celiac_disease_from_a_to_z by hedongchenchen


									Celiac Disease
(gluten-sensitive enteropathy)
• Celiac Disease is a chronic disease of the digestive
• It interferes with the digestion and absorption of
  food nutrients.
• People with celiac sprue cannot tolerate gliadin, the
  alcohol-soluble fraction of gluten
• Celiac disease is caused by an autoimmune reaction
  to gluten.
• The root cause of celiac disease is unknown, but
  inheritance is a risk factor.
• Celiac Sprue is a lifelong disease, and if untreated it is
  associated with increased mortality .
              What Is Gluten ?
• “Gluten” is a general term for a composite of
  the storage proteins gliadin and glutenin.
• These proteins (conjoined with starch) comprise 80%
  of the total protein in wheat/rye/barley seed .

• Corn (maize), sorghum, and rice are considered safe
  for a patient to consume. They do contain types of
  gluten that do not trigger the disease
• Approximately 3 million people in Europe and
  another 3 million people in the United States are
  estimated to be affected by celiac sprue.
• The highest prevalence of celiac sprue is in Ireland
  and Finland and in places to which Europeans
  emigrated, notably North America and Australia.
• The incidence of celiac sprue is increasing among
  certain populations in Africa, Asia (India), and the
  Middle East.
• Celiac disease can be seen first time in any
  part of life.
• Usually, this disorder can be discovered in
  childhood .
• Celiac Disease has a strong hereditary component.
• The prevalence of the condition in first-degree
  relatives is approximately 10%.
• A strong association exists between celiac sprue and
  two human leukocyte antigen (HLA) haplotypes (DQ2
  and DQ8).
• Damage to the intestinal mucosa is seen with the
  presentation of gluten-derived peptide gliadin,
  consisting of 33 amino acids.
• Helper T cells mediate the inflammatory response.
• People with celiac disease have abnormally high
  levels of associated antibodies like: anti-gliadin, which
  unite with antigens (toxic amino acid sequences) that
  are found in wheat, rye, and barley.

• In people with celiac disease, the immune system
  treats gluten as a foreign invader and produces
  elevated levels of antibodies to get rid of it, causing
  symptoms and associated discomfort.
• As a result, intestinal villi gets damaged (shortening
  and villous flattening)
 How does gluten cause negative effects?
• Certain Pro- and Gln-rich gliadin peptide fragments
  survive the digestion process & make it to the gut
• These peptides are deamidated by tissue
  transglutaminase (tTGase)
• APCs in HLA-DQ2 or –DQ8 positive individuals
  express these deamidated peptide fragments on
  class II MHC molecules
• The resulting CD4+ T-cell mediated immune response
  can eventually result in the development of celiac
• Therefore, the only currently recognized
  treatment for celiac disease is complete
  abstinence from food grains containing gluten
  proteins .
                  Risk Factors
• Having a risk factor for Celiac Disease makes the
  chances of getting a condition higher but does not
  always lead to Celiac Disease

• Celiac Disease can be associated with disorders such
  as thyroid disease, Anemia of unknown cause,
  type I diabetes or other autoimmune disorders.

• Family history of Celiac or autoimmune disorders.
• Race: Celiac sprue is most prevalent in Western
  Europe and the United States. The incidence is
  increasing in Africa and Asia.
• Sex: Incidence of celiac sprue is slightly higher in
  females than in males.
• Age: The age distribution of patients with celiac
  disease is bimodal, the first at 8-12 months and the
  second in the third to fourth decades. The mean age
  at diagnosis is 8.4 years (range, 1-17 y).
• Italian race : celiac is common in Italy and
• Large amounts of gluten at weaning are associated
  with an increased risk for developing celiac disease.
• Finally, repeated rotavirus infections in infancy
  appear to be associated with a higher risk of
  developing celiac disease autoimmunity in
  genetically predisposed individuals
       Histology of Celiac Disease
• The histological lesion of gluten sensitivity
  primarily affects the proximal small bowel.
• the damage caused by celiac disease can be
  more extensive than once thought, and that it
  likely affects the entire small bowel, rather
  than just the lamina propriaand crypt regions.
• The lesion is described as subtotal villus
The difference between normal (left) and abnormal (right) Mucosa
We can see counts of lymphocytes and plasma cells in lamina of damaged Mucosa

• Celiac Disease has wide spread symptoms.

• The list of signs and symptoms of Celiac
  Disease includes about 35 symptoms .

• Symptoms in children may differ from them in
• The most common symptoms in adults are :

   1-abdomenal pain
   3-Pale stools
   4-Weight loss
• The most common symptoms in children are :

    1-Delayed growth
    4-Weight loss
    5-Bloating abdomen
Celiac Disease in Children
• Older children with celiac disease who present
  with GI manifestations may have onset of
  symptoms at any age.

• The variability in the age of symptom onset
  possibly depends on the amount of gluten in
  the diet and other environmental factors

• Physical findings depend on extent of celiac disease.
• Celiac disease may occur in asymptomatic individuals
  without any positive clinical findings
• Bloating of the abdomen is a relatively common
  finding .
• Dry mucosal membranes with vomiting or diarrhea
  indicate the degree of dehydration.
• Some extraintestinal manifestations of celiac
  disease can be shown in some patints.

• The most common signs we can see are:

     1-weight loss        6-peripheral neuropathy
     2-fluid retention    7-infertility
     3-anemia             8-muscle weakness
     5-bruising easily
Dermatitis herpetiformis:

• A blistering skin rash that involves the elbows,
  knees, and buttocks are associated with dermal
  granular IgA deposits .

• Dermatitis herpetiformis is a rare occurrence in
  childhood and is described almost exclusively in
  teenagers and adults.
Dental enamel hypoplasia:

• These enamel defects involve only the permanent
  dentition and may be the only presenting
  manifestation of celiac disease.

• Often, GI symptoms are minimal or absent.
Short stature and delayed puberty:

• 10% of children with idiopathic short stature may
  have celiac disease that can be detected on serologic

• Adolescent girls with untreated celiac disease may
  have delayed onset of menarche.
Arthritis and arthralgia:

• Arthritis can be a common extraintestinal
  manifestation of adults with celiac disease, including
  those on a gluten-free diet.

• 3% of children with juvenile chronic arthritis may
  have celiac disease.
Psychiatric disorders:

• celiac disease can be associated with some
  psychiatric disorders, such as depression and anxiety.

• These conditions can be severe and usually respond
  to a gluten-free diet.
 First, a thorough physical examination is
 conducted, including a series of blood tests.

Second, a duodenal biopsy is performed with
 multiple samples from multiple locations in
 the small intestine.

 And third,the gluten-free diet is implemented
• Physical Examination:

  1-pallor (due to anemia)
  2-hypotension (low blood pressure)
  3-edema (due to low levels of protein, [albumin] in
 the blood)
  4-dermatitis herpetiformis (skin lesions)
5-easy bruising (lack of vitamin K)
6-loss of various sensations in extremities including
vibration, position and light touch (vitamin
7-signs of severe vitamin/mineral deficiencies which
may include:
     -diminished deep tendon reflexes
     -muscle spasms
• Serologic Tests

  – EMA (Immunoglobulin A anti-endomysium
  – AGA (IgA anti-gliadin antibodies) Some people do
    not produce IgA antibodies.
  – DGP (Deamidated gliadin peptide antibody)
  – tTGA (IgA anti-tissue transglutaminase)

• It is the key of diagnosis.
• a biopsy of the small intestine [jejunal] is
  called for.
• the tissue samples are examined under a
  microscope for signs of injury
The biopsy can be taken by inserting a tube through the mouth and throat

Celiac Disease
• Under the microscope, we see the atrophy of
  Mucosa and Elevated numbers of T-cell
  lymphocytes .

• The small bowel biopsy samples of persons
  with dermatitis herpetiformis often show
  similar damage.

• The prognosis for patients with correctly diagnosed
  and treated celiac sprue is excellent.

• The prognosis for patients with celiac sprue who are
  not responding to gluten withdrawal and
  corticosteroid treatment is generally poor.

• Mortality is usually associated with water and
  electrolyte depletion.

• There are two main ways to treat and control
  celiac disease:

      1- Diet
      2- Corticosteroids
 Diet:

• Complete elimination of gluten-containing grain
  products, which include wheat, rye, and barley, is
  essential to treatment.
• After an initial period of avoidance, oats might be
  reintroduced into the diet of patients with celiac
• These patients should be monitored carefully for
  recurrent symptoms.
Avoid the gluten
       Gluten-free food
These days you can find meals made
especially for celiac disease patints,
  which have no gluten content.

• Corticosteroids have anti-inflammatory properties
  and cause profound and varied metabolic effects.

• These agents modify the body's immune response to
  diverse stimuli.
• Prednisone: Can be used in patients with
  refractory celiac sprue

 Adult:
  30-40 mg/d PO; taper off completely in 6-8 wk
 Pediatric:
   1 mg/kg/d PO; not to exceed 30 mg/d; taper off completely
  in 6-8 wk
The End

          By: Usef Bada

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