[通过来自利伯维尔大学医院(加蓬)内科的十项观察,了解撒哈拉以南非洲的斯蒂尔氏病]。

Medecine tropicale et sante internationale Pub Date : 2025-01-06 eCollection Date: 2025-03-31 DOI:10.48327/mtsi.v5i1.2025.629
Josaphat Iba Ba, Annick Mfoumou, Ingrid Nseng-Nseng Ondo, Arthur Kanganga Ekomy, Léonie Esther Ledaga Lentombo, Marielle Igala, Ulrich Davy Kombila, Jean Bruno Boguikouma
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引用次数: 0

摘要

简介:介绍。斯蒂尔氏病(SD)是一种罕见的全身性炎症性疾病,最常见于儿童。在成人形式中,它可以是原发性的,也可以是婴儿形式的复苏。我们报告了在加蓬人口中对斯蒂尔氏病的10次观察,目的是确认其在该国的存在并研究其具体特征。材料和方法:这是一项回顾性、描述性和分析性研究,于2003年12月1日至2021年12月31日在利伯维尔的CHU内科进行,使用了该部门住院和/或门诊随访的患者记录。所选患者符合Yamaguchi和Fautrel标准。详细分析了流行病学、社会经济、临床、生物学、形态学、免疫学、治疗学、进化学和季节性数据。结果:纳入10例患者,男4例,女6例,多为学生,平均年龄22岁。诊断为SD的时间为31.3个月,100%的病例发烧,80%的病例关节和皮肤受累,70%的病例耳鼻喉受累。炎症综合征,主要是嗜中性粒细胞增多症,肝脏体征和高铁蛋白血症与免疫检查总是阴性共存。皮质类固醇治疗(n=10)联合甲氨蝶呤从一开始(n=1)或在皮质抵抗的情况下(n=4)是规则。1例死亡,1例失访。讨论与结论:SD的临床和生物学表现与高加索人群相当。然而,我们的研究证实了肝脏、淋巴结和心脏损伤的发生率较低,这需要在更大的队列中得到其他研究的证实。这种疾病的高成本是一种排除性诊断,这可能部分解释了诊断SD的困难。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Still's disease in sub-Saharan Africa through ten observations from the internal medicine department of Libreville University Hospital (Gabon)].

Introduction: Introduction. Still's Disease (SD) is a rare systemic inflammatory disorder that is most common in children. In the adult form, it can be primary or the resurgence of an infantile form. We report 10 observations of Still's disease in the Gabonese population, with the aim of confirming its existence in this country an studying its specific features.

Material and methods: This was a retrospective, descriptive and analytical study carried out in the Internal Medicine Department of the CHU of Libreville from 1.12.2003 to 31.12.2021, using the records of patients hospitalized in this department and/or followed up on an outpatient basis. The selected patients fulfilled the Yamaguchi and Fautrel criteria. Epidemiologic, socioeconomic, clinical, biological, morphologic, immunologic, therapeutic, evolutionary and seasonal data were detailed.

Results: Ten patients (4 men and 6 women), mostly students with a mean age of 22 years, were included. The time to diagnosis of SD was 31.3 months, with fever in 100% of cases, joint and skin involvement in 80%, and ENT involvement in 70%. An inflammatory syndrome, predominantly neutrophilic hyperleukocytosis, hepatic signs, and hyperferritinemia coexisted with an immunologic work-up that was always negative. Corticosteroid therapy (n=10) combined with per os methotrexate from the beginning (n=1) or in cases of corticoresistance (n=4) was the rule. One patient died and one was lost to follow-up.

Discussion and conclusion: The clinical and biological presentation of SD is comparable to that of the Caucasian population. However, our study confirms in the low incidence of liver, lymph node and heart damage, which needs to be confirmed by other studies in a larger cohort. The high cost of this disease, which is a diagnosis of exclusion, may partly explain the difficulties in diagnosing SD.

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