IF 2.3 4区 医学 Q2 DEVELOPMENTAL BIOLOGY
Angela W Tang, Paula M Prieto Jimenez, Ian K T Miller, Juliette C Madan, Jaden Nguyen, Meiqian Ma, Melissa Silverman, Bahare Farhadian, Jenny Wilson, Alka Goyal, Cindy Manko, Yinka Davies, Shervin Rabizadeh, Jennifer Frankovich
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引用次数: 0

摘要

简介小儿急发性神经精神综合征(PANS)是一种免疫介导的疾病,其特征是突发性神经行为改变。炎症性肠病(IBD)包括溃疡性结肠炎(UC)和克罗恩病(CD),是以胃肠道炎症为特征的慢性疾病。我们描述了八名同时患有 PANS 和 IBD 的患者:所有同时患有 IBD 和 PANS 的患者均来自斯坦福大学免疫行为健康诊所、西达-西奈医学中心小儿炎症性肠病项目和达特茅斯神经免疫精神障碍(NIPD)诊所。通过病历审查收集数据:结果:共发现 8 例患有 IBD 的 PANS 患者。其中五例为男性。PANS患者的平均发病年龄为9.3岁,IBD患者的平均发病年龄为15.6岁。在 8 个病例中,7 个病例的 PANS 比 IBD 的发病时间平均早 8.4 年。七名患者(88%)的一级亲属患有免疫介导疾病,其中五人患有银屑病或银屑病关节炎。5名患者本身患有关节痛或关节炎(63%)。PANS早于IBD治疗的5个病例在IBD得到控制后都没有出现严重的行为复发:结论:PANS、关节主诉和自身免疫家族史(包括银屑病)这三者可能代表了 PANS 的一个亚群,他们罹患 IBD 和其他免疫介导疾病的风险较高。对于有这三联征的儿童,临床医生应降低门槛,在有指征时使用血红蛋白、CRP、粪便钙蛋白等生物标记物和诊断性内镜检查来评估胃肠道炎症。PANS症状可能会随着IBD的有效治疗而得到改善。在我们的队列中,关节不适的发病率很高,一级家族成员中也有银屑病患者,这表明这一 PANS 亚群可能与银屑病和关节炎有着相同的免疫机制。应研究用于 IBD 和关节炎的治疗策略在 PANS 中的潜在应用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Eight cases of Pediatric Acute Onset Neuropsychiatric Syndrome (PANS) with Inflammatory Bowel Disease (IBD): Immunologic Intersections.

Introduction: Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS) is an immune-mediated disease characterized by abrupt onset neurobehavioral changes. Inflammatory bowel disease (IBD) includes ulcerative colitis (UC) and Crohn's disease (CD), chronic conditions characterized by gastrointestinal inflammation. We describe eight individuals with both PANS and IBD.

Methods: All individuals with both IBD and PANS were identified from Stanford Immune Behavioral Health Clinic, Cedars-Sinai Medical Center Pediatric Inflammatory Bowel Disease Program, and Dartmouth Neuroimmune Psychiatric Disorders (NIPD) Clinic. Data was collected by chart review.

Results: Eight cases of PANS with IBD were identified. Five were male. Mean age of onset was 9.3 years for PANS and 15.6 years for IBD. PANS preceded development of IBD in 7 of 8 cases by a mean of 8.4 years. Seven patients (88%) had a first-degree relative with an immune-mediated disease, including 5 with psoriasis or psoriatic arthritis. Five patients themselves had arthralgias or arthritis (63%). All of the 5 cases where PANS preceded IBD treatment sufficiently for analysis were free of major behavioral relapses after IBD was managed.

Conclusion: The triad of PANS, joint complaints, and family history of autoimmunity, including psoriasis, may represent a subset of PANS at heightened risk for IBD and additional immune-mediated disorders. For children with this triad, clinicians should have a low threshold to evaluate for gastrointestinal inflammation with biomarkers like hemoglobin, CRP, fecal calprotectin and diagnostic endoscopy when indicated. PANS symptoms may improve with effective treatment of IBD. The high prevalence of joint complaints in our cohort and psoriasis in first-degree family members suggests this subset of PANS may share immune mechanisms with psoriasis and arthritis. Treatment strategies used in IBD and arthritis should be studied for potential application in PANS.

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来源期刊
Developmental Neuroscience
Developmental Neuroscience 医学-发育生物学
CiteScore
4.00
自引率
3.40%
发文量
49
审稿时长
>12 weeks
期刊介绍: ''Developmental Neuroscience'' is a multidisciplinary journal publishing papers covering all stages of invertebrate, vertebrate and human brain development. Emphasis is placed on publishing fundamental as well as translational studies that contribute to our understanding of mechanisms of normal development as well as genetic and environmental causes of abnormal brain development. The journal thus provides valuable information for both physicians and biologists. To meet the rapidly expanding information needs of its readers, the journal combines original papers that report on progress and advances in developmental neuroscience with concise mini-reviews that provide a timely overview of key topics, new insights and ongoing controversies. The editorial standards of ''Developmental Neuroscience'' are high. We are committed to publishing only high quality, complete papers that make significant contributions to the field.
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