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土耳其慢性复发性多灶性骨髓炎儿科患者的临床经验

Clinical experiences in Turkish paediatric patients with chronic recurrent multifocal osteomyelitis.

作者信息

Sözeri Betül, Ayaz Nuray Aktay, Atıkan Başak Yıldız, Karadağ Şerife Gül, Çakan Mustafa, Argın Mehmet, Sezak Murat

机构信息

Division Pediatric Rheumatology, Health Sciences University, Istanbul Umraniye Training and Research Hospital, İstanbul, Turkey.

Division Pediatric Rheumatology, Health Sciences University, Istanbul Kanuni Sultan Suleyman Education and Research Hospital, İstanbul, Turkey.

出版信息

Turk J Pediatr. 2019;61(6):879-884. doi: 10.24953/turkjped.2019.06.009.

DOI:10.24953/turkjped.2019.06.009
PMID:32134582
Abstract

Sözeri B, Aktay Ayaz N, Yıldız Atıkan B, Karadağ ŞG, Çakan M, Argın M, Sezak M. Clinical experiences in Turkish paediatric patients with chronic recurrent multifocal osteomyelitis. Turk J Pediatr 2019; 61: 879-884. Chronic recurrent multifocal osteomyelitis (CRMO) is a clinical entity which occurs mainly in children and adolescents with recurrent episodes of pain occurring over several years. Cause and physiopathology of disease is still uncertain. We aim to assess clinical characteristics and treatment options, need and response to anti-inflammatory therapies in children diagnosed chronic recurrent multifocal osteomyelitis Demographic data and clinical features of seventeen children diagnosed with CRMO in 2 pediatric rheumatology centers in Turkey were reviewed retrospectively. The diagnosis was based on clinical findings, radiological images and histopathological and microbiological studies. A total of 17 patients were included in the study. The median age of diagnosis was 9.6±4.2 years. The mean follow-up time was 31.6 months (range 6-35 months). Most patients (n: 10) had a recurrent multifocal disease course ( > 6 months), 6 patients had a persistent course and a patient had only one episode of CRMO. MEFV gene mutations were detected in 4 patients whose clinical features reduced with colchicine therapy. All patients had received nonsteroidal anti-inflammatory drugs but only one had complete response. Thirteen children with NSAID failure subsequently received corticosteroids, sulfasalazine, methotrexate, Anti TNF α drugs, or a combination of these drugs. This study is the largest cohort of pediatric CRMO patients in our country. Clinical evolution and imaging investigations should be closely done to avoid delays in diagnosis. Ethnic differences create changes in the presentation of the disease and response to treatment.

摘要

索泽里·B、阿克泰·阿亚兹·N、于尔迪兹·阿提坎·B、卡拉达格·ŞG、恰坎·M、阿尔金·M、塞扎克·M。土耳其慢性复发性多灶性骨髓炎儿科患者的临床经验。《土耳其儿科学杂志》2019年;61: 879 - 884。慢性复发性多灶性骨髓炎(CRMO)是一种主要发生在儿童和青少年中的临床病症,疼痛反复发作持续数年。该病的病因和病理生理学仍不确定。我们旨在评估诊断为慢性复发性多灶性骨髓炎的儿童的临床特征、治疗选择、对抗炎治疗的需求和反应。回顾性分析了土耳其2个儿科风湿病中心诊断为CRMO的17名儿童的人口统计学数据和临床特征。诊断基于临床发现、影像学图像以及组织病理学和微生物学研究。共有17例患者纳入研究。诊断时的中位年龄为9.6±4.2岁。平均随访时间为31.6个月(范围6 - 35个月)。大多数患者(n = 10)有复发性多灶性病程(> 6个月),6例患者病程持续,1例患者仅有一次CRMO发作。4例临床特征经秋水仙碱治疗后减轻的患者检测到MEFV基因突变。所有患者均接受了非甾体抗炎药治疗,但只有1例完全缓解。13例非甾体抗炎药治疗失败的儿童随后接受了皮质类固醇、柳氮磺胺吡啶、甲氨蝶呤、抗TNFα药物或这些药物的联合治疗。本研究是我国最大的儿科CRMO患者队列。应密切进行临床演变和影像学检查以避免诊断延误。种族差异导致疾病表现和对治疗反应的变化。

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