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与 PANS 症状持续相关的因素:第二部分-临床表现、共病及 IVIG 治疗史。

Factors Associated with Symptom Persistence in PANS: Part II-Presenting Features, Medical Comorbidities, and IVIG Treatment History.

机构信息

Division of Rheumatology, Department of Pediatrics, School of Medicine, Stanford University, Palo Alto, California, USA.

Brain Inflammation Collaborative, Delafield, Wisconsin, USA.

出版信息

J Child Adolesc Psychopharmacol. 2023 Nov;33(9):365-377. doi: 10.1089/cap.2023.0023. Epub 2023 Oct 30.

Abstract

Individuals with Pediatric Acute Onset Neuropsychiatric Syndrome (PANS) experience neuropsychiatric symptoms following an infection or other trigger. Although PANS is typically described as relapsing-remitting, a large community-based 2017 study revealed a range of courses. The present study examined clinical predictors of symptom persistence, measured as , in this same sample. A 146-question online survey gathered histories (infections and other triggers, medical and developmental comorbidities), symptomatology, interventions, and outcomes (including school functioning) of PANS patients. Multivariate analyses were applied to examine associations between these variables and across the disease course. Among the 646 subjects included, significant relationships were found between greater symptom persistence and higher rates of medical comorbidities (especially rashes, headaches, chronic sinusitis, frequent diarrhea, and immune deficiencies), developmental diagnoses, and respondent-perceived developmental lags. Subjects with greater symptom persistence were significantly more likely to report PANS exacerbations associated with infections in close contacts, vaccinations, environmental triggers, and exacerbations of comorbidities and were more likely to report PANS recurrences triggered by Epstein Barr Virus, mycoplasma, and sinus infections. More persistent PANS was also associated with significantly higher frequencies of certain symptoms (sleep disturbance, urinary incontinence, muscle pain, brain fog, sensory defensiveness, irritability, and aggression-related symptoms), less effectiveness of intravenous immunoglobulin in combating symptoms, and more difficulty attending school. Our results suggest high symptom persistence in PANS to be associated with more pervasive medical and neuropsychiatric symptoms. Differences in symptom persistence are associated with both intrinsic (e.g., immune competence) and extrinsic (e.g., infections, treatment) factors. Because extrinsic factors are potentially modifiable, it is critical that providers be aware of current guidelines on PANS evaluation and treatment.

摘要

儿科急性发作的神经精神综合征(PANS)患者在感染或其他诱因后会出现神经精神症状。尽管 PANS 通常被描述为复发缓解型,但 2017 年一项大型基于社区的研究显示了一系列病程。本研究在同一样本中检查了症状持续存在的临床预测因素,以作为衡量标准。一项包含 146 个问题的在线调查收集了 PANS 患者的病史(感染和其他诱因、医疗和发育合并症)、症状、干预措施和结果(包括学校功能)。采用多变量分析来检查这些变量与整个病程中 之间的关联。在包括的 646 名受试者中,发现症状持续存在与更高的医疗合并症发生率(尤其是皮疹、头痛、慢性鼻窦炎、频繁腹泻和免疫缺陷)、发育诊断以及受访者感知到的发育滞后之间存在显著关系。症状持续存在较多的患者更有可能报告与密切接触者感染、疫苗接种、环境触发因素以及合并症恶化相关的 PANS 恶化,也更有可能报告由 Epstein Barr 病毒、支原体和鼻窦感染引发的 PANS 复发。更持久的 PANS 也与某些症状(睡眠障碍、尿失禁、肌肉疼痛、脑雾、感觉防御、易怒和与攻击性相关的症状)的出现频率更高、静脉注射免疫球蛋白对症状的治疗效果更差以及上学困难程度更高相关。我们的研究结果表明,PANS 中症状持续存在与更普遍的医疗和神经精神症状相关。症状持续存在的差异与内在因素(例如免疫能力)和外在因素(例如感染、治疗)都有关。由于外在因素是潜在可改变的,因此提供者了解当前关于 PANS 评估和治疗的指南至关重要。

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