Biomaterial Database

Prevalence of celiac disease in Down syndrome in the United States. 2000

D A Zachor, and E Mroczek-Musulman, and P Brown

Department of Pediatrics, University of Alabama at Birmingham, USA.

BACKGROUND Numerous studies in Europe have documented a high prevalence of celiac disease in Down syndrome. This study was undertaken to estimate the prevalence of celiac disease in Down syndrome in the southeastern United States. METHODS Seventy-five patients with Down syndrome were screened using immunoglobulin (Ig)A-anti antiendomysium antibodies, IgA-antigliadin antibodies, and total IgA level. When either antiendomysium or antigliadin antibodies produced positive findings, patients were referred to a pediatric gastroenterologist for consideration of a duodenal biopsy. RESULTS Thirteen percent (10/75) were positive for antiendomysium antibodies. Half of these patients were also positive for antigliadin antibodies. Six of 10 patients positive for antiendomysium antibodies underwent intestinal biopsy. Changes consistent with celiac disease were documented in five. Histologic findings ranged from focal to total villous atrophy. None had IgA deficiency. CONCLUSIONS There was a high prevalence of positivity to antiendomysium antibody in Down syndrome. Antiendomysium antibody was a more sensitive screening test than antigliadin antibody. The prevalence of celiac disease in Down syndrome in the southeastern United States was 1 in 14 cases. Screening with antiendomysium antibody and IgA for all children with Down syndrome is recommended, even if there are no gastrointestinal symptoms.

UI	MeSH Term	Description	Entries
D007070	Immunoglobulin A	Represents 15-20% of the human serum immunoglobulins, mostly as the 4-chain polymer in humans or dimer in other mammals. Secretory IgA (IMMUNOGLOBULIN A, SECRETORY) is the main immunoglobulin in secretions.	IgA,IgA Antibody,IgA1,IgA2,Antibody, IgA
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D009210	Myofibrils	The long cylindrical contractile organelles of STRIATED MUSCLE cells composed of ACTIN FILAMENTS; MYOSIN filaments; and other proteins organized in arrays of repeating units called SARCOMERES .	Myofilaments,Myofibril,Myofilament
D002446	Celiac Disease	A malabsorption syndrome that is precipitated by the ingestion of foods containing GLUTEN, such as wheat, rye, and barley. It is characterized by INFLAMMATION of the SMALL INTESTINE, loss of MICROVILLI structure, failed INTESTINAL ABSORPTION, and MALNUTRITION.	Gluten Enteropathy,Sprue, Celiac,Sprue, Nontropical,Celiac Sprue,Gluten-Sensitive Enteropathy,Sprue,Disease, Celiac,Enteropathies, Gluten,Enteropathies, Gluten-Sensitive,Enteropathy, Gluten,Enteropathy, Gluten-Sensitive,Gluten Enteropathies,Gluten Sensitive Enteropathy,Gluten-Sensitive Enteropathies,Nontropical Sprue
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D004314	Down Syndrome	A chromosome disorder associated either with an extra CHROMOSOME 21 or an effective TRISOMY for chromosome 21. Clinical manifestations include HYPOTONIA, short stature, BRACHYCEPHALY, upslanting palpebral fissures, epicanthus, Brushfield spots on the iris, protruding tongue, small ears, short, broad hands, fifth finger clinodactyly, single transverse palmar crease, and moderate to severe INTELLECTUAL DISABILITY. Cardiac and gastrointestinal malformations, a marked increase in the incidence of LEUKEMIA, and the early onset of ALZHEIMER DISEASE are also associated with this condition. Pathologic features include the development of NEUROFIBRILLARY TANGLES in neurons and the deposition of AMYLOID BETA-PROTEIN, similar to the pathology of ALZHEIMER DISEASE. (Menkes, Textbook of Child Neurology, 5th ed, p213)	Mongolism,Trisomy 21,47,XX,+21,47,XY,+21,Down Syndrome, Partial Trisomy 21,Down's Syndrome,Partial Trisomy 21 Down Syndrome,Trisomy 21, Meiotic Nondisjunction,Trisomy 21, Mitotic Nondisjunction,Trisomy G,Downs Syndrome,Syndrome, Down,Syndrome, Down's
D004386	Duodenum	The shortest and widest portion of the SMALL INTESTINE adjacent to the PYLORUS of the STOMACH. It is named for having the length equal to about the width of 12 fingers.	Duodenums
D005260	Female		Females