留给他们自己：是时候针对儿童罕见病的慢性疼痛进行靶向治疗了。
Left to themselves: Time to target chronic pain in childhood rare diseases.
机构信息
Biobehavioral Pediatric Pain Lab, Department of Psychiatry & Behavioral Sciences, Boston Children's Hospital, Boston, MA, 02115, USA; Center for Pain and the Brain (P.A.I.N Group), Department of Anesthesiology, Critical Care & Pain Medicine, Boston Children's Hospital, Boston, MA 02115, USA; Department of Psychiatry, Harvard Medical School, Boston, MA, 02115, USA.
Center for Pain and the Brain (P.A.I.N Group), Department of Anesthesiology, Critical Care & Pain Medicine, Boston Children's Hospital, Boston, MA 02115, USA; Department of Anesthesiology, Harvard Medical School, Boston, MA, 02115, USA.
出版信息
Neurosci Biobehav Rev. 2021 Jul;126:276-288. doi: 10.1016/j.neubiorev.2021.03.008. Epub 2021 Mar 24.
BACKGROUND
Chronic pain is prevalent among patients with rare diseases (RDs). However, little is understood about how biopsychosocial mechanisms may be integrated in the unique set of clinical features and therapeutic challenges inherent in their pain conditions.
METHODS
This review presents examples of major categories of RDs with particular pain conditions. In addition, we provide translational evidence on clinical and scientific rationale for psychosocially- and neurodevelopmentally-informed treatment of pain in RD patients.
RESULTS
Neurobiological and functional overlap between various RD syndromes and pain states suggests amalgamation and mutual modulation of the respective conditions. Emotional sequelae could be construed as an emotional homologue of physical pain mediated via overlapping brain circuitry. Given their clearly defined genetic and molecular etiologies, RDs may serve as heuristic models for unraveling pathophysiological processes inherent in chronic pain.
CONCLUSIONS
Systematic evaluation of chronic pain in patients with RD contributes to sophisticated insight into both pain and their psychosocial correlates, which could transform treatment.
背景
慢性疼痛在罕见病（RD）患者中普遍存在。然而，人们对生物心理社会机制如何整合到其疼痛状况所固有的独特临床特征和治疗挑战中还知之甚少。
方法
本综述介绍了具有特殊疼痛状况的主要类别 RD 的例子。此外，我们还提供了关于基于心理社会和神经发育的 RD 患者疼痛治疗的临床和科学依据的转化证据。
结果
各种 RD 综合征和疼痛状态之间的神经生物学和功能重叠表明，各自的情况可以合并和相互调节。情绪后果可以被理解为通过重叠的大脑回路介导的身体疼痛的情绪同源物。鉴于它们明确的遗传和分子病因，RD 可以作为阐明慢性疼痛中固有病理生理过程的启发式模型。
结论
对 RD 患者慢性疼痛的系统评估有助于深入了解疼痛及其心理社会相关因素，从而改变治疗方法。
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