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儿童重症组织细胞坏死性淋巴结炎(菊池-藤本病)的临床特征。

Clinical Characteristics of Severe Histiocytic Necrotizing Lymphadenitis (Kikuchi-Fujimoto Disease) in Children.

机构信息

Department of Pediatrics, Seoul National University College of Medicine, Seoul, Republic of Korea; Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Republic of Korea; Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Republic of Korea.

Department of Radiology, Seoul National University Bundang Hospital, Seongnam, Republic of Korea.

出版信息

J Pediatr. 2016 Apr;171:208-12.e1. doi: 10.1016/j.jpeds.2015.12.064. Epub 2016 Feb 4.

DOI:10.1016/j.jpeds.2015.12.064
PMID:26852178
Abstract

OBJECTIVES

To analyze the clinical characteristics of children with Kikuchi-Fujimoto disease focusing on cases with prolonged fever.

STUDY DESIGN

This was a retrospective study of children diagnosed with Kikuchi-Fujimoto disease from March 2003 to February 2015 in South Korea. Electronic medical records were searched for clinical and laboratory manifestations.

RESULTS

Among 86 histopathologically confirmed cases, the mean age was 13.2 (SD ± 3.1) years, and male to female ratio was 1:1.32. Cervical lymph node enlargement, found in 85 of the patients (99%), was predominantly unilateral in 64 (75%), and involved the cervical lymph node level V in 67 (81%). Fever was present in 76% of the cases, with a median duration of 9 days (IQR 0.25-17.0). Multivariate analysis revealed that a high fever peak ≥ 39.0°C (P = .010) and presentation with ≥ 2 systemic symptoms other than fever (P = .027) were factors that were significantly associated with longer fever duration. As the size of the largest lymph node's short diameter increased, the fever duration increased (P = .015). Leukopenia (P = .022) also had a significant association with a longer fever duration. Patients with sonographic findings of conglomerated enlarged lymph nodes had a longer median duration of fever compared with those with separate enlarged lymph nodes (11 vs 4.5 days, P = .019).

CONCLUSIONS

Patients with high fever, more systemic symptoms, leukopenia, and larger lymph nodes with a conglomerated distribution may benefit from early recognition and selective consideration of corticosteroid therapy.

摘要

目的

分析以发热时间延长为特点的儿童组织细胞坏死性淋巴结炎的临床特征。

研究设计

本研究是一项回顾性研究,纳入了 2003 年 3 月至 2015 年 2 月期间在韩国被诊断为组织细胞坏死性淋巴结炎的儿童病例。检索电子病历以获取临床和实验室表现。

结果

在 86 例经组织病理学证实的病例中,平均年龄为 13.2 岁(标准差±3.1 岁),男女比例为 1:1.32。85 例(99%)患者存在颈部淋巴结肿大,64 例(75%)以单侧为主,67 例(81%)累及颈淋巴结Ⅴ区。76%的病例存在发热,中位数持续时间为 9 天(IQR 0.25-17.0)。多变量分析显示,高热峰值≥39.0°C(P=.010)和除发热外存在≥2 种全身症状(P=.027)是发热持续时间延长的显著相关因素。随着最大淋巴结短径的增大,发热持续时间也随之增加(P=.015)。白细胞减少(P=.022)与发热持续时间延长也显著相关。与淋巴结单独肿大相比,超声显示淋巴结聚集肿大的患者发热持续时间中位数更长(11 天 vs. 4.5 天,P=.019)。

结论

发热较高、全身症状较多、白细胞减少、淋巴结肿大呈聚集分布的患者可能需要早期识别,并考虑选择皮质类固醇治疗。

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