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WASN Online Lecture Series - 2015 WASN Spring Conference 3. Review of Celiac Disease and in Children

Objectives

Encopresis, Constipation • The learner should be able to identify the and Celiac Disease. causes of encopresis in children. • The learner should be able to discuss the strategies for treating encopresis in children. Victor Uko MD FAAP DCH (UK) MRCPCH (UK) • The learner should be able to define celiac Pediatric Gastroenterologist disease. Gundersen Health System • The learner should be able to discuss the April 23rd , 2015 prevalence of celiac disease in the population and amongst relatives

Definition and Prevalence of Constipation • Defined as a delay or difficulty in that is present for 2 weeks or more and sufficient to cause significant distress to the patient Constipation and Encopresis • Relatively common condition • 3% of general pediatric office visits in the US • 25% of pediatric clinic visits in the US • 95% of cases are functional (no underlying cause).

Definition and Classification Prevalence of Encopresis Encopresis • Passage of stool in the underpants typically in • Affects 3% of 4 year olds and 1.6% of 10 a child that has already been toilet trained year olds (>4yrs) • Most commonly affects 5-10 year olds • May be voluntary (most often behavioral) or • Median age based on two large studies was involuntary 7-9 years • Introduced as a term in 1926 by Weissenberg • Rarely affects adolescents • 2 broad classifications: • Affects more boys than girls (M:F = 3-6:1) - Functional Encopresis (90% of cases) - Organic Encopresis - due to a defined disease process. (Anatomical, neurologic, metabolic)

1 WASN Online Lecture Series - 2015 WASN Spring Conference 3. Review of Celiac Disease and Encopresis in Children

Physiology of Normal Defecation Causes of Encopresis

Stool enters the • Functional Encopresis - Occurs in 90% of cases Internal anal sphincter relaxes - No identifiable disease process • Organic Stool enters the - Uncommon reason for encopresis - There is often a clearly recognized External anal sphincter underlying condition (under voluntary control) - The causes may be anatomical, neurologic, or metabolic

Functional Encopresis Functional Encopresis

• Seen in 90% of all cases of chronic encopresis • 1/3rd of parents studied believed that their child had an organic or emotional problem • Most children with this withhold stool and this leads to chronic stretching of the rectal • Other common perceptions: child is careless, walls attention-seeking, stubborn and lazy • May be triggered by an event: • Increased risk for enuresis (soiling with urine) in up to 40% of affected patients - Passage of painful stool • Increased risk for urinary tract infections - Unsubstantiated fears especially in girls - Difficulties with toilet training - Issues with the use of public toilets (e.g. school)

Evaluation of Patients with Organic Encopresis Encopresis • 5-10% of all cases of chronic encopresis • Good clinical history and physical examination • Anatomic • Plain abdominal radiograph - Determine the degree of stool impaction in the - , ectopic anus or anal colon • - Prior bowel surgery - Screen for Hirschprung’s disease and voiding • Neurologic abnormalities like dyssynergic defecation - Hirschprung's disease, Spinal cord damage • Rectal mucosal biopsy - Confirm Hirschprung’s disease or anal • Metabolic achalasia - • Blood tests to evaluate for , underlying abnormalities and celiac disease

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Plain Abdominal X ray Anorectal Manometry

Treatment of Encopresis Prognosis of Encopresis • Fecal disimpaction - Oral (stimulants and osmotic laxatives) • Prolonged and consistent treatment is often required - • Maintenance Therapy • Frequent relapses occur - Daily laxatives • 10 year follow up study of patients with - High fiber diet (age in years + 5 = daily revealed: requirement) - 46% remained constipated • Behavioral modification - 25% continued to have encopresis - Avoid withholding stool - 56% still had recurrent - Scheduled bowel habits: Sit on the toilet for 10 minutes or 1 minute per year of life after meals (2-3 times a day).

Summary of Encopresis • Functional constipation is the most common cause for encopresis • More common in boys than girls • Often initiated by a precipitating event such as painful stools • Enuresis (soiling with urine) and Urinary tract infections (UTI) are frequent associations Celiac Disease • Children and families often feel isolated and ostracized • Treatment involves: laxatives, behavioral modification and counseling when deemed appropriate • Treatment is a long term process with frequent relapses

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Definition of Celiac Disease Epidemiology of Celiac Disease • Females more affected than males • An immune mediated (disorder • Affects ~1% in North America and Europe of the intestine). • Affects other ethnic populations including: • There is a permanent sensitivity to gluten • Middle East • Affects genetically susceptible individuals - Iran: prevalence amongst 2,000 blood donors was 1:166 • Patients may be fall into one of the following • North Africa groups: - Saharawis (1 in 18 children affected) • Symptomatic • Asia - Gastrointestinal - common cause of chronic in - Non gastrointestinal children and adults in India • Asymptomatic • South America

Pathogenesis of Celiac Disease

• Genetic • Environmental • Dietary Pathogenesis - Gluten (present in wheat, rye and barley) - α- gliadin (main toxic component of Gluten) • Immune responses - Humoral - T cell - Mucosal

Pathogenesis of Celiac Disease

Clinical Presentation

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Clinical Presentation of Celiac Gastrointestinal Manifestations Disease of Celiac Disease • Gastrointestinal (“classical”) • Most common age of presentation: 6-24 months • Non-gastrointestinal (“atypical”) - Chronic or recurrent diarrhea - Abdominal pain and distension • Asymptomatic - Anorexia - Failure to thrive or weight loss - - Constipation - Irritability

Malnourished patients with Celiac Disease Non Gastrointestinal Manifestations Most common age of presentation: older child to adult • Dermatitis Herpetiformis • Dental enamel hypoplasiaof permanent teeth • Osteopenia/Osteoporosis • Short Stature • Delayed Puberty/Menarche • Iron-deficient anemia resistant to oral Fe • • Arthritis • Epilepsy with occipital calcifications

Image courtesy of CDHNF/NASPGHAN

Dermatitis Herpetiformis in Celiac Disease Dental Enamel Defects in Celiac Disease

• Erythematous macule > urticarial papule > tense vesicles • Severe pruritus • Symmetric distribution • 90% no GI symptoms • 75% villous atrophy • Gluten sensitive

Image courtesy of CDHNF/NASPGHAN Image courtesy of CDHNF/NASPGHAN

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Osteoporosis in Celiac Disease Short Stature and Delayed Puberty in Celiac Disease • Short stature occurs in children and teens - ~10% of short children and teens have evidence of celiac disease • Delayed menarche - High prevalence in teens with untreated Celiac Disease

Low bone mineral density improves in children on a gluten-free diet

Image courtesy of CDHNF/NASPGHAN

Iron Deficiency Anemia in Celiac Disease Asymptomatic Celiac Disease • The most common non-gastrointestinal finding in some adult studies of patients with celiac Silent Latent disease • Most patients would not respond to oral iron • Silent: Patient has no or minimal symptoms, alone without treating the celiac disease. “damaged” mucosa and positive serology • 5-8% of adults with unexplained iron deficiency anemia have celiac disease • Identified by screening asymptomatic • In children with newly diagnosed Celiac individuals from groups at risk such: Disease: • First degree relatives - Anemia is common • patients - Little evidence that celiac disease is • Type 1 patients, etc. common in children presenting with anemia

Asymptomatic Celiac Disease Disorders Associated with Celiac Disease Silent Latent • The prevalence of Celiac Disease is higher in patients who have the following: • Latent: Patient has no symptoms, normal small • Certain genetic disorders or syndromes intestinal mucosa - Downs (4-19%) • Patients may have positive serology (blood tests) - Turners (4-8%) • Identified by following in time asymptomatic • Other autoimmune conditions individuals previously identified at screening from groups at risk - Type I Diabetes (3.5 - 10%) • These individuals, given the “right” circumstances, - Auto immune thyroiditis (4-8%) will develop at some point in time mucosal • 1st degree relatives (~5%) changes (± symptoms)

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DiagnosisDiagnosis

• Diagnosis of Celiac Disease requires: - Characteristic small intestinal DiagnosisDiagnosis histology in a symptomatic child - Complete symptom resolution on gluten-free diet • Serological (blood tests) may support diagnosis

Serological Tests Serological Test Comparison

• Antigliadin antibodies (AGA) Sensitivity % Specificity % • Antiendomysial antibodies (EMA) AGA-IgG 69 – 85 73 – 90 AGA-IgA 75 – 90 82 – 95 • Anti tissue transglutaminase antibodies (TTG) EMA (IgA) 85 – 98 97 – 100 *TTG (IgA) 90 – 98 94 – 97

Histology in Celiac Disease

Endoscopic Findings

Normal Appearing Scalloping Nodularity

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Treatment of Celiac Disease • Only treatment for celiac disease is a gluten-free diet (GFD) • Advisable to see a dietitian TreatmentTreatment knowledgeable in Celiac Disease - Strict, lifelong diet - Avoid: • Wheat • Rye • Barley

Gluten-Containing Grains to Avoid Sources of Gluten

Wheat Bulgar Filler • OBVIOUS SOURCES - Bread Wheat Bran Couscous Graham flour - Bagels Wheat Starch Durum Kamut - Cakes Wheat Germ Einkorn Matzo - Cereal Flour/Meal Barley Emmer - Cookies Semolina Barley Malt/Extract Faro - Pasta / noodles Spelt Rye Triticale - Pastries / pies - Rolls

Sources of Gluten Ingredients to Question (may contain gluten) • POTENTIAL SOURCES – Candy • Seasonings and spice – Communion wafers blends or mixes – Cured Pork Products • Modified food starch – mixes • Malt/ malt extract/ flavoring –Gravy • Modified hop extract and – Imitation meat / seafood yeast-malt sprout extract – Sauce • Dextrin – Self-basting turkeys • Caramel color – Soy sauce

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Other Potential Sources of Gluten Gluten-Free Grains and Starches Contamination

• Lipstick/Gloss/Balms • Amaranth •Potato • Arrowroot • Quinoa • Mouthwash/Toothpaste • Buckwheat •Rice • Play Dough •Corn • Sorghum •Flax • Tapioca • Stamp and Envelope Glues • Millet •Teff • Montina • Flours made from nuts, • Vitamin, Herbal, and •Oats* beans and seeds Mineral preparations *for possible cross-contamination with gluten containing grains • Prescription or OTC

Some Complications of Celiac Disease • Short stature • Nutritional Deficiencies • Osteoporosis and bone fractures Complications • Infertility • Gluten ataxia and other neurological disturbances • Intestinal lymphoma

Celiac Disease Complicated by CT Scan Showing Occipital Enteropathy-Associated T-cell Lymphoma Calcifications in Celiac Disease (EATL)

By permission of G. Holmes, Derby (UK)

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Non Celiac Gluten Sensitivity Summary of Celiac Disease • Celiac disease is an immune disorder that occurs in genetically predisposed individuals • Presence of clinical symptoms that overlap • Patients have a permanent and life long with celiac disease and wheat allergy sensitivity to gluten • Negative immune allergen test to wheat • Symptoms may be gastrointestinal or non • Negative celiac disease serology and normal gastrointestinal or asymptomatic (silent) duodenal histology • Diagnosis is made by a combination of • Resolution of symptoms on gluten free diet endoscopy, serology and resolution of (double blind) symptoms on gluten free diet • Major complications occur if untreated • 1st degree relatives and other high risk groups should be screened for the disease • Treatment is a strict gluten free diet

References • Baker SS, et al. Constipation in infants and children: evaluation and treatment. J Pediatr Gastroenterol Nutr 1999;29:612–626 • Fasano, et al. Arch of Intern Med, Volume, 2003; 163: 286-292 Thank youThank you • Hill et al. Guideline for the Diagnosis and Treatment of Celiac Disease in Children: Recommendations of the North American Society for Pediatric Gastroenterology, and Nutrition. J Pediatr Gastroenterol Nutr 1999 2005; 40:1–19 • Sapone et al BMC Medicine 2012 10:13 • Green PH et al Celiac Disease N Engl J Med. 2007;357:1731-43

References • Farrell RJ, and Kelly CP. Am J Gastroenterol 2001;96:3237-46. • ESPGAN working group. Arch Dis Child 1990;65:909 • Garioch JJ, et al. Br J Dermatol. 1994;131:822-6. • Fry L. Baillieres Clin Gastroenterol. 1995;9:371-93. • Reunala T, et al. Br J Dermatol. 1997;136-315-8.

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