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- W4224988780 abstract "Ultra-short coeliac disease (USCD) is a novel celiac disease (CD) subtype limited to the duodenal bulb (D1). HLA haplotypes and flow cytometry have not been assessed yet.To compare genetic, clinical, serologic, histopathologic and inmmunophenotypic parameters between USCD and conventional celiac disease (CCD) patients.Prospective single-center study in children and adult patients undergoing duodenal biopsies on a gluten-containing diet. Biopsies for histology and flow cytometry were taken separately from D1 and distal duodenum. Biopsies in seronegative patients with celiac lymphogram were repeated after 2 years on a gluten-free diet.Among 505 included patients, 127 were diagnosed with CD, of whom 7 (5.5%) showed USCD. HLADQ2 was significantly less common in USCD compared to CCD (71% vs. 95%, p 0.003). Likewise, USCD patients showed more frequent non-significant seronegativity (28% vs. 8%, p 0.07) and significantly lower titrations (7–15 IU/ml) of tissue transglutaminase antibodies (tTG-IgA) (60% vs. 13%, p < 0.001). Biopsies from D1 revealed significant less NK cells down-expression in USCD patients (1.4 vs. 5, p 0.04).Up to 5.5% of CD patients showed USCD. A lower frequency of HLADQ2, along with less serum tTG-IgA titration and duodenal NK cell suppression, were differential features of USCD.La enfermedad celiaca ultracorta (ECUC) es un nuevo fenotipo de la enfermedad celiaca, que afecta exclusivamente al bulbo duodenal (D1), cuyas características genéticas e inmunológicas no han sido descritas.Comparar las características genéticas, clínicas, serológicas, histológicas e inmunológicas entre ECUC y enfermedad celiaca convencional (ECC).Estudio prospectivo, unicéntrico, en el que se realizaron biopsias duodenales a pacientes adultos y pediátricos que seguían una dieta con gluten. Se realizó análisis histológico y por citometría de flujo por separado de duodeno distal y D1. En pacientes seronegativos con histología o linfograma concordante con EC, se repitió la biopsia tras 2 años con dieta sin gluten.Se incluyeron 505 pacientes, siendo diagnosticados 127 de enfermedad celiaca, de los cuales 7 (5,5%) tenían ECUC. Los pacientes con ECUC expresaron el haplotipo DQ2 con menor frecuencia que en la ECC (71% vs. 95%, p 0,003) y presentaron mayor seronegatividad (28% vs. 8%, p 0,07) y, de manera significativa, titulaciones bajas de anticuerpos antitransglutaminasa (7-15 IU/ml) (60% vs. 13%, p < 0,001). Comparado con la ECC, la citometría de flujo en las biopsias bulbares reveló una reducción atenuada significativa de células NK (1,4 vs. 5, p 0,04) en pacientes con ECUC.Un 5,5% de los pacientes celiacos presentaron ECUC. Comparada con la ECC, las características diferenciales de la ECUC son menor expresión de HLADQ2, títulos más bajos de anticuerpos antitranglutaminasa y menor supresión de células NK a nivel bulbar." @default.
- W4224988780 created "2022-04-28" @default.
- W4224988780 date "2022-04-01" @default.
- W4224988780 modified "2023-09-25" @default.
- W4224988780 title "TOPICS" @default.
- W4224988780 doi "https://doi.org/10.1053/s0146-0005(22)00032-5" @default.
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