celiac_disease_from_a_to_z by hedongchenchen

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									Celiac Disease
(gluten-sensitive enteropathy)
                    Introduction
• Celiac Disease is a chronic disease of the digestive
  tract.
• 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
                   Frequency
• 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 .
              Pathophysiology
• 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
  disease
• 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
  descendents.
• 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
  atrophy.
The difference between normal (left) and abnormal (right) Mucosa
We can see counts of lymphocytes and plasma cells in lamina of damaged Mucosa
                 Symptoms


• 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
  adults.
• The most common symptoms in adults are :

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

    1-Delayed growth
    2-irritability
    3-Anemia
    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
                       Signs

• 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
     4-osteoporosis
     5-bruising easily
                Complications
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
  testing.

• 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.
                 Diagnosis
 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
deficiency)
7-signs of severe vitamin/mineral deficiencies which
may include:
     -diminished deep tendon reflexes
     -muscle spasms
• Serologic Tests

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

• 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
      Normal




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.
                    Prognosis

• 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.
                 Treatment

• 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
  disease.
• 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:

• 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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