasm and cramps?

A: Cramps are stronger and more painful than spasms, occurring while the muscle is in its most shortened state.

Skeletal muscles are those that move the body skeleton to do work as we choose. Examples are muscles that move the mouth, arms,  hands, legs, and feet.

What Are Muscle Spasm, Muscle Cramps In Celiac Disease and/or Gluten Sensitivity?

Dermatitis Herpetiformis In Childhood

muscle wastingWhat Is Muscle Wasting?

M uscle wasting, or atrophy, is a muscle disorder resulting from the loss of muscle tissue which is characterized by thin muscles that have reduced strength and endurance.

What Is Muscle Wasting In Celiac Disease and/or Gluten Sensitivity?

Bleeding: Fecal Occult Blood In Children 

Muscle fiber anatomy. Courtesy NIH.
Muscle Fiber Anatomy. Courtesy NIH.

What Is Muscle Weakness?

M uscle weakness is the impaired status of muscle function characterized by decreased or low muscle strength and inability to perform normal work such as lifting a pot off the stove.

Q: How do muscles work?

A: Muscles do their work by contracting or shortening. For example, to move the foot up and down at the ankle, muscles attached to the foot by tendons must contract to shorten or relax to return to their resting length. Calf muscles contract to point the foot down (flexion) while the shin muscles relax (extension).  For the foot to point up, calf muscles relax while the opposing shin muscles contract.

Each muscle is made up of individual muscle fibers. A muscle fiber is a long cylindrical cell that contains many nuclei, mitochondria, and sarcomeres. Each muscle fiber is surrounded by a thin layer of connective tissue called the endomysium.

Approximately 20–80 of these muscle fibers are grouped together in a parallel arrangement called a muscle fascicle or fiber bundle that is encapsulated by a perimysium. A distinct muscle is formed by enveloping a large number of muscle fascicles in a thick collagenous external sheath extending from the tendons called the epimysium.1

Muscles fall into three types:

  • Voluntary muscles.  These muscles, also called skeletal, we can control by will. Voluntary muscles function by contracting their fibers to draw one part of the body toward another in flexion while opposing muscles that extend or pull a body part away from another. They move our bones to perform activities such as walking to get somewhere, chewing to eat food, lifting to do work, and moving the eyeball to look at something.
  • Involuntary muscles. These muscles work independently of our conscious control. They are needed for internal organs, sphincters, and other parts to do their work, such as peristalsis in the gut that must function at all times to digest and move food, the squirting of bile juice into the duodenum by the Sphincter of Odi in the presence of fat eaten, and action of the pupil to see.
  • Cardiac muscles. These muscles are specialized to keep the heart functioning at all times.

  Muscle weakness can involve all types of muscles.

What Is Muscle Weakness In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. http://www.ncbi.nlm.nih.gov/books/NBK57140/ [↩]

Osteitis Fibrosa Cystica 

Deep Vein Thrombosis in the Right Leg with Swelling and Redness. Coutesy wikipedia.
Deep Vein Thrombosis in the Right Leg with Swelling and Redness. Courtesy wikipedia.

What Is Antiphospholipid Syndrome?

Antiphospholipid syndrome (APS) is an autoimmune disease and a blood clotting disorder characterized by these clinical and laboratory criteria:

Clinical criteria – recurrent vascular thrombosis (clots in veins/arteries) from hypercoagulability (abnormal excessive clotting) and/or recurrent complications of pregnancy that include loss of the fetus  (miscarriage) and pre-eclampsia or eclampsia.

Laboratory criteria – persistently elevated anticardiolipin, anti–beta-2 glycoprotein I, and/or lupus anti-coagulant antibodies in blood.

In antiphospholipid syndrome autoantibodies are produced by the body and directed against negatively charged phospholipids that are found in the outer layer of cell membranes and platelets. B2-glycoprotein-I (a protein in blood plasma) has been found as a major target antigen for antiphospholipid antibodies.

Q: Are phospholipids important in the body?

A: Yes.  Phospholipid molecules are an essential part of cell membranes. They form a barrier around cells that protect the cell, allow movement of oxygen in and carbon dioxide out of the cell, and regulate other small molecules through the cell wall. Because phospholipids are widespread in the body, this disorder can produce a large variety of symptoms and affect many organs.

One severe effect of APS is the development of a blood clot in a vein deep in the arm or leg, called deep vein thrombosis (DVT). DVT can cause pain, swelling, redness, or increased warmth in the affected limb. Deep vein clots can break off, travel to the lungs, and cause pulmonary embolism.1 Pulmonary embolism is a medical emergency.

Treatment is with anticoagulant medications and blood monitoring.

What Is Antiphospholipid Syndrome In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. http://www.nhlbi.nih.gov/health/health-topics/topics/ebc/signs.html [↩]

Osteomalacia

Drawing shows changes in airways during asthma attack. wikipedia
Drawing shows changes in airways during asthma attack. wikipedia

What Is Asthma?

Asthma is a chronic immune respiratory condition characterized by narrowing and inflammation of the lung airways (large bronchi, bronchial tubes and small bronchioles) in response to an allergen as the trigger or stimulus. As such, asthma occurs in episodes and does not result in progressive loss of pulmonary function.

During an asthma attack, airways constrict, trapping air so lungs become overinflated.  Normally, bronchial airways bring air to millions of air sacs that are attached to the ends of bronchioles. Air sacs, called alveoli, are only one cell thick to allow for rapid exchange of gases.

That is, oxygen from air breathed into the sacs moves into the bloodsteam and carbon dioxide is released from the bloodstream to air that is breathed out of air sacs.

The outer walls of bronchioles are made up of muscles which, in the process of breathing, normally contract on expiration to help expel air and then relax. During an asthma attack, these muscles abnormally constrict, impairing airflow into and out of the alveoli. This is called bronchospasm.

Common  allergens that cause inflammation include airborne dust mite feces, mold, and pollen and foods such as wheat, cow’s milk, eggs, and peanuts. Non-allergenic triggers include exercise, air pollution, smoking, and viral respiratory infection.

Q: What effect does inflammation have on the lungs?

A: Inflammation causes local tissue edema or swelling of the bronchioles and mucus formation. Inflammation with increased mucus secretions and edema narrows the airways that connect to alveoli which makes breathing difficult.  Two things happen:

  1. Inflammation  impairs exchange of gases in alveoli, resulting in lack of sufficient oxygen (O2) for body cell functions, called hypoxia, and build-up of carbon dioxide (CO2) in blood, called CO2 retention.
  2. Inflammation narrows passageways because of swelling, which reduces the movement of air to and from the alveoli through the airways, and this puts stress on the right side of the heart.

Treatment is aimed at controlling bronchospasm and reducing inflammation. Untreated asthma can be disabling and life threatening.

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