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针对乳糜泻患者一级亲属的个体化筛查策略。

Towards an individual screening strategy for first-degree relatives of celiac patients.

机构信息

Department of Pediatrics, Leiden University Medical Center (LUMC) Leiden, Leiden, The Netherlands.

Department of Pediatrics, Rijnstate Hospital Arnhem, Wagnerlaan 55, 6815 AD, Arnhem, The Netherlands.

出版信息

Eur J Pediatr. 2018 Nov;177(11):1585-1592. doi: 10.1007/s00431-018-3199-6. Epub 2018 Jul 4.

Abstract

Celiac disease (CD) is known to be more prevalent in first-degree relatives of patients. In this retrospective cohort study of 609 relatives between 1994 and 2016, we investigated the effect of sex, HLA type, and age at time of index celiac diagnosis. Pearson's chi-square test and Kaplan-Meier survival analysis were used as statistical analyses. CD screening was carried out for 427 relatives (70%), resulting in a prevalence of 15%. HLA typing in 335 relatives showed HLA-DQ2/DQ8 positivity in 87.5%. In 63% of children and all parents, celiac disease was diagnosed at first screening. It was diagnosed significantly more often in females, HLA-DQ2 homozygosity, and children (all p < 0.05). In children aged 0-1 year at time of index diagnosis, celiac disease was diagnosed after consecutive screening in 58%, after 3.9 ± 2.5 (max 10) years (p < 0.001).Conclusion: Future screening policies for relatives of celiac patients should include retesting, especially in HLA-positive relatives younger than 10 years of age. In addition, one-time celiac-specific antibody testing alone could be sufficient to rule out the disease in adolescent siblings and parents of newly diagnosed celiac patients. What is Known: • Celiac disease is more prevalent in first-degree relatives of celiac patients (risk 3-12%). • HLA-DQ2 homozygous sisters/daughters are at highest risk (25%). What is New: • If younger than 10 years of age, repeated testing is necessary in HLA-DQ2/DQ8-positive first-degree relatives when celiac disease is diagnosed in a family. • One-time celiac-specific antibody testing alone could be sufficient to rule out the disease in adolescent siblings and parents of newly diagnosed celiac patients.

摘要

乳糜泻(CD)在患者的一级亲属中更为常见。在这项针对 1994 年至 2016 年间 609 名亲属的回顾性队列研究中,我们研究了性别、HLA 类型和索引乳糜泻诊断时的年龄对其的影响。采用 Pearson 卡方检验和 Kaplan-Meier 生存分析进行统计学分析。对 427 名亲属(70%)进行了 CD 筛查,结果发现患病率为 15%。对 335 名亲属进行的 HLA 分型显示 HLA-DQ2/DQ8 阳性率为 87.5%。在 63%的儿童和所有父母中,乳糜泻在首次筛查时被诊断出。女性、HLA-DQ2 纯合子和儿童的诊断率明显更高(均 P<0.05)。在索引诊断时年龄为 0-1 岁的儿童中,58%在连续筛查后被诊断出乳糜泻,3.9±2.5(最长 10)年后(P<0.001)。结论:未来针对乳糜泻患者亲属的筛查政策应包括重复检测,特别是在 HLA 阳性且年龄小于 10 岁的亲属中。此外,单次乳糜泻特异性抗体检测可能足以排除新诊断为乳糜泻患者的青少年兄弟姐妹和父母的疾病。已知:•乳糜泻在乳糜泻患者的一级亲属中更为常见(风险 3-12%)。•HLA-DQ2 纯合子姐妹/女儿的风险最高(25%)。新发现:•如果年龄小于 10 岁,在家族中诊断出乳糜泻时,HLA-DQ2/DQ8 阳性的一级亲属需要重复检测。•单次乳糜泻特异性抗体检测可能足以排除新诊断为乳糜泻患者的青少年兄弟姐妹和父母的疾病。

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