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Anemia, Refractory Iron Deficiency In Childhood (Unresponsive To Iron)

Psoriasis_on_back[1]

 What Is Psoriasis?

P soriasis is a chronic, autommune relapsing skin disorder characterized by scaling, erythema (redness), and less commonly, pustulation.1 

The body surface area affected and the degree to which psoriasis is a problem varies considerably among patients and over time.2 Often there are additional manifestations in the nails and in joints.3

Q: Are there different forms of psoriasis?

A: There are five forms of psoriasis. The lesions in all forms are itchy and red but vary in appearance and severity. Plaque psoriasis is the most common form observed in more than 80% of patients. Atypical forms include guttate, inverse, pustular, and erythrodermic psoriasis.4

  • Plaque psoriasis features thickened or raised red areas that have a distinct edge and are covered with silvery white buildup of flaky skin typically on elbows, knees, scalp and buttocks.
  • Gutate psoriasis appears as small, flat red patches with shiny buildup that are not usually painful, just itchy. There may be a few or many patches and they can group together.
  • Inverse psoriasis affects folds of skin, armpits and the groin area. Lesions are deep red with shiny buildup. It can be a thin red area along a crease line or involve, for example, the whole armpit.
  • Pustular psoriasis features an itchy, red base followed by blisters of white, non-infectious pus that appears glossy after a day or two and then sloughs in cycles. These areas may be limited to certain areas such as the hands and feet or be more widespread.
  • Erythrodermic psoriasis involves large areas of the body’s surface, inflaming normal skin and changing it into very red, raw looking flesh that is painful, swollen and itchy. This form requires extensive treatment, and complications can be life-threatening. Fortunately, this form of psoriasis is the least common.

Psoriasis in children has been reported to differ from that among adults being more frequently itchy and plaque lesions are relatively thinner, softer, and less scaly, face and flexural involvement is common and guttate type is the characteristic presentation.5

In children, psoriasis is a common skin disorder with about one third of all patients having onset of disease in the first or second decade of life. A chronic disfiguring skin disease, such as psoriasis, in childhood is likely to have profound emotional and psychological effects, and hence requires special attention.6

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

Sources:
  1. Addolorato G, Parente A, de Lorenzi G, et al. Rapid regression of psoriasis in a coeliac patient after gluten-free diet. A case report and review of the literature. Digestion. 2003;68(1):9-12. [↩]
  2. Stern, R. S., Nijsten, T., Feldman, S. R., Margolis, D. J. and Rolstad, T.

    Psoriasis is common, carries a substantial burden even when not extensive, and is associated with widespread treatment dissatisfaction. J. Invest. Dermatol. Symp.. 2004 Mar;9(2):136-9.. [↩]

  3. Weigle N, McBane S. Psoriasis. Am Fam Physician. 2013 May 1;87(9):626-33. [↩]
  4. Weigle N, McBane S. Psoriasis. Am Fam Physician. 2013 May 1;87(9):626-33. [↩]
  5. Dogra S, Kaur I. Childhood psoriasis. Indian J Dermatol Venereol Leprol. 2010 Jul-Aug;76(4):357-65. doi: 10.4103/0378-6323.66580. [↩]
  6. Dogra S, Kaur I. Childhood psoriasis. Indian J Dermatol Venereol Leprol. 2010 Jul-Aug;76(4):357-65. doi: 10.4103/0378-6323.66580. [↩]

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?

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/ [↩]

Anemia, Severe Iron Deficiency In Pregnancy 

Dermatitis Herpetiformis. Blisters Opened Giving Relief From Pain and Itching.
Dermatitis Herpetiformis On Forearm. Skin Is Darkened Where Old Blisters Healed.

What Is Dermatitis Herpetiformis?

D ermatitis herpetiformis (DH) is an autoimmune extremely itchy, painful bullous skin rash (blistering eruptions) arising from the underlying dermis layer of skin as a consequence of gluten sensitivity.

Dermatitis herpetiformis is characterized by multiple intensely itchy, red blisters appearing on the elbows which can extend down the forearm to the wrist and the knees. Less usual areas involve the back, buttocks, scalp, and abdomen.

Q: Do the blisters leave a mark when healed?

A: Crops of skin eruptions begin with itching or a burning sensation in reddened papules. There are grouped vesicles and tense blisters. The blister contents may be serous or bloody, with symmetrical distribution (eg, both knees or both elbows). Fluid filled elements rupture leaving denuded areas of sore skin and crust. Subsequently, there is residual hypopigmentation (a white area) or hyperpigmentation (dark area).1

Rupture of blisters begins relief from intense burning and itching.

Dermatitis Herpetiformis Eruptions On Knees.
Dermatitis Herpetiformis Eruptions On Knees. Notice White Areas Showing Loss of Pigmentation From Healed Blisters.

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

Primary care providers should be aware of this skin condition, as they are more likely than a gastroenterologist to be confronted with this type of presentation of celiac disease.2

Sources:
  1. Mendes FB, Hissa-Elian A, de Abreu MA, Gonçalves VS. Review: dermatitis herpetiformis. An Bras Dermatol. 2013 Jul-Aug;88(4):594-9. [↩]
  2. Robinson BL, Davis SC, Vess J, Lebel, J. Primary care management of celiac disease. Autoimmune Disorders. Nurse Practitioner. February 2015: Vol 40 – Issue 2; 28–34. [↩]

Spina Bifida 

pityriasis rubraWhat Is Pityriasis Rubra Pilaris?

P ityriasis rubra pilaris (PRP) is a chronic generalized exfoliative dermatitis (sloughing skin) characterized by erythema (redness), scaling, dilated plugged hair follicles, and keratoderma (thickened skin) of the hands and feet that is often associated with anemia and low serum albumin.

It may manifest either as Type I classical adult onset PRP, Type II atypical adult (onset) PRP, or Type VI PRP (HIV-associated PRP pityriasis rubra pilaris) in contrast to classical juvenile (Type III) and circumscribed juvenile (Type IV) encountered among children.1

Q: Who is affected in the general population?

A: All ages are affected. Pityriasis rubra pilaris occurs all over the world but with racial variations – it is 1 in 5,000 in Great Britain and 1 in 50,000 in India.2

What Is Pityriasis Rubra Pilaris In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Sehgal VN, Srivastava G, Dogra S. Adult onset pityriasis rubra pilaris. Indian J Dermatol Venereol Leprol. 2008 Jul-Aug;74(4):311-21. [↩]
  2. Sehgal VN, Srivastava G, Dogra S. Adult onset pityriasis rubra pilaris. Indian J Dermatol Venereol Leprol. 2008 Jul-Aug;74(4):311-21. [↩]

Bronchoalveolitis Leading to Bronchitis and Pneumonia

Constipation in a young child as seen on X-ray. Lowest circle shows hard feces in the pelvis. Source, James Heilman, MD.

What Is Chronic Constipation?

C hronic constipation is an intestinal motility disorder characterized by abnormal stool formation, consistency, and evacuation.

Motility disorder means the normal rhythmic movement of intestinal muscles, called peristalsis, that moves food matter through the gut is hampered or dysfunctional.

Studies show that methane gas present in the colon induces constipation by delaying transit time, which is the time it takes for stool to pass through the colon.

Researchers investigating the relationship between methane and constipation found that methane positivity was detected in 75% of patients with slow transit, 44% of patients with normal transit and and 28% of the patients who were controls. However, methane positivity was not related with stool consistency.1

Other researchers investigating the total amount of methane produced found that there was significantly more methane production in patients with constipation (21.1 ppm vs. 6.1 ppm, respectively) than in controls without constipation.2

Q. How does methane get into the colon?

A. Methane is produced in the colon by intestinal methanogens (microbes) that metabolize hydrogen, one of the end products of normal anaerobic (meaning without oxygen) bacterial fermentation.  Fermentation of the undigested starchy part of carbohydrates produces hydrogen in the intestine which is the substrate (food) for methane production by intestinal methanogens.

Hydrogen and methane are excreted in the flatus and in breath giving the opportunity to indirectly measure their production using breath testing. Methane is detected in 30%-50% of the healthy adult population worldwide.3

Other common causes of constipation include not getting enough exercise, not drinking enough fluids, not eating enough fiber in the diet, not eating foods that supply microbes needed by the colon (probiotics), not eating foods that nourish the good microbe population (prebiotics) and supply minerals needed for healthy movement of stool, and food sensitivities. Too much cows milk is a common cause of stool that forms into balls.

Who is Affected in the General Population? Chronic constipation is a remarkably common and costly condition that can negatively impact the quality of life and result in a major social and economic burden. Based on the definition, either self-reported or using Rome criteria, chronic constipation can affect up to 27% of the population. There is strong evidence that constipation occurs more frequently in women.4

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

Sources:
  1. Triantafyllou K, Chang C, Pimentel M. Methanogens, Methane and Gastrointestinal Motility. J Neurogastroenterol Motil. 2014 Jan;20(1):31-40. Epub 2013 Dec 30. [↩]
  2. Triantafyllou K, Chang C, Pimentel M. Methanogens, Methane and Gastrointestinal Motility. J Neurogastroenterol Motil. 2014 Jan;20(1):31-40. Epub 2013 Dec 30. [↩]
  3. Triantafyllou K, Chang C, Pimentel M. Methanogens, Methane and Gastrointestinal Motility. J Neurogastroenterol Motil. 2014 Jan;20(1):31-40. Epub 2013 Dec 30. [↩]
  4. Sanchez MI, Bercik P. Epidemiology and burden of chronic constipation. Can J Gastroenterol. 2011 Oct;25 Suppl B:11B-15B. [↩]

Night Blindness – Nyctalopia

What Is Steatorrhea?

S teatorrhea is a condition of abnormal stool composition and consistency that is due to high fat content because fat from food is not  digested and absorbed into the body but rather passes out with stool.1

Q: How does fatty stool look?

A: The stool in steatorrhea appears pale, bulky and may float in the toilet. If stool is loose, it varies from a pudding consistency to whipped cream. If it is solid, it can vary from thin sqiggles to big turds that smear the sides of the toilet bowl. All types have a foul smell.

Fat of any kind must be digested, or broken down, by enzymes into fatty acids and glycerol and then these parts only are absorbed through the small intestinal lining into the body. The failure to digest or absorb fats deprives the body of these foodstuffs needed for many functions such as blood clotting and vital cell parts such as eye and brain structure.

Fat malabsorption produces malabsorption of vitamins A, D, E, and K because these vitamins can only be absorbed along with fatty acids.

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

Sources:
  1. Murray JA, The widening spectrum of celiac disease. American Journal of Clinical Nutrition. Mar 1999;69 (3):354-365. [↩]

Dysgeusia (Impaired Taste)

DyspareuniaWhat Is Autoimmune Thyroiditis (Hypothyroidism)?

A utoimmune thyroiditis, also called Hashimoto’s thyroiditis or Hashimoto’s Disease, is an autoimmune destruction of thyroid tissue characterized by insufficient thyroid hormone circulating in the body that causes formation of a goiter (enlarged thyroid gland) and hypothyroidism.

Hypothroidism refers to the condition of markedly reduced secretion of thyroid hormone. There are other causes of hypothyroidism besides Hashimoto’s thyroiditis.

Hashimoto’s thyroiditis is often associated with other autoimmune diseases such as celiac disease.

In Hashimoto’s thyroiditis, a profusion of antibodies are produced, which build up in the blood. Left untreated so that much of the thyroid gland is destroyed, this condition may progress to the very serious and life-threatening condition called myxedema.

Note: In myxedema, protein, electrolytes, and water abnormally accumulate in between cells which produce firm, inelastic puffy skin that is cool, dry, rough, scaly, and may appear yellow; in some people, areas such as the ankles become crusty with a look of tree bark. Many systemic changes develop shown by significant slowing of mental and physical functions. Please see below.

Q: What thyroid tissue is targeted for destruction?

A: In Hashimoto’s thyroiditis, high levels of autoantibodies target thyroglobulin and thyroid peroxidase, leading to inflammation and destruction of the thyroid gland. The resulting fibrosis or scarring of the gland results in lack of thyroid hormone production.

The thyroid gland consists of a large number of closed vesicles that contain a homogenous substance called colloid, which contains the thyroglobulin. Thyroglobulin is an iodine-containing protein secreted by the thyroid gland and stored within its colloid, from which the thyroid hormones thyroxine (T4) and triiodothyroinine (T3) are derived.1

T3 is the active hormone and is made from T4. Thyroid hormones affect metabolism, brain development, breathing, heart and nervous system functions, body temperature, muscle strength, skin dryness, menstrual cycles, weight, and cholesterol levels.

Thyroid hormone production is regulated by thyroid-stimulating hormone (TSH), which is made by the pituitary gland in the brain. Normally, when thyroid hormone levels in the blood are low, the pituitary releases more TSH. When thyroid hormone levels are high, the pituitary decreases TSH production.

Hashimoto’s disease, with or without the development of hypothyroidism, is treated with synthetic thyroxine, which is man-made T4. Health care providers prefer to use synthetic T4, such as Synthroid® (Levothyroxine), rather than synthetic T3, because T4 stays in the body longer, ensuring a steady supply of thyroid hormone throughout the day. The thyroid preparations made with animal thyroid are not considered as consistent as synthetic thyroid.2

What Is Autoimmune Thyroiditis In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Taber’s Cyclopedic Medical Dictionary. 19th ed. F.A. Davis Company. Philadelphia, PA. [↩]
  2. National Endocrine and Metabolic Diseases Information Service. [↩]

Hypocalciuria (Low Urine Calcium)

IgA Molecule.
Depiction of the IgA Molecule

What Is IgA Deficiency?

I gA deficiency (IgAD) is an immunodeficiency disease characterized by lack of immunoglobulin A type antibody production, called IgA antibody, with no detectable levels in blood or secretions.

Q: What is an IgA antibody?

A: IgA is an antibody of the immune system that is secreted by plasma cells (specialized white blood cells) through epithelial cell linings of mucosal surfaces into mucosa secretions to protect the lining from microbe invasion.

In fact, immunoglobulin class A is the main protein of the mucosal immune system. This includes mucosa of the eye surface, digestive tract, respiratory tract, urinary tract, and genital tract.

Both major histocompatibility complex (MHC) and non-MHC genes contribute to susceptibility to the disease. The former genes appear to be located in different parts of the MHC region depending on the HLA haplotype. The latter show a marked overlap with genes associated with a variety of autoimmune disorders including Graves’ disease, systemic lupus erythematosus, type 1 diabetes and celiac disease, suggesting common pathophysiological mechanisms. The involvement of genes associated with autoimmunity may suggest that IgAD in itself is an autoimmune disease.1

IgA deficiency may progress into a common variable immunodeficiency (CVID).2

What Is IgA Deficiency (IgAD) In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Wang N, Hammarström L. IgA deficiency: what is new? Curr Opin Allergy Clin Immunol. 2012 Dec;12(6):602-8. doi: 10.1097/ACI.0b013e3283594219. [↩]
  2. Binek A, Jarosz-Chobot P.Selective immunoglobulin A deficiency. Pediatr Endocrinol Diabetes Metab. 2012;18(2):76-8. [↩]