Celiac disease 2020
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This document discusses celiac disease, a genetic autoimmune disorder where the ingestion of gluten leads to damage in the small intestine. It provides details on: - The typical presentation of celiac disease in children, including diarrhea, failure to thrive, and malnutrition. - The diagnostic approach, including testing for anti-tissue transglutaminase antibodies and biopsy showing villus atrophy in the small intestine. - The lifelong treatment of a strict gluten-free diet. Complications can include malnutrition, micronutrient deficiencies, and lymphoma if left untreated.
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Celiac disease 2020
- 1. Celiac Disease Prof. Imran Iqbal Prof of Paediatrics (2003-2018) Prof of Pediatrics Emeritus, CHICH Multan, Pakistan
- 2. In the name of Our Creator Allah, the most Gracious, the most Merciful
- 3. It contains estimated 200 Billion Stars, the sun lies roughly 27,000 light-years from the centre of the galaxy. And there are estimated minimum 100 Billion Galaxies in the Universe.
- 4. Case Scenario • A 3.5 years old child is admitted in MER with H/O loose motions, generalized body swelling, lethargy and poor feeding for last two weeks. There is also H/O diarrhea off and on for last two years. Child is taking animal milk and some solid foods. Patient is vaccinated according to EPI schedule • What is your differential diagnosis ?
- 6. Investigations • Hb 8.4, TLC 9.6, PLT 96000 • S. ALBUMIN 2.6, • Na 129, K 3.0, • RBS 47mg/dl • Ca 8.4, PO4 2.3, Mg 1.4, • Creatinine 0.5, • ALT 275, ALP 225 U/L, PO4 2.3
- 7. Diagnosis • Severe Acute Malnutrition • Kwashiorkor • Malabsorption • ? cause
- 8. Differential Diagnosis • Celiac Disease • Post-enteritis malabsorption • Giardiasis • Tropical Enteropathy (Tropical Sprue) • Lactose intolerence • Cystic fibrosis • AIDS • Protein losing enteropathy • Eosinophilic Gastroenteritis
- 9. Celiac disease Celiac (Greek) = Abdominal
- 10. Celiac Disease • Gluten enteropathy • Autoimmune disorder • Genetically predisposed • 1 in 100 persons • Villus atrophy in small intestine
- 11. Frequency of Celiac Disease in Other Siblings oMonozygotic twin (∼70%) oHLA-matched sibling (∼30%–40%) oFirst-degree relative (∼10%)
- 12. Associated Diseases • Autoimmune thyroiditis (∼15%) • Type I diabetes (≥ 8%) • Selective IgA deficiency (1.7%–7.7%) • Down Syndrome (5%–12%) • Williams Syndrome (8.2%) • Turner Syndrome (4.1%–8.1%)
- 14. Typical Celiac Disease • Age 2 to 10 yrs • Recurrent / chronic Diarrhea • Malabsorption • Abdominal distension • Anemia • Failure to thrive • Poor weight gain • Short stature
- 15. Atypical Celiac Disease • Age more than 10 yrs • Resistant anemia • Short stature • Delayed Puberty • Osteopenia / Osteoporosis • Aphthous stomatitis • Dental Enamel Hypoplasia • Dematitis Herpetiformis • Subfertility in women • Peripheral Neuropathy
- 16. The Celiac Iceberg 16 Symptomatic Celiac Disease Silent Celiac Disease Latent Celiac Disease Genetic susceptibility: - DQ2, DQ8 Positive serology Manifest mucosal lesion Normal Mucosa
- 17. Silent Celiac Disease • No or minimal symptoms, • “ damaged” mucosa • positive serology • Identified by screening asymptomatic individuals from groups at risk : First degree relatives Down syndrome Type 1 diabetes
- 18. Latent Celiac Disease • No or minimal symptoms, • “ normal” mucosa • normal / abnormal serology • May develop clinical disease or abnormal serology later on:
- 20. Diagnosis • Symptoms • Signs on Physical Examination • Serological Antibody tests on Gluten diet – Anti-transglutaminase Ig A - 10 X ULN • HLA detection – DQ2 ,DQ8 • Small Bowel Biopsy – Villus atrophy • Clinical Response to Gluten free diet
- 21. Tissue Transglutaminase • Enzyme in Intestinal Mucosa • Catalyzes metobolism of Gluten peptides • Cross linkage of gliadin peptide to tissue transglutaminase enzyme during these reactions results in formation of new epitopes (antigens) • Stimulation of immune system by these antigens results in formation of Anti-tissue transglutaminase antibodies
- 22. Anti-transglutaminase - Ig A • Most sensitive and specific test • Child should be on Gluten containing diet • Antibody titer correlates with Villus atrophy • > 10 X ULN is taken as positive evidence • Small bowel Histology needed if strong clinical suspicion and Values < 10 X ULN • Antibody titer decreases to normal after strict Gluten free diet
- 23. HLA detection – DQ2 ,DQ8 • Indicates genetic predisposition • Broadly distributed in population • Seen in 95% of Celiac Disease patients • Only 10 % of HLA DQ2 ,DQ8 develop Celiac Disease
- 25. 25 Histological Features Marsh criteria Normal 0 Infiltrative 1 Hyperplastic 2 Partial atrophy 3a Subtotal atrophy 3b Total atrophy 3c Horvath K. Recent Advances in Pediatrics, 2002.
- 26. Villus atrophy – Flat mucosa • Protein Energy Malnutrition • Celiac Disease • Post-enteritis malabsorption • Giardiasis • Tropical Enteropathy (Tropical Sprue) • Lactose intolerence • Cow Milk Protein Allergy • AIDS
- 27. Diagnosis of Celiac Disease • Symptoms and signs • – Anti-tissue-Transglutaminase Ig A - 10 X ULN • Clinical Response to Gluten free diet
- 28. Management • Gluten free diet (avoid Wheat, Barley) • Vitamin A • Vitamin D • Micronutrients • Iron therapy (Oral / IV)
- 31. Gluten free Foods • Cereals - Rice, Corn, Millet • Meat • Egg • Milk • Lentils • Fruits • Vegetables
- 33. Complications • Malnutrition • Severe Acute Malnutrition • Micronutrient deficiencies • Celiac crisis • Non-Hodgkin Lymphoma
- 34. Celiac crisis Acute onset or rapid progression of gastrointestinal symptoms attributable to Celiac disease requiring hospitalization and/or parenteral nutrition
- 35. Complications in Celiac crisis • Severe dehydration • Neurologic dysfunction • Renal dysfunction: creatinine >2.0 mg/d • Metabolic acidosis: pH <7.35 • Hypoproteinemia (Albumin < 3.0 g/dL) • Electrolyte abnormalities: • Hyponatremia, • Hypocalcemia, • Hypokalemia • Hypomagnesemia
- 36. Management of Celiac crisis • Hypoglycemia - 10% D/W 5ml/ kg • Dehydration - Ringer Lactate + KCL 4ml/100ml • Ca-gluconate in infusion 1 – 2 ml/kg • MgSO4 (IV/IM: 25-50 mg/kg q6hr for 1 day • Antibiotics • Blood packed cells transfusion if needed • Salt free albumin or FFP (10ml/kg) • Steroids: Hydrocortisone 5mg/kg/dose 6 hourly • Micronutrients: Vit-K (5mg ), Vit-A (2 lac IU), Vit-D (2 lac IU), Folic Acid (5mg OD) and Zinc (20mg OD) • Gluten free diet
- 37. Medical Ethics