背景:镰状细胞病(SCD)是一种常染色体隐性遗传疾病,其特征在于存在称为镰状血红蛋白(HbS)的突变形式的血红蛋白(Hb)。患有SCD的个体易受各种骨关节并发症的影响。骨髓炎是影响胫骨的常见感染,股骨和肱骨的骨干,和椎骨。
目的:本研究的目的是确定SCD患者骨髓炎的诊断指标。
方法:这项研究是描述性的,分析,非干预性,对患有血管闭塞性危象(VOC)和/或骨髓炎的SCD儿科患者的前瞻性研究,通过实验室和放射学特征鉴定。纳入符合纳入标准的患者的回顾性资料。统计分析包括对队列中主要和次要结局的描述。
结果:本研究共纳入28名儿童。参与者的年龄从11个月到13岁不等。男性占参与者的大多数(64.3%)。大多数参与者(89.3%)的血培养没有增长;然而,7.1%有沙门氏菌,只有3.6%的人患有革兰氏阳性球菌。大多数病例(75%)患有白细胞增多症。血小板增多主要存在于VOC患者(40%)。CRP为1-4.9mg/dL,主要是骨髓炎患者(50%)。骨髓炎或骨髓炎和VOC患者的铁蛋白水平超过5000ng/mL(50%)。超声检查显示28例接受检查的患者中有24例没有髋关节积液。X线平片检查28例未见异常;MRI,3例骨髓水肿伴骨质强化,两名(66.7%)患者并发骨髓炎,最后(33.3%)患有骨髓炎和VOC。在接受检查的28人中,只有7人进行了抽吸,其中6例(85.7%)并发骨髓炎,而最后一个(14.3%)患有急性胸部综合征。
结论:根据本研究的结果,我们建议进行个性化的多学科检查(血液学,传染病,骨科手术,和介入放射学)用于怀疑患有VOC的骨髓炎的SCD患者,考虑整个临床病史以及实验室和MRI结果。
BACKGROUND: Sickle cell disease (SCD) is an autosomal recessive genetic disorder characterized by the presence of a mutated form of hemoglobin (Hb) known as sickle hemoglobin (HbS). Individuals with SCD are susceptible to a variety of osteoarticular complications. Osteomyelitis is a commonly seen infection affecting the tibia, diaphysis of the femur and humerus, and vertebras.
OBJECTIVE: The aim of this study was to define the indicators suggesting the diagnosis of osteomyelitis in patients with SCD.
METHODS: This study is a descriptive, analytical, non-interventional, prospective study of pediatric patients with SCD admitted with vaso-occlusive crisis (VOC) and/or osteomyelitis, which were identified by laboratory and radiological features. Retrospective data was included for patients who met the inclusion criteria. The statistical analysis included a description of the primary and secondary outcomes in the cohort.
RESULTS: A total of 28 children were included in this study. Participants\' ages ranged from 11 months to 13 years. Males represented the majority (64.3%) of the participants. The blood culture of most of the participants (89.3%) showed no growth; however, 7.1% had salmonella, and only 3.6% had Gram-positive cocci. Most cases (75%) had leukocytosis. Thrombocytosis was present mainly in patients with VOC (40%). CRP was 1-4.9 mg/dL, mainly in patients with osteomyelitis (50%). The ferritin level exceeded 5000 ng/mL in patients with osteomyelitis or both osteomyelitis and VOC (50%). Ultrasound examinations revealed no hip effusion in 24 of the 28 examined patients. A plain X-ray examination showed no abnormality in 24 out of the 28 examined cases; with MRI, three cases exhibited marrow edema with bone enhancement, two (66.7%) were complicated by osteomyelitis, and the last (33.3%) had osteomyelitis and VOC. Aspiration was performed only in seven of the 28 examined, of which six (85.7%) were complicated by osteomyelitis, while the last one (14.3%) had acute chest syndrome.
CONCLUSIONS: Based on the outcomes of this study, we recommend an individualized multidisciplinary examination (hematology, infectious disease, orthopedic surgery, and interventional radiology) for SCD patients with suspected osteomyelitis admitted with VOC, considering the entire clinical history and laboratory and MRI results.