Castleman病变异型POEMS综合征表现为儿童多神经根神经病变:1例报告。

IF 1.6 4区 医学 Q3 CLINICAL NEUROLOGY
Magda Delgado-Landa, Selma Scheffler-Mendoza, Eduardo López-Corella, Ana Luisa Rodríguez-Lozano, Francisco Rivas-Larrauri, Melissa Espinosa-Navarro, Hiromi Onuma-Zamayoa, Marco Antonio Yamazaki-Nakashimada
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引用次数: 0

摘要

POEMS综合征(多神经病变、器官肿大、内分泌病变、单克隆蛋白、皮肤变化)是一种罕见的多系统疾病,与浆细胞异常有关,通常影响成年人。儿科病例非常罕见,以前报道的最小患者为10岁。我们提出一个6岁男孩的情况下,其症状包括进行性下肢无力,淋巴结病,和血小板增多。最初误诊为格林-巴利综合征,淋巴结病变显示Castleman病伴色素沉着、多毛、乳头水肿和肝肿大,证实了诊断。尽管没有单克隆蛋白和硬化性骨病变,多学科方法导致皮质类固醇,沙利度胺和环磷酰胺治疗成功,导致完全缓解。这个病例强调了儿童POEMS综合征的诊断挑战,因为它的异质性和与其他疾病的重叠。早期识别是至关重要的,因为儿科患者表现出良好的预后,及时和适当的治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Castleman Disease Variant POEMS Syndrome Presenting as Polyradiculoneuropathy in a Child: A Case Report.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare multisystemic disorder linked to plasma cell abnormalities, typically affecting adults. Pediatric cases are extraordinarily rare, with the youngest previously reported patient being 10 years old. We present the case of a 6-year-old boy whose symptoms included progressive lower limb weakness, lymphadenopathy, and thrombocytosis. Initially misdiagnosed as Guillain-Barré syndrome, the finding of lymphadenopathy revealing Castleman disease along with hyperpigmentation, hypertrichosis, papilledema, and hepatomegaly confirmed the diagnosis. Despite the absence of monoclonal protein and sclerotic bone lesions, the multidisciplinary approach led to successful treatment with corticosteroids, thalidomide, and cyclophosphamide, resulting in full remission. This case highlights the diagnostic challenges of POEMS syndrome in children, given its heterogeneity and overlap with other conditions. Early recognition is crucial, as pediatric patients show favorable prognosis with prompt and appropriate treatment.

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来源期刊
Journal of Child Neurology
Journal of Child Neurology 医学-临床神经学
CiteScore
4.20
自引率
5.30%
发文量
111
审稿时长
3-6 weeks
期刊介绍: The Journal of Child Neurology (JCN) embraces peer-reviewed clinical and investigative studies from a wide-variety of neuroscience disciplines. Focusing on the needs of neurologic patients from birth to age 18 years, JCN covers topics ranging from assessment of new and changing therapies and procedures; diagnosis, evaluation, and management of neurologic, neuropsychiatric, and neurodevelopmental disorders; and pathophysiology of central nervous system diseases.
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