The risk of celiac disease in 107 families with at least two affected siblings.
Journal article, 2004
OBJECTIVES: Screening for celiac disease (CD) in the apparently healthy members of 107 nuclear families with two affected siblings (sib pair) and estimating the risk of CD in siblings and parents. METHODS: One hundred seven families from Sweden and southern Norway with at least two affected children were investigated. Frozen sera from 187 of the 192 healthy parents and from 94 of 95 siblings without diagnosed CD were examined for total immunoglobulin A (IgA) and anti-endomysial antibodies (EMA). Individuals with positive antibody titers underwent small intestinal biopsy. RESULTS: Positive test for EMA was found in 6 of 94 (6.3%) siblings without previously diagnosed CD and in 8 of 189 (4.2%) parents. CD was confirmed by small intestinal biopsy in all siblings and seven parents. The estimated risk for CD in multiply affected families was 26.3% for siblings and 12.9% for parents. An unexpected male preponderance was found among the new CD cases (10 males, 3 females). CONCLUSION: The risk of CD in the members of nuclear families with two affected children is approximately three times higher than that when only one child is affected. The high male preponderance of new cases is unexpected and could not be explained fully by more silent disease in males as compared with females. Considering the high level of knowledge about CD in these families, the number of undiagnosed cases is surprisingly high. The authors suggest that serologic screening should be offered to all first-degree relatives of patients with CD.
Adult
genetics
Adolescent
Norway
Sweden
Male
Autoantibodies
Sex Factors
epidemiology
Preschool
Middle Aged
Humans
blood
Aged
blood
Pedigree
Celiac Disease
Child
Risk Factors
Risk Assessment
Biological Markers
epidemiology
Infant
analysis
Child
Prevalence
Genetic Predisposition to Disease
blood
epidemiology
Female
Author
Audur Gudjonsdottir
University of Gothenburg
Staffan Nilsson
Chalmers, Department of Mathematical Statistics
University of Gothenburg
Johan Ek
Bengt Kristiansson
University of Gothenburg
Henry Ascher
University of Gothenburg
Journal of Pediatric Gastroenterology and Nutrition
0277-2116 (ISSN) 1536-4801 (eISSN)
Vol. 38 3 338-42Subject Categories
MEDICAL AND HEALTH SCIENCES
PubMed
15076637