Public Meeting on Gluten-Free Food Labeling - Text Version of PowerPoint Presentation by Frank A. Hamilton
Slide 1 - Celiac Disease-A not so Uncommon Disorder
Frank A. Hamilton, M.D., MPH
National Institutes of Health
National Institute of Diabetes, Digestive and Kidney Diseases
August 19, 2005
Slide 2 - Definition
Celiac disease is an immune-mediated enteropathy caused by a permanent sensitivity to gluten in genetically susceptible individuals.
It occurs in symptomatic subjects with gastrointestinal and non-gastrointestinal symptoms, and in some asymptomatic individuals, including subjects affected by:
Type 1 diabetes
Selective IgA deficiency
First degree relatives of individuals with celiac disease
Slide 3 - Clinical Manifestations
Non-gastrointestinal ( “atypical”)
Slide 4 - The Celiac Iceberg
Image of The Celiac Iceberg. A grey triangle with mountain peaks at the top. The bottom of the triangle is labeled "Latent Celiac Disease." Brackets on the side of the triangle enclose this region and label it as "Normal Mucosa." Above that across the middle of the triangle is horizontal line. Above the line, the area is titled "Silent Celiac Disease." A bracket on the side of the triangle that runs from this horizontal line to the top of the triangle names the region "Manifest mucosal lesion." Then there is a dotted-line running across the top of the triangle near the peaks. A arrow pointing to the top of the triangle indicates the top regain as "Symptomatic Celiac Disease. Along the bottom of the triangle is a bracket running the length of the triangle base labeling the region "Genetic susceptibility: - DQ2, DQ8 Positive serology."
Slide 5 - Gastrointestinal Manifestations (“Classic”)
Most common age of presentation: 6-24 months
Chronic or recurrent diarrhea
Failure to thrive or weight loss
Rarely: Celiac crisis
Slide 6 - Typical Celiac Disease
Two photos lined up side by side. The first photo on the left is of a naked baby with his eyes hidden by a bar. The baby has abdominal distention. The picture on the right is a back view of a naked child. There are folds around the buttocks primarily due to weight loss.
Slide 7 - Non Gastrointestinal Manifestations
Dental enamel hypoplasia of permanent teeth
Slide 8 - Dermatitis herpetiformis
Two photos of Dermatitis Herpetiformis sores. One image is of an elbow the other a close up of skin.
Slide 9 - 3 – Asymptomatic
No or minimal symptoms, “damaged” mucosa and positive serology
Identified by screening asymptomatic individuals from groups at risk such:
First degree relatives
Down syndrome patients
Type 1 diabetes patients, etc.
Slide 10 - 3 – Asymptomatic
Latent: No symptoms, normal mucosa
- May show positive serology. Identified by following in time asymptomatic individuals previously identified at screening from groups at risk. These individuals, given the “right” circumstances, will develop at some point in time mucosal changes (± symptoms)
Slide 11 - Associated Conditions
Graph displaying associated conditions data. Transcript of presentation provides speaker explanation.
Slide 12 - Relatives
Healthy population: 1:133
1st degree relatives: 1:18 to 1:22
2nd degree relatives: 1:24 to 1:39
Slide 13 - Major Complications of Celiac Disease
Dental enamel hypoplasia
Gluten ataxia and other neurological disturbances
Refractory celiac disease and related disorders
Slide 14 - Epidemiology
The “old” Celiac Disease Epidemiology:
A rare disorder typical of infancy
Wide incidence fluctuates in space (1/400 Ireland to 1/10000 Denmark) and in time
A disease of essentially European origin
Slide 15 - “Mines” of Celiac Disease Were Found Among:
photo - diagram explaining where "mines" of Celiac disease were found
Slide 16 - Celiac Disease Epidemiological Study in USA
photo - diagram displaying epidemiological data
Projected number of celiacs in the U.S.A.: 2,115,954
Actual number of known celiacs in the U.S.A.: 40,000
For each known celiac there are 53 undiagnosed patients.
A. Fasano et al., Arch Int Med 2003;163:286-292.
Slide 17 - Celiac Disease Prevalence Data
Prevalence on clinical diagnosis*
Prevalence on screening data
*based on classical, clinical presentation
Fasano & Catassi, Gastroenterology 2001; 120:636-651.
Slide 18 - Celiac Disease Icebergs
photo - bar graph displaying data for Celiac disease icebergs in Ireland, Italy, Netherlands, Sweden and USA
Slide 19 - The Global Village of Celiac Disease
In many areas of the world Celiac Disease is one of the commonest, lifelong disorders affecting around 1% of the general population.
Most cases escape diagnosis and are exposed to the risk of complications.
Active Celiac Disease case-finding is needed but mass screening should be considered.
The impact of Celiac Disease in the developing world needs further evaluation.
Slide 20 - Diagnosis
Confirm diagnosis before treating
- Diagnosis of Celiac Disease mandates a strict gluten-free diet for life
- following the diet is not easy
- QOL implications
- Diagnosis of Celiac Disease mandates a strict gluten-free diet for life
Failure to treat has potential long term adverse health consequences
- increased morbidity and mortality
Slide 21 - Serological Tests
Role of serological tests:
Identify symptomatic individuals who need a biopsy
Screening of asymptomatic “at risk” individuals
Supportive evidence for the diagnosis
Monitoring dietary compliance
Slide 22 - Serological Tests (cont.)
Antigliadin antibodies (AGA)*
*Antiendomysial antibodies (EMA)
*Anti tissue transglutaminase antibodies (TTG)
- first generation (guinea pig protein)
- second generation (human recombinant)
*2004 Consensus Conf. Best tests
Slide 23 - Treatment
Only treatment for celiac disease is a gluten-free diet (GFD)
- Strict, lifelong diet
Slide 24 - Oats –are they Safe?
Studies from 1970’s suggested that oats were toxic in CD
Oats contain a protein-avenin
Avenin- similar to wheat gliadin
Both are prolamins –rich in glutamine and proline, both amino acids
Slide 25 - OATS
Avenin- proportion of proline and glutmaine is very low in oats compared to gliadin in wheat
2004, Random. Clin Trial in children fed GFD vs. GFD with oats Hogberg Gut May 1, 2004 53(5)649-654.
Slide 26 - Findings
First large study to indicate that oats in GFD do not prevent normalization of the small bowel tissue or celiac markers.
Other evidence supporting the safety of oats; G. Kilmartin Gut, January 1, 2003
In CD, oats are not toxic and immunogenic, Srinivasan BMJ 1996:1300-01
Slide 27 - Sources of Gluten
- Pasta / noodles
- Pastries / pies
Slide 28 - Treatment – 6 Elements in RX
Consultation with a skilled dietitian Education about the disease
Lifelong adherence to a gluten-free diet
Identification and treatment of nutritional deficiencies
Access to an advocacy group
Continuous long-term follow-up by a multidisciplinary team
Slide 29 - Barriers to Compliance
Ability to manage emotions – depression, anxiety
Ability to resist temptation – exercising restraint
Feelings of deprivation
Fear generated by inaccurate information
Slide 30 - Factors that Improve Adherence
Internal Adherence Factors Include:
Knowledge about the gluten-free diet
Understanding the risk factors and serious complications can occur to the patient
Ability to break down big changes into smaller steps
Ability to simplify or make behavior routine
Ability to reinforce positive changes internally
Positive coping skills
Ability to recognize and manage mental health issues
Trust in physicians and dietitians
Slide 31 - Histological Features
photo - tissue sections
Horvath K. Recent Advances in Pediatrics, 2002.