Name
CELIAC DISEASE
DESCRIPTION
DETAIL
D.D. : 1. INADEQUATE DIGESTION - POSTGASTRECTOMY - DEFICIENCY OR INACTIVATION OF PANCREATIC LIPASE - EXOCRINE PANCREATIC INSUFFICIENCY - CRO PANCREATITIS - PANCREATIC CARCINOMA - CYSTIC FIBROSIS - PANCREATIC INSUFFICIENCY- CONGENITAL OR ACQUIRED - GASTRINOMA - ACID INACTIVATION OF LIPASE - DRUGS LIKE ORLISTAT 2. REDUCED INTRADUODENAL BILE ACID CONCENTRATION / IMPAIRED MICELLE FORMATION -* LIVER DISEASES - PARENCHYMAL LIVER DISEASE - CHOLESTATIC LIVER DISEASE * BACTERIAL OVERGROUTH IN SMALL INTESTINE - ANATOMIC STASIS LIKE AFFERENT LOOP STASIS, BLIND LOOP STASIS, STRICTURE , FISTULA - FUNCTIONAL STASIS LIKE DIABETES , SCLERODERMA, INTESTINAL PSEUDOOBSTRUCTION * INTERRUPTED INTEROHEPATIC CIRCULATION OF BILE SALTS - ILEAL RESECTION - CROHNS DISEASE * DRUGS WHICH BIND OR PRECIPITATE BILE SALTS LIKE NEOMYCIN, CHOLESTYRAMINE, CAL CARBONATE 3. IMPAIRED MUCOSAL ABSORPTION / MUCOSAL LOSS OR DEFECT * INTESTINAL RESECTION OR BYPASS * INFLAMMATION, INFILTRATION OR INFECTION - CROHNS DISEASE - AMYLOIDOSIS - SCLERODERMA - LYMPHOMA - EOSINOPHILIC ENTERITIS - MASTOCYTOSIS - TROPICAL SPRUE - CELIAC DISEASE - COLLAGENOUS SPRUE - WIPPLES DISEASE - RADIATION ENTERITIS - FOLATE & VIT B12 DEFICIENCY - INFECTIONS LIKE SALMONELLA, GIARDIASIS - GRAFT VS HOST DISEASE * GENETIC DISEASES - DISACCHARIDASE DEFICIENCY - AGAMMAGLOBULINEMIA - ABETALIPOPROTEINEMIA - HARTNUP DISEASE - CYSTINURIA 4. IMPAIRED NUTRIENT DELIVERY TO AND / OR FROM INTESTINE * LYMPHATIC OBSTRUCTION - LYMPHOMA - LYMPHANGIECTASIA * CIRCULATORY DISORDERS - CONGESTIVE HEART FAILURE - CONSTRICTIVE PERICARDITIS - MESENTERIC ARTRY ATHEROSCLEROSIS - VASCULITIS 5. ENDOCRINE & METABOLIC DISORDERS - DIABETES - HYPOPARATHYROIDISM - ADRENAL INSUFFICIENCY - HYPERTHYROIDISM - CARCINOID SYNDROMEOTHER TESTS : * SMALL INTESTINAL BIOPSY * TRIAL OF GLUTEN FREE DIET β’ Positive anti-gliadin IgA and IgG β’ Positive anti-endomysial antibodies and IgA tissue transglutaminase β’ IgA deficient patients have false-negative IgA antiendomysial and IgA antitransglutaminase antibodies β’ 72 hour fecal fat showing greater than 7% fat malabsorption β’ D-Xylose test showing malabsorption of this sugar β’ Decreased calcium β’ Decreased prothrombin time β’ Decreased neutral fats β’ Decreased cholesterol β’ Decreased vitamin A β’ Decreased vitamin B12 (rare) β’ Decreased vitamin D β’ Decreased vitamin C β’ Decreased folic acid β’ Decreased iron β’ Decreased total protein β’ Anemia
TYPENOTES
RISK FACTORS β’ First order relatives - 10% incidence β’ 71% in monozygotic twinsGENERAL MEASURES: Removal of gluten from the diet. Rice, corn and soybean fl our are safe, palatable substitutes. Levels of IgA antigliadin normalize with gluten abstinence. DIET Removal of gluten - wheat, rye, barley and those with gluten additives DRUG(S) OF CHOICE: * Usually none * Prednisone, 40-60 mg/day po in cases of refractory sprue * Refractory disease : . Steroids . Azathioprine . Cyclosporine . Possible future role of infliximab (Remicade) ALTERNATIVE DRUGS : May require supplemental calcium, calcium carbonate, 500 mg po bid, and vitamin D (ergocalciferol) 10-100 mcg/day; in severe malabsorption, up to 2.5 mg/day may be required PATIENT MONITORING : Repeat endoscopy after 6-8 weeks on a gluten-free diet (in selected cases) PREVENTION/AVOIDANCE : Avoid all gluten containing products POSSIBLE COMPLICATIONS : β’ Malignancy - less than 10% of patients (50% of which are small bowel lymphoma) β’ Refractory sprue - may respond to prednisone 40-60 mg/day po. Refractory sprue unresponsive to corticosteroid therapy raises the specter of adult-onset autoimmune enteropathy or cryptic T-cell lymphoma. In this circumstance, screening for antienterocyte autoantibodies and careful scrutiny of the small intestine, including retroperitoneal lymph node biopsy with full thickness small bowel biopsy may be needed. β’ Chronic ulcerative jejunoileitis - associated with multiple ulcers, intestinal bleeding, strictures, perforation, obstruction, peritonitis - 7% mortality β’ Osteoporosis secondary to decreased vitamin D and calcium absorption β’ Dehydration β’ Electrolyte depletion β’ Refractory cases may need total parenteral nutrition β’ Death (rare) EXPECTED COURSE/PROGNOSIS : Good with correct diagnosis and adherence to gluten free diet. Feel better in seven days. All symptoms usually disappear in four to six weeks. It is unknown whether strict dietary adherence decreases cancer risk. AGE-RELATED FACTORS: Pediatric: Children reaching adolescence may outgrow intolerance to wheat but should be cautioned to watch for signs of recurrence in middle age
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
SERUM PROTEIN TOTAL, COMPLETE BLOOD COUNT, SERUM FOLATE, SERUM IRON SATURATION ( SERUM TRANSFERRIN SATU. ), SCHILLING TEST, URINARY D-XYLOSE TEST, STOOL FAT CONTENT 24 HRS, BIOPSY, D-XYLOSE ABSORPTION