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- W2034762579 abstract "Aims: Coeliac disease (CD) occurs in approx. 10-15 % of first-degree relatives. Manifestation of the disease is restricted to HLA-DQ2 or DQ8-positive individuals, but the exact mode of inheritance is unclear and certain pedigrees show that CD is not co-inherited with HLA-DQ allels. In this study, we investigated the occurrence of CD in the offsprings of the patients. Methods: Patients diagnosed with CD in childhood by Interlaken or by current ESPGHAN criteria were prospectively followed up and their children were screened using IgA and IgG endomysial (EMA) and transglutaminase antibodies. Seropositive subjects were referred for jejunal biopsy. Offsprings positive for EMA and/or with biopsy-proven CD were regarded as affected. The results were compared with the evaluation of children of CD patients diagnosed as adults. Children first evaluated below the age of six years or on a gluten-restricted diet were yearly re- screened. Results: Altogether 481 offsprings (228 males) of 270 CD parents were studied with a median number of 2 children per parent and at a median age of 7.3 years. Percentage of affected offsprings varied according to the mode the coeliac parent was initially diagnosed (table), and was significantly higher already at an earlier median age (4.0 years) for childhood-diagnosed CD parents than for adult-diagnosed CD parents at a median child age of 7.7 years. HLA-DQ typing was performed in 85 offsprings. All affected and 76% of not affected offsprings carried DQ2 or DQ8.TABLESummary: Occurrence rate of CD was 38% in the second generation which seems to be much higher than for first-degree relatives in general. Conclusions: Our data suggest that nearly half of HLA-DQ2 or DQ8-positive offsprings were affected raising the possibility that dominantly inherited gene(s) might be operative at least in certain subsets of CD patients. These data also warrant the careful screening of offsprings." @default.
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- W2034762579 date "2005-05-01" @default.
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- W2034762579 title "IS COELIAC DISEASE A DOMINANTLY INHERITED DISORDER?" @default.
- W2034762579 doi "https://doi.org/10.1097/00005176-200505000-00084" @default.
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